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1.  595 Urticarial Rash Associated with Chest Pain 
Background
Urticaria may be the first manifestation of an underlying systemic disease (tumors, infections, collagen vascular or thyroid disease. Differential diagnosis must be made with many entities that can be manifested with a similar skin injury.
Methods
A 49 year-old man who during 2 years has monthly multi-days episodes of generalized pruritic papular skin lesions, responding to steroids but not to antihistamines. Occasionally associated with joint pain. Two skin injuries biopsies informed of simple urticaria.
One year after skin lesion onset, he began with chest pain episodes suggestive of angina pectoris with elevated necrosis enzyme markers and ischemic changes on EKG. Angina episodes were sometimes preceded by skin lesion outbreak and it responded to steroid. Coronary catheterization was negative twice, so the diagnosis was vasospastic angina. Later he presented cough, wheezing, elevation of transaminases, LDH, FA, GGT, CPR and fibrinogen, 800 eosinophils in peripheral blood. Sputum eosinophils 40 to 60%.Chest X-Ray objective a thickened left hilum and doubtful left parahilar infiltrated.
Results
Allergologic study—Skin prick test with aeroallergens and wide food battery were negative. Specific IgE against Anisakis, latex, Echinococcus and other blood parameters including serology, autologous patient serum skin test were all normal/negative. Tryptase determination at baseline and during skin lesion shoot: normal. Other explorations—ECO-cardio: inferior basal akinesia and inferoposterior hypokinesia, LVEF 60%, normal RV systolic function and valves. CT scan visualize mediastinal and abdominal adenopathy, splenomegalia and ureterolithiasis. Mediastinoscopy and biopsy of right paratracheal grainy adenopathy confirms the diagnosis of sarcoidosis. ACE: 250 U/L. Gallium67 scan suggestive mediastinal sarcoidosis. Heart RM scan: no evidence of morphological criteria for cardiac sarcoidosis diagnosis.
Conclusions
Sarcoidosis is a multisystem granulomatous disease of unknown etiology. It may affect almost any organ, predominantly lung, lymph nodes and skin. Cardiac involvement is 25% but only symptomatic in 5%. We report a patient with sarcoidosis and vasoespastic angina. It´s described cases of cardiac sarcoidosis and vasospastic angina. In this case we cannot demonstrate cardiac injury. Sarcoidosis is a great simulating of cutaneous lesions and it can imitate to urticaria.
doi:10.1097/01.WOX.0000411710.02257.fa
PMCID: PMC3512883
2.  Sarcoidosis in a 65-year-old woman presenting with a lung mass and pericardial effusion: a case report 
Introduction
Sarcoidosis is a multi-systemic disorder of unknown origin and most commonly affects the lungs. Diagnosis relies on the presence of non-caseating granulomas on histologic specimens. In high-resolution computed tomography, the most characteristic findings are peribronchovascular thickening, perilymphatic nodular distribution, and bilateral hilar adenopathy. Confluent nodular opacities or large masses are rare manifestations of the disease. It is well recognized that sarcoidosis can mimic infectious, malignant, and granulomatous conditions. Here, we report a case with a high initial index of suspicion for lung malignancy in terms of clinical, lung imaging, and endoscopic findings.
Case presentation
A 65-year-old Caucasian woman, lifelong non-smoker with an unremarkable medical history, presented with a 10-month history of progressive breathlessness, dry cough, fatigue, arthralgias, and mild weight loss. The only significant clinical finding was bilateral enlargement of auxiliary lymph nodes. High-resolution computed tomography revealed a soft tissue density mass at the right hilum which was surrounding and narrowing airways and vascular components, nodules with vascular distribution, enlarged mediastinal lymph nodes, and pericardial effusion. Our patient underwent a bronchoscopy, which revealed the presence of submucosal infiltration and narrowing of the right upper bronchus. Endobronchial biopsies showed non-caseating granulomas. As local sarcoid reactions with non-caseating granulomas can be observed near tumors, our patient underwent video-assisted thoracoscopy and surgical removal of an auxiliary lymph node, both of which confirmed the presence of non-caseating granulomas and the diagnosis of sarcoidosis. She was treated with steroids with improvement of clinical and imaging findings. However, while on a maintenance dose, she presented with a pleural effusion, which, after the diagnostic work-up, proved to be sarcoidosis-related. Treatment with initially high doses of steroids plus a steroid-sparing agent led to resolution of the effusion.
Conclusions
We report a case with a high initial index of suspicion for lung malignancy. Clinicians should always be aware that sarcoidosis enters the differential diagnosis of patients presenting with a lung mass that encases and narrows bronchial and vascular structures with associated pericardial effusion. Rarely, pleural effusion can be the presenting symptom of disease relapse despite maintenance treatment.
doi:10.1186/1752-1947-6-259
PMCID: PMC3443668  PMID: 22937889
Sarcoidosis; Lung cancer; Pleural effusion; Pericardial effusion.
3.  Atypical Presentation of Hepatic Sarcoidosis 
Introduction
Sarcoidosis is a multisystem inflammatory disease characterized by noncaseating granulomas. It affects approximately 34 in 100,000 African Americans. Hepatic involvement is rare and is typically subclinical with only 0.1% to 0.9% of patients with clinically significant GI symptoms. We present a case of previously undiagnosed sarcoidosis with initial presentation of nausea, vomiting, pruritus, and severe abdominal pain with a cholestatic pattern of liver injury.
Case Presentation
A 42-year-old African American woman with prior cholecystectomy and a history of diabetes mellitus, type 2 was admitted with severe right upper quadrant pain, nausea, vomiting, pruritus and a 45 pound weight loss over the course of 3 months. Physical exam showed diffuse abdominal pain without peritoneal signs worse in the RUQ and epigastric region with positive Murphy's sign. Labs revealed cholestatictransaminitis with elevated direct bilirubin and dramatically elevated alkaline phosphatase. RUQ ultrasound and MRCP were normal. An infectious workup was negative and the patient was not taking any hepatotoxic drugs. Serum anti-mitochondrial antibody was negative and subsequent biopsy revealed a large number of noncaseating granulomas with notable autoimmune biliary pathology on initial pathology read. Although a chest x-ray was normal, a chest CT revealed mediastinal lymphadenopathy. A fine needle aspiration showed noncaseating granulomatous lymphadenitis. ACE level was elevated at 101 U/L. Diagnosis of multisystem sarcoidosis was made. Prednisone was started and the patient was discharged several days later with nearly complete resolution of her symptoms.
Discussion
Sarcoidosis is a rare inflammatory disease identified most commonly in African American patients. Hepatic involvement is typically subclinical and found on routine blood work in 10%–30% of patients with pulmonary sarcoidosis. In this atypical case the patient presented with a clinical picture mimicking acute biliary pathology and no symptoms or classic pulmonary findings of pulmonary sarcoidosis. A few rare cases of symptomatic GI sarcoidosis with a biliary obstructive picture are noted in the literature, however, these patients are exclusively males and also presented with fever and hepatosplenomegaly, so sarcoidosis was not high on the initial differential. Infectious etiologies of granulomatous hepatitis including HIV, TB and fungal infection were ruled out. She was not on any drugs reported to cause granulomatous hepatitis. Primary biliary cirrhosis became the initial working diagnosis. However, the patient's AMA negative status and a pathology addendum that noted only minimal biliary tree involvement prompted a reevaluation and workup for sarcoidosis. In this atypical case of hepatic sarcoidosis the patient presented with a clinical picture consistent with acute biliary pathology and no symptoms of sarcoidosis. However, demonstration of a granulomatous hepatitis without other apparent cause and negative AMA in a high-risk demographic patient led to a correct diagnosis.
PMCID: PMC3764591
4.  Thin-section CT findings in Pseudomonas aeruginosa pulmonary infection 
The British Journal of Radiology  2012;85(1020):1533-1538.
Objective
The aim of this study was to assess clinical and pulmonary thin-section CT findings in patients with acute Pseudomonas aeruginosa (PA) pulmonary infection.
Methods
We retrospectively identified 44 patients with acute PA pneumonia who had undergone chest thin-section CT examinations between January 2004 and December 2010. We excluded nine patients with concurrent infections. The final study group comprised 35 patients (21 males, 14 females; age range 30–89 years, mean age 66.9 years) with PA pneumonia. The patients' clinical findings were assessed. Parenchymal abnormalities, enlarged lymph nodes and pleural effusion were evaluated on thin-section CT.
Results
Underlying diseases included malignancy (n=13), a smoking habit (n=11) and cardiac disease (n=8). CT scans of all patients revealed abnormal findings, including ground-glass opacity (n=34), bronchial wall thickening (n=31), consolidation (n=23) and cavities (n=5). Pleural effusion was found in 15 patients.
Conclusion
PA pulmonary infection was observed in patients with underlying diseases such as malignancy or a smoking habit. The CT findings in patients with PA consisted mainly of ground-glass attenuation and bronchial wall thickening.
Advances in knowledge
The CT findings consisted mainly of ground-glass attenuation, bronchial wall thickening and cavities. These findings in patients with an underlying disease such as malignancy or a smoking habit may be suggestive of pneumonia caused by PA infection.
doi:10.1259/bjr/54468236
PMCID: PMC3611710  PMID: 22844034
5.  MMP1 and MMP7 as Potential Peripheral Blood Biomarkers in Idiopathic Pulmonary Fibrosis 
PLoS Medicine  2008;5(4):e93.
Background
Idiopathic pulmonary fibrosis (IPF) is a chronic progressive fibrotic lung disease associated with substantial morbidity and mortality. The objective of this study was to determine whether there is a peripheral blood protein signature in IPF and whether components of this signature may serve as biomarkers for disease presence and progression.
Methods and Findings
We analyzed the concentrations of 49 proteins in the plasma of 74 patients with IPF and in the plasma of 53 control individuals. We identified a combinatorial signature of five proteins—MMP7, MMP1, MMP8, IGFBP1, and TNFRSF1A—that was sufficient to distinguish patients from controls with a sensitivity of 98.6% (95% confidence interval [CI] 92.7%–100%) and specificity of 98.1% (95% CI 89.9%–100%). Increases in MMP1 and MMP7 were also observed in lung tissue and bronchoalveolar lavage fluid obtained from IPF patients. MMP7 and MMP1 plasma concentrations were not increased in patients with chronic obstructive pulmonary disease or sarcoidosis and distinguished IPF compared to subacute/chronic hypersensitivity pneumonitis, a disease that may mimic IPF, with a sensitivity of 96.3% (95% CI 81.0%–100%) and specificity of 87.2% (95% CI 72.6%–95.7%). We verified our results in an independent validation cohort composed of patients with IPF, familial pulmonary fibrosis, subclinical interstitial lung disease (ILD), as well as with control individuals. MMP7 and MMP1 concentrations were significantly higher in IPF patients compared to controls in this cohort. Furthermore, MMP7 concentrations were elevated in patients with subclinical ILD and negatively correlated with percent predicted forced vital capacity (FVC%) and percent predicted carbon monoxide diffusing capacity (DLCO%).
Conclusions
Our experiments provide the first evidence for a peripheral blood protein signature in IPF to our knowledge. The two main components of this signature, MMP7 and MMP1, are overexpressed in the lung microenvironment and distinguish IPF from other chronic lung diseases. Additionally, increased MMP7 concentration may be indicative of asymptomatic ILD and reflect disease progression.
Naftali Kaminski and colleagues find increased levels of specific proteins in the bloodstream of individuals with idiopathic pulmonary fibrosis, and suggest that these proteins may ultimately provide a biomarker for the disease.
Editors' Summary
Background.
Idiopathic pulmonary fibrosis (IPF) is a serious disease in which the lungs become progressively scarred or thickened for unknown reasons. In healthy people, air is taken in through the mouth or nose and travels down the windpipe into tubes in the lungs called the airways. Each airway has many small branches that end in alveoli, tiny air sacs with thin walls that are surrounded by small blood vessels called capillaries. When air reaches the alveoli, the oxygen in it passes into the bloodstream and is taken to the organs of the body to keep them working. In IPF, the alveoli and the space around them (the “interstitial” area) gradually become scarred and thickened, which stops oxygen's movement into the bloodstream. When only small areas of the lung are scarred, IPF may cause no symptoms. But, as more of the lung becomes damaged, IPF eventually causes breathlessness, even when resting. There is no effective treatment for IPF, although steroids and drugs that suppress the body's immune system are often tried in an attempt to slow its progression. On average, half of the people with IPF die within three years of diagnosis, often from respiratory or heart failure.
Why Was This Study Done?
It can be difficult to diagnose IPF—there are many lung diseases with similar symptoms, including numerous other interstitial lung diseases—and currently, physicians can only follow the progression of IPF by repeatedly testing their patients' lung function or by doing multiple chest X-rays. If proteins could be identified whose level in blood indicated disease activity (so-called “peripheral blood biomarkers”), it would be easier to diagnose and monitor patients. In addition, the identification of such biomarkers might suggest new drug targets for the treatment of IPF. In this study, the researchers look for peripheral blood biomarkers in IPF by using a “multiplex analysis” system to measure the level of several proteins in patient blood samples simultaneously.
What Did the Researchers Do and Find?
The researchers measured the levels of 49 plasma proteins (plasma is the fluid part of blood) in 74 patients with IPF and 53 healthy people (controls) and used a technique called “recursive partitioning” to define a five-protein signature that distinguished patients from unaffected study participants (controls). Matrix metalloproteinase 7 (MMP7) and MMP1—the two plasma proteins whose levels were most increased in patients with IPF compared to controls—were key components of this signature. Concentrations of MMP7 and MMP1 were higher in bronchoalveolar lavage samples (fluid obtained by washing out the lungs with saline) and in lung tissue samples from patients with IPF than in similar samples taken from healthy individuals. Plasma concentrations of MMP7 and MMP1 were significantly higher in patients with IPF than in patients with hypersensitivity pneumonitis, an interstitial lung disease that mimics IPF, but not increased in patients with chronic obstructive pulmonary disease or sarcoidosis, two other lung diseases. In an independent validation group, patients with IPF and familial pulmonary fibrosis had increased plasma concentrations of MMP7 and MMP1 that correlated with the severity of their disease. In addition, MMP7 concentrations were raised in close relatives of people with familial pulmonary fibrosis who had normal lung function tests but some lung scarring.
What Do These Findings Mean?
These findings provide evidence for a protein signature in the blood for IPF and suggest MMP1 and MMP7 may be useful as biomarkers for IPF. These two matrix metalloproteinases have previously been suggested to be involved in the development of IPF. However, additional work is probably needed to confirm that increased plasma concentrations MMP7 and MMP1 are specific for IPF, since it may be that these markers will not distinguish IPF from other interstitial lung diseases.
Additional Information.
Please access these Web sites via the online version of this summary at http://dx.doi.org/10.1371/journal.pmed.0050093.
Read a related PLoS Medicine Perspective article
The MedlinePlus Encyclopedia has a page on idiopathic pulmonary fibrosis (in English and Spanish) and on pulmonary fibrosis
The US National Heart Lung and Blood Institute and the British Lung Foundation also provide information on IPF for patients and relatives
Some of the researchers involved in this study provide more details about what might go wrong in IPF in a recent PLoS Medicine article
doi:10.1371/journal.pmed.0050093
PMCID: PMC2346504  PMID: 18447576
6.  Acute Klebsiella pneumoniae pneumonia alone and with concurrent infection: comparison of clinical and thin-section CT findings 
The British Journal of Radiology  2010;83(994):854-860.
The purpose of this study was to identify the clinical and thin-section CT findings in patients with acute Klebsiella pneumoniae pneumonia (KPP) alone and with concurrent infection. We retrospectively identified 160 patients with acute KPP who underwent chest thin-section CT examinations between August 1998 and August 2008 at our institution. The study group comprised 80 patients (54 male, 26 female; age range 18–97 years, mean age 61.5) with acute KPP alone, 55 (43 male, 12 female; age range 46–92 years, mean age 76.0) with KPP combined with methicillin-resistant Staphylococcus aureus (MRSA) and 25 (23 male, 2 female; age range 56–91 years, mean age 72.7) with KPP combined with Pseudomonas aeruginosa (PA). Underlying diseases in patients with each type of pneumonia were assessed. Parenchymal abnormalities were evaluated along with enlarged lymph nodes and pleural effusion. In patients with concurrent pneumonia, underlying conditions such as cardiac diseases, diabetes mellitus and malignancy were significantly more frequent than in patients with KPP alone. The mortality rate in patients with KPP combined with MRSA or PA was significantly higher than in those with KPP alone. In concurrent KPP, CT findings of centrilobular nodules, bronchial wall thickening, cavity, bronchiectasis, nodules and pleural effusion were significantly more frequent with concurrent pneumonia than in those with KPP alone.
doi:10.1259/bjr/28999734
PMCID: PMC3473742  PMID: 20647513
7.  Radiological findings in acute Haemophilus influenzae pulmonary infection 
The British Journal of Radiology  2012;85(1010):121-126.
Objective
The aim of this study was to assess pulmonary thin-section CT findings in patients with acute Haemophilus influenzae pulmonary infection.
Methods
Thin-section CT scans obtained between January 2004 and March 2009 from 434 patients with acute H. influenzae pulmonary infection were retrospectively evaluated. Patients with concurrent infection diseases, including Streptococcus pneumoniae (n=76), Staphylococcus aureus (n=58) or multiple pathogens (n=89) were excluded from this study. Thus, our study group comprised 211 patients (106 men, 105 women; age range, 16–91 years, mean, 63.9 years). Underlying diseases included cardiac disease (n=35), pulmonary emphysema (n=23), post-operative status for malignancy (n=20) and bronchial asthma (n=15). Frequencies of CT patterns and disease distribution of parenchymal abnormalities, lymph node enlargement and pleural effusion were assessed by thin-section CT.
Results
The CT findings in patients with H. influenzae pulmonary infection consisted mainly of ground-glass opacity (n=185), bronchial wall thickening (n=181), centrilobular nodules (n=137) and consolidation (n=112). These abnormalities were predominantly seen in the peripheral lung parenchyma (n=108). Pleural effusion was found in 22 patients. Two patients had mediastinal lymph node enlargement.
Conclusion
These findings in elderly patients with smoking habits or cardiac disease may be characteristic CT findings of H. influenzae pulmonary infection.
doi:10.1259/bjr/48077494
PMCID: PMC3473957  PMID: 21224303
8.  Gamma/delta cells in tissue from patients with sarcoidosis. 
Thorax  1996;51(11):1123-1126.
BACKGROUND: Because gamma/delta T lymphocytes (gamma delta cells) respond to myco-bacterial antigens in vitro and accumulate in the skin lesions of patients with certain granulomatous infections (leprosy, leishmaniasis), it was hypothesised that these cells might have a role in the pathogenesis of sarcoidosis, a disease also characterised by granuloma formation. Having failed to demonstrate an increase in gamma delta cells in the blood of patients with sarcoidosis, the aim of this study was to examine samples of bronchoalveolar lavage (BAL) fluid and biopsy tissue. METHODS: Samples from 23 patients (13 women) with newly diagnosed sarcoidosis, of mean age 31 years and median percentage of lymphocytes in the BAL fluid of 31%, were studied. Controls included normal subjects and patients with other interstitial lung diseases (ILD). Cytopreparations of BAL fluid (n = 13) and cryostat sections (five mediastinal nodes, 14 transbronchial biopsies) were stained with alkaline phosphatase-antialkaline phosphatase and monoclonal antibodies to CD3, CD4, CD8, CD25, and gamma delta T cell receptor (TCR). RESULTS: All patients had typical chest radiographs (16 stage I, four stage II, three stage III). All were Mantoux negative with negative tuberculosis cultures. Compared with normal controls and patients with other interstitial lung diseases there was no increase in gamma delta cells in the BAL fluid (sarcoidosis, 1% (range 0-4%) total cells; ILD, 1% (0-2%); controls, 0.5% (0-2%); p > 0.05, Kruskal-Wallis). Likewise, there was no increase in gamma delta cells in the transbronchial biopsy specimens (sarcoidosis, 1/high power field (hpf) (range 0-2); ILD, < 1/hpf (0-4); controls < 1/hpf (0-2); p > 0.05). gamma delta cells were rarely seen in the lymph nodes in spite of the presence of numerous granulomas. CONCLUSION: These results provide further evidence that gamma delta cells are not increased in most patients with sarcoidosis.
PMCID: PMC1090524  PMID: 8958896
9.  AB 69. Coexistence of Wilson’s disease and sarcoidosis in a 35-year-old female 
Journal of Thoracic Disease  2012;4(Suppl 1):AB69.
Background
The simultaneous diagnosis of two relatively rare co-existing diseases.
Patients and methods
Description of clinical and laboratory findings.
Results
A thirty-five year-old female was referred to a neurology department for symptoms of resting, postural and kinetic tremor of the upper extremities as well as head tremor. Diagnostic workup revealed Kaiser-Fleischer rings in both eyes, high levels of copper in the urine (200 μg/24 h with normal value [n.v.] <100, low levels of ceruloplasmine (17.5 mg/dL-n.v.22-58) and marginally low serum copper (0.6 μg/mL-n.v.0.7-1.4). A diagnosis of Wilson’s disease was established. The patient’s chest radiograph, however, showed enlarged pulmonary hili which were confirmed, by computed tomography, to represent enlarged lymph nodes. The patient’s angiotensin converting enzyme was 72.2 U/L (n.v. 12-68), spirometry was normal (FEV1: 87%, FVC: 88%, Dlco: 81%, FRC: 89%, RV: 82%, TLC: 84%) and she did not have considerable hemoglobin desaturation during a six-minute walk test (97% to 96%, distance walked: 360 m). A bronchoalveolar lavage was performed: Cells: 0.132×106, alveolar macrophages 44%, lymphocytes 42%, neutrophils 6%, mononuclear 3%, eosinophils 5%. Ratio CD4/CD8: 2.57. Τhe patient was started on triethylenetetramine (Trientin) for her primary disease and was followed up for her stage I sarcoidosis. Three years later she remains clinically stable with no respiratory symptoms, with unchanged findings from spirometry and computed tomography regarding sarcoidosis. The coexistence of these two diseases is rare. Only one similar case has been reported. It concerned a forty-three year-old male, who presented with symptoms and signs of cirrhosis and no neurologic symptoms. He had been diagnosed with sarcoidosis nine years earlier and been treated with corticosteroids.
Conclusions
The existence of one rare disease should not deter the search towards a coexisting disease if signs and symptoms are not compatible with the first one.
doi:10.3978/j.issn.2072-1439.2012.s069
PMCID: PMC3537433
10.  Interferon-alpha-induced sarcoidosis in a patient being treated for hepatitis C 
Patient: Female, 43
Final Diagnosis: —
Symptoms: Diarrhea • generalized weakness • headache • lightheadedness • nausea • rash • short of breath • vomiting
Medication: —
Clinical Procedure: —
Specialty: Pulmonology
Objective:
Rare diseae
Background:
IFN-alpha-2b in combination with ribavirin is now the standard of care for the treatment of hepatitis C. Sarcoidosis is a chronic multisystem granulomatous disorder characterized by noncaseating granulomas in the involved organs. The pathologic hallmark of sarcoidosis is the presence of noncaseating granulomas in the interstitium that typically involve the lymphatics.
Case Report:
A 43-year-old woman presented to our care with 2-week history of nausea, vomiting, diarrhea, shortness of breath, migraine headache, maculopapular rash, generalized weakness, and lightheadedness. She had been treated for hepatitis C with telaprevir, ribavirin, and interferon-alpha-2b for 6 months. Chest radiograph showed bilateral diffuse prominence of bronchovascular markings. CT of the chest revealed bilateral diffuse centrilobular nodules with associated intralobular septal thickening, thickening of the central peribronchovascular interstitium, nodularity of the major fissures, and mediastinal lymphadenopathy. These findings were suspicious for atypical pulmonary sarcoidosis, possibly interferon-induced. The pathology of the mediastinal lymph node biopsy revealed noncaseating granulomatous inflammation consistent with the diagnosis of pulmonary sarcoidosis. Pathology of the skin punch biopsy showed giant-cell granulomatous inflammation without necrosis. The patient was started on prednisone 40 mg daily with a steroid tapering course for 8 weeks.
Conclusions:
The management of IFN-induced sarcoidosis includes the discontinuation of IFN therapy with or without the administration of systemic corticosteroids. With the increasing prevalence of HCV in the United States, it is likely that more IFN-alpha-induced sarcoidosis will be encountered by clinicians.
doi:10.12659/AJCR.890180
PMCID: PMC4043539  PMID: 24900166
Hepatitis C; Interferons - adverse effects; Sarcoidosis - chemically induced
11.  Smoking Inhibits the Frequency of Bronchovascular Bundle Thickening in Sarcoidosis 
Academic radiology  2011;18(7):885-891.
Rationale/Objectives
Smoking has been associated with decreased incidence and prevalence of sarcoidosis, but few studies have evaluated effects of smoking on clinical parameters of the disease. The objectives were to determine the association of smoking with radiographic patterns and to evaluate the associations of these smoking-related radiographic patterns on airflow obstruction in sarcoidosis.
Methods
Clinical data and CT scans of 124 patients with sarcoidosis were reviewed. CT scans were assessed for lymph nodes, nodules, bronchiectasis, bronchovascular bundle thickening, displaced hilum, fibrosis, ground glass, emphysema, pleural changes, and alveolar opacities. CT patterns were compared between patients with and without a history of smoking. The effect of smoking on the associations between radiographic patterns and airflow obstruction was assessed with multivariable analysis.
Results
Smokers had less frequency of bronchovascular bundle thickening than nonsmokers (11/38 subjects(29%) vs. 50/86 subjects(58%),p=0.003) and more emphysema (7/38 subjects(18%) vs. 1/86 subjects(1%),p=0.001). Patients who had bronchovascular bundle thickening were less likely to have ever smoked (11/61 subjects(18%) vs. 27/63 subjects(43%), p=0.003) or be current smokers (4/61 subjects(7%) vs. 15/63 subjects(24%),p=0.008). Age (p=0.003) and bronchovascular bundle thickening (p=0.02) were independent predictors of airflow obstruction. There were no differences in smoking history between patients with airflow obstruction versus those without (10/37 subjects(27)% vs. 28/87 subjects(32%),p=0.63).
Conclusions
In patients with sarcoidosis, smoking is associated with decreased frequency of bronchovascular bundle thickening, a very important clinical manifestation of the lung disease. Further, bronchovascular bundle thickening and age are the only independent predictors of airflow obstruction, and smoking does not confound these associations.
doi:10.1016/j.acra.2011.02.015
PMCID: PMC3115466  PMID: 21530329
Smoking; CT scan; X-ray; Sarcoidosis; Airway Obstruction; Granuloma
12.  Sarcoidosis with Multi-organ Involvement Presenting as Ventricular Tachycardia 
Introduction:
Sarcoidosis is a granulomatous disease which can affect any organ, but most commonly presents with pulmonary manifestations. We present an unusual case of sarcoidosis with multiorgan involvement which initially manifested as ventricular tachycardia; the patient was subsequently found to have extensive cardiac, respiratory, skin, and hepatic involvement.
Case Report:
An otherwise healthy 35-year-old African American soldier presented to emergency care with hypotension and tachycardia greater than 200 beats per minute. He was found to be in sustained ventricular tachycardia with a pulse. Defibrillation was performed, the patient was loaded with amiodarone, and hospitalized. Cardiac catheterization showed no evidence of atherosclerotic coronary artery disease. A cardiac MRI revealed high density lesions in the lateral wall and apex, and diffusely decreased left ventricular wall motion. An implantable cardiac defibrillator (ICD) was placed. Further questioning revealed a six month history of twenty pounds weight loss, persistent nasal congestion, and subcutaneous nodules. Biopsy of two subcutaneous nodules from the left upper extremity and nasal mucosa biopsy demonstrated noncaseating granulomas. His evaluation was notable for a mixed pattern hepatic injury, normocytic anemia, elevated lactate dehydrogenase, and a CT chest showing hilar and mediastinal adenopathy with multiple pulmonary nodules. Sarcoidosis was suspected and additional testing for HIV, EBV, vasculitis, lymphoma, fungal infection, and mycobacterial infection was negative. Pulmonary function tests showed normal basic spirometry and DLCO. Transthoracic echocardiogram demonstrated normal ejection fraction, and an 8mm cystic structure partially incorporated in the left ventricular wall. This was suspected to be a granulomatous accumulation. Sestamibi imaging showed a dense defect involving the apex and a medium sized, moderate severity, inferior wall defect consistent with an infiltrative process. Interrogation of his ICD six months after placement demonstrated no recurrence of arrhythmia. Initially, he was treated with 40mg prednisone daily. After a three-month course he had continued active disease and was treated with infliximab. His treatment is ongoing; lab parameters are improving.
Discussion:
Cardiac sarcoidosis is rare. Conduction abnormalities are the most common finding, and arrhythmias are second. Heart failure, valvular dysfunction, and chronic effusion are also frequently observed, and one case report describes a large left atrial mass which behaved like a myxoma. This patient's case is unusual because of his large degree of sinus and cardiac involvement, as well as his unusual left ventricular cystic structure. Sarcoidosis should be considered in all patients who have unexplained structural heart disease, particularly young individuals. Treatment of cardiac sarcoidosis is aimed at controlling inflammation and preventing compromise of cardiac structure or function. Sources agree that steroids are an effective initial treatment, but the initial dose and optimal duration are unclear. This patient's course suggests that infliximab is an efficacious treatment option in severe cases.
PMCID: PMC4175938
13.  Meticillin-resistant Staphylococcus aureus and meticillin-susceptible S. aureus pneumonia: comparison of clinical and thin-section CT findings 
The British Journal of Radiology  2012;85(1014):e168-e175.
Objectives
The purpose of this study was to compare the clinical and thin-section CT findings in patients with meticillin-resistant Staphylococcus aureus (MRSA) and meticillin-susceptible S. aureus (MSSA).
Methods
We retrospectively identified 201 patients with acute MRSA pneumonia and 164 patients with acute MSSA pneumonia who had undergone chest thin-section CT examinations between January 2004 and March 2009. Patients with concurrent infectious disease were excluded from our study. Consequently, our study group comprised 68 patients with MRSA pneumonia (37 male, 31 female) and 83 patients with MSSA pneumonia (32 male, 51 female). Clinical findings in the patients were assessed. Parenchymal abnormalities, lymph node enlargement and pleural effusion were assessed.
Results
Underlying diseases such as cardiovascular were significantly more frequent in the patients with MRSA pneumonia than in those with MSSA pneumonia. CT findings of centrilobular nodules, centrilobular nodules with a tree-in-bud pattern, and bronchial wall thickening were significantly more frequent in the patients with MSSA pneumonia than those with MRSA pneumonia (p=0.038, p=0.007 and p=0.039, respectively). In the group with MRSA, parenchymal abnormalities were observed to be mainly peripherally distributed and the frequency was significantly higher than in the MSSA group (p=0.028). Pleural effusion was significantly more frequent in the patients with MRSA pneumonia than those with MSSA pneumonia (p=0.002).
Conclusions
Findings from the evaluation of thin-section CT manifestations of pneumonia may be useful to distinguish between patients with acute MRSA pneumonia and those with MSSA pneumonia.
doi:10.1259/bjr/65538472
PMCID: PMC3474104  PMID: 21750126
14.  HRCT findings of pulmonary sarcoidosis; relation to pulmonary function tests 
Background
Chest-X-ray has several limitations in detecting the extent of pulmonary disease in sarcoidosis. It might not reflect the degree of pulmonary involvement in patients with sarcoidosis when compared to computed tomography of the thorax. We aimed to investigate the HRCT findings of pulmonary sarcoidosis and to find out the existence of possible relations between HRCT findings and PFTs. In addition, we aimed to investigate the accordance between HRCT findings and conventional chest-X-ray staging of pulmonary sarcoidosis.
Method
45 patients with sarcoidosis with a mean age 29.7+/− 8.4 years were evaluated. Six of them were female and 39 were male. The type, distribution and extent of the parameters on HRCT/CTs were evaluated and scored. Chest-X-rays were evaluated for the stage of pulmonary sarcoidosis. Correlations were investigated between HRCT/CT parameter scores, Chest X-Ray stages and pulmonary function parameters.
Results
Nodule, micronodule, ground glass opacity and consolidation were the most common HRCT findings. There were significant correlations between pulmonary function parameters, HRCT pattern scores, and chest-X-ray stages. A significant correlation between chest-x-ray score and total HRCT score was found.
Conclusions
Pulmonary sarcoidosis patients might have various pulmonary parenchymal changes on HRCT. Thorax HRCT was superior to chest-X-ray in detecting pulmonary parenchymal abnormalities. The degree of pulmonary involvement might be closely related to the loss of pulmonary function measured by PFTs. Chest-X-ray is considered to have a role in the evaluation of pulmonary sarcoidosis.
doi:10.1186/2049-6958-8-8
PMCID: PMC3573933  PMID: 23384173
15.  Combination of endobronchial ultrasound-guided transbronchial needle aspiration with standard bronchoscopic techniques for the diagnosis of stage I and stage II pulmonary sarcoidosis 
Respirology (Carlton, Vic.)  2011;16(3):467-472.
Background and objective
Standard bronchoscopic techniques (transbronchial lung biopsy and endobronchial biopsy) provide a diagnosis in 70% of patients with pulmonary sarcoidosis. Previous data suggest that endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) has a high sensitivity in patients with sarcoidosis. The feasibility and utility of combining EBUS-TBNA with standard bronchoscopic techniques is unknown. The aim of this study was to evaluate the feasibility, safety and efficacy of combined EBUS-TBNA and standard bronchoscopic techniques in patients with suspected sarcoidosis and enlarged mediastinal or hilar lymphadenopathy.
Methods
Forty consecutive patients with suspected pulmonary sarcoidosis and enlarged mediastinal or hilar lymph nodes (radiographical stage I and stage II) underwent EBUS-TBNA followed by transbronchial biopsies and endobronchial biopsies under conscious sedation.
Results
Thirty-nine out of 40 patients successfully underwent combined EBUS-TBNA and standard bronchoscopy. Twenty-seven patients were diagnosed with sarcoidosis, eight had tuberculosis, two had reactive lymphadenopathy, two had lymphoma and one had metastatic adenocarcinoma. In patients with sarcoidosis, the sensitivity of EBUS-TBNA for detection of noncaseating granulomas was 85%, compared with a sensitivity of 35% for standard bronchoscopic techniques (P < 0.001). The diagnostic yield of combined EBUS-TBNA and bronchoscopy was 93% (P < 0.0001).
Conclusions
Combination of EBUS-TBNA with standard bronchoscopic techniques is safe and feasible, and optimizes the diagnostic yield in patients with pulmonary sarcoidosis and enlarged intrathoracic lymphadenopathy.
doi:10.1111/j.1440-1843.2011.01933.x
PMCID: PMC3361303  PMID: 21261784
endobronchial ultrasound; mediastinal lymphadenopathy; sarcoidosis; transbronchial biopsy
16.  Usefulness of Endobronchial Ultrasound-Guided Transbronchial Needle Aspiration for Diagnosis of Sarcoidosis 
Yonsei Medical Journal  2013;54(6):1416-1421.
Purpose
Endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) is an accurate and minimally invasive technique used routinely for investigation of mediastinal and hilar lymphadenopathy. However, few studies have addressed its role in comparison to the traditional diagnostic approaches of transbronchial lung biopsy (TBLB), endobronchial biopsy (EBB), and bronchoalveolar lavage (BAL) in the diagnosis of sarcoidosis. We evaluated the usefulness of EBUS-TBNA in the diagnosis of sarcoidosis compared to TBLB, EBB, and BAL.
Materials and Methods
Consecutive patients with suspected sarcoidosis (stage I and II) on chest radiography and chest computed tomography were included. All 33 patients underwent EBUS-TBNA, TBLB, EBB, and BAL during the same session between July 2009 and June 2011. EBUS-TBNA was performed at 71 lymph node stations.
Results
Twenty-nine of 33 patients, were diagnosed with histologically proven sarcoidosis; two patients were compatible with a clinical diagnosis of sarcoidosis during follow-up; and two patients were diagnosed with metastatic carcinoma and reactive lymphadenopathy, respectively. Among 29 patients with histologically proven sarcoidosis in combination with EBUS-TBNA, TBLB, and EBB, only EBUS-TBNA and TBLB revealed noncaseating granuloma in 18 patients and one patient, respectively. The overall diagnostic sensitivities of EBUS-TBNA, TBLB, EBB, and BAL (CD4/CD8 ≥3.5) were 90%, 35%, 6%, and 71%, respectively (p<0.001). The combined diagnostic sensitivity of EBUS-TBNA, TBLB, and EBB was 94%.
Conclusion
EBUS-TBNA was the most sensitive method for diagnosing stage I and II sarcoidosis compared with conventional bronchoscopic procedures. EBUS-TBNA should be considered first for the histopathologic diagnosis of stage I and II sarcoidosis.
doi:10.3349/ymj.2013.54.6.1416
PMCID: PMC3809855  PMID: 24142646
Endobronchial ultrasound; transbronchial needle aspiration; sarcoidosis; mediastinal lymphadenopathy
17.  Unilateral Pulmonary Hilar Tumor Mass: Is It Always Lung Cancer? 
Mædica  2013;8(1):30-33.
ABSTRACT
Sarcoidosis is a multisystem inflammatory disease of unknown etiology, characterized by noncaseating epithelioid cell granulomas. In sarcoidosis, the most common radiological findings are mediastinal and bilateral hilar lymph node enlargement. We present a case of sarcoidosis with a rare radiological aspect of pulmonary hilar tumor mass.
A 54-year-old female patient, active smoker (40 packs/year), with a history of cutaneous lupus, was admitted in our institute for progressive dyspnea and dry cough. At admission physical examination and laboratory tests were normal. Pulmonary function tests diagnosed an obstructive syndrome. Chest X-ray showed a tumor mass of the right pulmonary hilum. Transbronchial biopsy was nondiagnostic. HRCT-scan showed a tumor mass in the right hilum, which raised the suspicion of a lung cancer. PET-CT scan revealed a high metabolic activity of the tumor mass and of a paratracheal right lymphadenopathy. Lymph node biopsy by mediastinoscopy showed noncaseating epithelioid-cell granulomas, sustaining the diagnosis of sarcoidosis. The outcome was favorable, with spontaneous remission without treatment, but with a relapse that responded after systemic corticotherapy.
In conclusion, even if a tumor mass in the pulmonary hilum is highly suggestive of lung cancer, a positive diagnosis should be made only after histological examination, because other benign conditions, like sarcoidosis, could have such an aspect.
PMCID: PMC3749758  PMID: 24023595
sarcoidosis; lung cancer; tumor mass
18.  Acute Posterior Multifocal Placoid Pigment Epitheliopathy as the Initial Manifestation of Sarcoidosis 
Purpose
To report an undiagnosed case of systemic sarcoidosis manifesting with bilateral acute posterior multifocal placoid pigment epitheliopathy (APMPPE).
Case Report
A 26-year-old Caucasian man was referred for management of unilateral visual loss together with a paracentral scotoma developing 2 weeks after a flu-like syndrome. Clinical signs and ancillary diagnostic investigations suggested APMPPE. Laboratory tests demonstrated elevated serum angiotensin converting enzyme and lysozyme levels. Chest CT-scan disclosed moderate hilar lymph node calcifications but QuantiFERON-TB gold test was negative and bronchoalveolar lavage and biopsies were unremarkable. Accessory salivary gland biopsy disclosed epithelioid and gigantocellular granuloma formation without caseum, confirming a diagnosis of sarcoidosis. The fellow eye was involved a few days later and the patient complained of dyspnea. Echocardiography disclosed severe granulomatous myocardial infiltration and high dose corticosteroids and intravenous cyclophosphamide were initiated. Systemic treatment controlled both cardiac and ocular lesions, and was tapered accordingly.
Conclusion
The constellation of “white dot syndromes” and systemic symptoms necessitates a general work-up to exclude granulomatous disorders such as sarcoidosis or tuberculosis. Delayed diagnosis of cardiac sarcoidosis may have life-threatening consequences and the ophthalmologist may be the first physician to diagnose the condition.
PMCID: PMC3306121  PMID: 22454756
APMPPE; Sarcoidosis; Dyspnea; Indocyanine Green Angiography; OCT
19.  The Heerfordt-Waldenström syndrome as an initial presentation of sarcoidosis 
Sarcoidosis is a granulomatous disease of unclear etiology, which commonly presents with cough, dyspnea, chest pain, fever, weight loss, arthralgias, and erythema nodosum. Heerfordt-Waldenström syndrome, a rare presentation of sarcoidosis, is characterized by the presence of parotid gland enlargement, facial palsy, anterior uveitis, and fever. Here we present a case of a 59-year-old nonsmoking African American woman who presented with 3 days of progressively worsening left facial droop, difficulty swallowing, and blurred vision. Over the prior 4 months, she had had a productive cough, fevers, night sweats, and an unintentional 30-pound weight loss. Physical examination revealed a left facial droop involving the forehead, cheek, and chin with an inability to close the left eyelid. Her serum angiotensin-converting enzyme level was twice the upper limit of normal. Prominent hilar markings were identified on chest x-ray, but no focal opacity was seen. Fine-needle aspiration of a preauricular lymph node revealed noncaseating granulomas consistent with granulomatous lymphangitis. The patient was given a diagnosis of Heerfordt-Waldenström syndrome, or uveoparotid fever. Treatment with a high-dose steroid improved her parotid gland enlargement, facial palsy, and anterior uveitis.
PMCID: PMC3777100  PMID: 24082416
20.  Interferon-inducible chemokines reflect severity and progression in sarcoidosis 
Respiratory Research  2013;14(1):121.
Background
Identification of serum proteins that track with disease course in sarcoidosis may have clinical and pathologic importance. We previously identified up-regulated transcripts for interferon-inducible chemokines CXCL9, and CXCL10, in blood of sarcoidosis patients compared to controls. The objective of this study was to determine whether proteins encoded by these transcripts were elevated in serum and identified patients with remitting vs. chronic progressive sarcoidosis longitudinally.
Methods
Serum levels of CXCL9, CXCL10, and proteins associated with inflammation and/or disease activity (sIL2R, ACE, ESR and CRP) were measured in a prospective cohort of sarcoidosis subjects and controls. Comparisons were made between groups and clinical course using pulmonary function measures and a severity score developed by Wasfi et al.
Results
In a cross-sectional analysis of 36 non-immunosuppressed sarcoidosis subjects, serum CXCL9, CXCL10, and sIL2R were significantly elevated compared to 46 controls (p < 0.0001). CXCL9 and CXCL10 were strongly inter-correlated (p = 0.0009). CXCL10 and CXCL9 were inversely correlated with FVC% predicted and DLCO% predicted, respectively. CXCL10 and CXCL9 significantly correlated with sarcoidosis severity score. sIL2R, ESR, CRP, and ACE serum levels did not correlate with pulmonary function measures or severity score. In the longitudinal analysis of 26 subjects, changes in serum CXCL10 level over time corresponded with progression versus remission of disease.
Conclusions
Interferon-γ–inducible chemokines, CXCL9 and CXCL10, are elevated in sarcoidosis and inter-correlated with each other. Chemokine levels correlated with measures of disease severity. Serial measurements of CXCL10 corresponded to clinical course.
doi:10.1186/1465-9921-14-121
PMCID: PMC4176097  PMID: 24199653
Sarcoidosis; CXCL10; CXCL9; Interferon inducible chemokines
21.  Prevalence of Echocardiographic Features Suggesting Cardiac Sarcoidosis in Patients With Pacemaker or Implantable Cardiac Defibrillator 
Korean Circulation Journal  2011;41(6):313-320.
Background and Objectives
Basal septal thinning or localized aneurysmal dilatation without coronary artery disease has been described as a characteristic finding suggestive of cardiac sarcoidosis. We sought to assess the prevalence of this characteristic echocardiographic finding in patients with pacemaker (PM) or implantable cardiac defibrillator (ICD).
Subjects and Methods
Echocardiography of patients who received PM or ICD were retrospectively analyzed. Patients with marked thinning and akinesia confined to the basal septum (type 1), or posterolateral wall resulting in localized aneurysmal outward bulging (type 2) without history of myocardial infarction or significant coronary stenosis were included for analysis.
Results
Among 1,357 consecutive patients, 21 exhibited suggestive echocardiographic findings (type 1/2=15/6) with a mean ejection fraction of 37±11%. The prevalence was 1.2% in the PM group and 4.0% in the ICD group. Only 3 patients showed histologically confirmable sarcoidosis in lymph nodes, lung and heart, respectively. Endomyocardial biopsy was attempted in 6 patients, but failed to demonstrate sarcoidosis. The 1-, 2-, 4- and 6-year clinical events (death, cardiac transplantation and hospital admission)-free survival rates were 100%, 85.7±7.6%, 75.0±9.7% and 48.6±12.4%, respectively. During follow-up, two patients with PM underwent ICD implantation, and another underwent heart transplantation.
Conclusion
Prevalence of echocardiographic features suggesting prevalence of cardiac sarcoidosis is low in patients who underwent device implantation. However, considering the very low yield of endomyocardial biopsy and the rare extracardiac manifestations in cardiac sarcoidosis, characteristic echocardiographic findings could be an adjunctive diagnostic criterion in these patients.
doi:10.4070/kcj.2011.41.6.313
PMCID: PMC3132693  PMID: 21779284
Sarcoidosis; Echocardiography; Pacemaker; Implantable cardioverter-defibrillators
22.  Pulmonary thin-section CT findings in acute Moraxella catarrhalis pulmonary infection 
The British Journal of Radiology  2011;84(1008):1109-1114.
Objective
Moraxella catarrhalis is an important pathogen in the exacerbation of chronic obstructive pulmonary disease. The aim of this study was to assess the clinical and pulmonary thin-section CT findings in patients with acute M. catarrhalis pulmonary infection.
Methods
Thin-section CT scans obtained between January 2004 and March 2009 from 292 patients with acute M. catarrhalis pulmonary infection were retrospectively evaluated. Clinical and pulmonary CT findings in the patients were assessed. Patients with concurrent infection including Streptococcus pneumoniae (n = 72), Haemophilus influenzae (n = 61) or multiple pathogens were excluded from this study.
Results
The study group comprised 109 patients (66 male, 43 female; age range 28–102 years; mean age 74.9 years). Among the 109 patients, 34 had community-acquired and 75 had nosocomial infections. Underlying diseases included pulmonary emphysema (n = 74), cardiovascular disease (n = 44) or malignant disease (n = 41). Abnormal findings were seen on CT scans in all patients and included ground-glass opacity (n = 99), bronchial wall thickening (n = 85) and centrilobular nodules (n = 79). These abnormalities were predominantly seen in the peripheral lung parenchyma (n = 99). Pleural effusion was found in eight patients. No patients had mediastinal and/or hilar lymph node enlargement.
Conclusions
M. catarrhalis pulmonary infection was observed in elderly patients, often in combination with pulmonary emphysema. CT manifestations of infection were mainly ground-glass opacity, bronchial wall thickening and centilobular nodules.
doi:10.1259/bjr/42762966
PMCID: PMC3473838  PMID: 21123308
23.  Sarcoidois: is it only a mimicker of primary rheumatic disease? A single center experience 
Background:
Sarcoidosis is known as a T helper 1 lymphocyte (Th1-Ly) mediated disease which can imitate or sometimes accompany many primary rheumatic diseases. The purpose of this study is to share the clinical, demographic and laboratory data of patients presenting with rheumatologic manifestations and diagnosed with sarcoidosis.
Methods:
A total of 42 patients (10 men) were included in the study. The patients were admitted to the rheumatology outpatient clinic for the first time with different rheumatic complaints between November 2011 and May 2013 and were diagnosed with sarcoidosis after relevant tests. Clinical, demographic, laboratory, radiological and histological data of these patients were collected during the 18-month follow-up period and then analyzed.
Results
Mean patient age was 45.2 years (20–70 years) and mean duration of disease was 3.5 years (1 month–25 years). Evaluation of system and organ involvement revealed that 20 (47.6%) patients had erythema nodosum, 3 (7.1%) had uveitis, 1 (2.3%) had myositis, 1 (2.3%) had neurosarcoidosis, 32 (76.2%) had arthritis and 40 (95.2%) had arthralgia. Of the 32 patients with arthritis, 28 (87.5%) had involvement of the ankle, 3 (9.4%) had involvement of the knee and 1 (3.2%) had involvement of the wrist. No patient had cardiac involvement. Thoracic computed tomography scan showed stage 1, 2, 3 and 4 sarcoidosis in 12 (28.5%), 22 (52.4%), 4 (9.5%) and 4 (9.5%) patients, respectively. Histopathology of sarcoidosis was verified by endobronchial ultrasound, mediastinoscopy and skin and axillary biopsy of lymphadenopathies, which revealed noncaseating granulomas. Laboratory tests showed elevated serum angiotensin-converting enzyme in 15 (35.7%) patients, elevated serum calcium level in 6 (14.2%) patients and elevated serum 1,25-dihydroxyvitamin D concentrations in 2 (4.7%) patients. Serological tests showed antinuclear antibody positivity in 12 (28.5%) patients, rheumatoid factor positivity in 7 (16.6%) patients and anticyclic citrullinated antibody positivity in 2 (4.8%) patients.
Conclusion:
Sarcoidosis can imitate or accompany many primary rheumatic diseases. Sarcoidosis should be considered not simply as an imitator but as a primary rheumatic pathology mediated by Th1-Ly. New studies are warranted on this subject.
doi:10.1177/1759720X13511197
PMCID: PMC3897166  PMID: 24489610
primary rheumatic disease; rheumatologic manifestations; sarcoidosis
24.  Rare coexistence of sarcoidosis and lung adenocarcinoma 
Case
An eighty year old African-American female was evaluated for cough, chest pain, asymptomatic anemia and 21 pound weight loss over a six month period. Computerized tomography (CT) revealed a spiculated 2.8 cm right upper lobe lung nodule, other smaller nodules and lymphadenopathy. Gallium scan revealed abnormal uptake of radiotracer in lacrimal, hilar and mediastinal glands. Broncho-alveolar lavage showed CD4/CD8 ratio of 2:1 with 15% lymphocytes. Biopsy of right upper lobe lesion and mediastinoscopic lymph node biopsy showed numerous matured uniform non-caseating granulomatous inflammation, however stains and culture for Acid fast bacilli (AFB)/fungal organisms were negative. Patient improved on oral steroids. Six months later she returned with worsening dyspnea and chest X-ray showed bilateral pleural effusions. Thoracocentesis revealed Thyroid transcription factor 1 (TTF1) positive adenocarcinoma cells and Video assisted thoracic surgery (VATS) procedure revealed numerous pleural, pericardial, diaphragmatic metastasis. Biopsy also was positive for TTF1 adenocarcinoma and positive for Epidermal Growth Factor receptor (EGFR) mutation, however negative for Anaplastic Lymphoma Kinase (ALK). Talc pleurodesis was performed. She was treated with erlotinib while steroid was kept on hold. Initial tumor burden decreased but follow-up PET scan six months later showed progression of tumor with lymphadenopathy. After discussion with patient and family, patient opted for hospice care.
Discussion
Oncocentric theory postulates sarcoidosis as an immunological reaction to dispersal of tumor antigen. Sarcocentric theory postulates that cell-mediated immune abnormalities induced by sarcoidosis in CD4 and CD8 cells is involved in the onset of lung cancer. Thus considerable controversy exists regarding sarcoidosis and malignancy. In our case, TTF1 adenocarcinoma cells from thoracocentesis suggest peripheral nodules in right upper lobe and lingula were likely metastatic, presenting as malignant pleural effusions. However if noncaseating granulomatous inflammation is expected as an immunological reaction to tumor antigen, it is very interesting to observe that initial tissue biopsy of primary right upper lobe mass and mediastinal lymph nodes showed matured uniform non-caseating granulomatous inflammation and no evidence of adenocarcinoma. This being said, it would be highly unlikely for sarcoidosis to progress to lung adenocarcinoma within six months. This adds further controversy to whether granulomatous inflammation is a precursor to future malignancy or whether this elderly African-American female was predisposed to develop granulomatous inflammation in presence of a tumor antigen. One can also speculate whether repeat tissue sampling from right upper lobe mass would have shown granulomatous inflammation or TTF1 adenocarcinoma.
Conclusion
While evidence is still lacking regarding association between sarcoidosis and lung adenocarcinoma, it is important for clinicians to exclude metastatic malignancy in patients exhibiting clinical and radiographic findings consistent with sarcoidosis.
doi:10.1016/j.rmcr.2013.12.008
PMCID: PMC4061445
Sarcoidosis; Lung adenocarcinoma; Non-caseating granulomas; TTF-1 mutation; Tumor
25.  Pulmonary granulomatous reaction: talc pneumoconiosis or chronic sarcoidosis? 
A chronic pulmonary granulomatous reaction was associated with an almost identical clinical picture in two patients exposed to talc. In both patients lung biopsy showed the deposition of talc particles and a heavy granulomatous reaction. At the time of diagnosis the Kveim test result was negative in both patients, urinary calcium excretion was normal, and there were no extrapulmonary manifestations and no response to steroid treatment. These findings point against sarcoidosis. The serum angiotensin-converting enzyme level, however, was raised in both patients. It was concluded that the patient who was exposed to talc in the rubber industry had a true talc pneumoconiosis. The other patient, who was exposed to cosmetic talcum powder, suffered from chronic sarcoidosis with talc deposition in the lungs, since an enlarged axillar lymph node containing granulomatous inflammation was discovered after two years' follow up. These cases show that it may be extremely difficult to differentiate between chronic sarcoidosis and talc pneumoconiosis even after careful clinical and histological analysis.
Images
PMCID: PMC1009240  PMID: 6691939

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