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1.  Sarcoidosis Presenting with Massive Pleural Effusion and Elevated Serum and Pleural Fluid Carbohydrate Antigen-125 Levels 
A 55-year-old woman was admitted for an elevated serum carbohydrate antigen-125 (CA-125) level, and a left pleural effusion, which were detected at a routine health examination. Computed tomography of the chest was performed upon admission, revealing extensive bilateral paratracheal and mediastinal lymph node enlargement with a massive left-sided pleural effusion. Subsequent analysis of the pleural fluid demonstrated consistency with an exudate, no evidence of malignant cells, and a normal adenosine deaminase. However, the pleural fluid and serum CA-125 levels were 2,846.8 U/mL and 229.5 U/mL, respectively. A positron emission tomography did not reveal any primary focus of malignancy. Finally, a surgical mediastinoscopic biopsy of several mediastinal lymph nodes was performed, revealing non-necrotizing granulomas, consistent with sarcoidosis. After a month of treatment of prednisolone, the left pleural effusion had resolved, and after 2 months the serum CA-125 level was normalized.
doi:10.4046/trd.2012.73.6.320
PMCID: PMC3538185  PMID: 23319994
Sarcoidosis; Pleural Effusion; CA-125 Antigen
2.  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
3.  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
4.  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.
5.  Comparison of cutaneous sarcoidosis with systemic sarcoidosis: a retrospective analysis 
Objective: To investigate clinical characteristics and treatment effect of sarcoidosis with cutaneous lesions in Chinese patients, and to compare them with previous works. Methods: Retrospective analysis was conducted based on clinical manifestations, systemic examinations and treatment of biopsy-proved 36 patients with sarcoidosis with cutaneous lesions in our hospital since 2000. Patients were divided into cutaneous sarcoidosis (CS) group without systemic involvement and systemic sarcoidosis (SS) group with systemic involvement according to whether extracutaneous systems were involved. Results: Male to female ratio was 1:4.1 in total 36 patients. Average age of onset was (43.6±15.8) years old in CS group and (54.4±11.5) years old in SS group. The most common cutaneous lesions were papulonodules (41.7%) and frequently found in limbs (61.1%). There were 26 patients in SS group, and lung was the most common organ with systemic involvement, followed by lymph nodes. In SS group, elevation of inflammatory parameters and evident changes of chest radiologic examination were often observed. 72.2% patients were treated with glucocorticoid and the overall therapeutic efficacy rate was 48.4%. The therapeutic efficacy in CS group (80%) was significantly higher than SS group (33.3%). Papulonodules type had better response to therapy and usually resolved after treatment. Lupus pernio type was resistant to treatment. Conclusion: Sarcoidosis occurs more frequently in females. Lung is the most commonly affected extracutaneous organ in SS patients. CS patients have better response to therapy than SS patients. Types of cutaneous lesions and existence of systemic involvement are related to prognosis of cutaneous lesions.
PMCID: PMC3885493  PMID: 24427359
Cutaneous sarcoidosis; systemic sarcoidosis; prognosis
6.  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
7.  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
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.  Fuchs' heterochromic uveitis and sarcoidosis. 
The British Journal of Ophthalmology  1995;79(11):1021-1023.
AIMS/BACKGROUND--The aetiology of Fuchs' heterochromic uveitis (FHU) is unknown although it can occur in combination with a number of different ocular conditions. Five patients with FHU who show an association with sarcoidosis were studied. METHODS--Four patients with clinical signs compatible with FHU who had elevated serum angiotensin converting enzyme levels (sACE), and a fifth case with a normal sACE and a positive Kveim test were described. RESULTS--All five cases had iris nodules, two later developed mutton fat keratic precipitates, and one had peripheral retinal periphlebitis. Of the four cases with elevated sACE, one had respiratory function test abnormalities and an abnormal chest x ray compatible with pulmonary sarcoidosis. Another had a chorioretinal scar and developed intermediate uveitis 2 years after presentation. CONCLUSIONS--In all of these cases a diagnosis of FHU may represent a specific secondary ocular response to sarcoidosis rather than a primary idiopathic uveitis syndrome. Although FHU remains a clinical diagnosis, routine uveitis investigations should still be performed in this group of patients.
PMCID: PMC505321  PMID: 8534647
10.  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
11.  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
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.  The late stages of pulmonary sarcoidosis 
Postgraduate Medical Journal  1970;46(538):530-536.
Pulmonary sarcoidosis is frequently preceded by bilateral hilar lymph-node enlargement (BHL). Although it is normally difficult to be certain in a patient without BHL that a stage of BHL has not in fact occurred in the past but been missed, there is good evidence that some cases of pulmonary sarcoidosis arise without preceding BHL. Although a prefibrotic infiltration of the lungs will usually show signs either of resolution or of developing fibrosis within 2 years, such an infiltration may remain apparently non-progressive, producing little functional defect, for much longer than this, and in such a case, substantial resolution remains possible. A high proportion of those who develop fibrosis show a characteristic pattern of strand-like fibrosis in the middle zones and lower part of the upper zones of the lungs, with emphysematous changes above and below. In densely fibrotic parts of the lungs, cavities may appear, probably from necrosis at the centres of large masses of hyaline fibrosis. A rare complication is the development of a ‘fungus ball’ due to the growth of Aspergillus in such a cavity.
Occasionally, fine focal fibrosis develops without distortion of lung architecture, but with severe disability.
A rare but important event is the development of bronchial stenoses. These may affect main, segmental or subsegmental bronchi, more especially the proximal parts of segmental or subsegmental bronchi.
Calcification has been observed to develop in a characteristic symmetrical fashion in the hilar lymphnodes and occasionally in small foci in the lungs in about 5% of patients with pulmonary sarcoidosis who initially had BHL, followed for periods ranging from 5 to 20 years.
In the late stages of sarcoid fibrosis of the lungs, the specific granuloma may be no longer convincingly demonstrable.
Images
PMCID: PMC2467273  PMID: 5481107
14.  B lymphocyte accumulations in human pulmonary sarcoidosis. 
Thorax  1992;47(11):964-967.
BACKGROUND: Although cell mediated immunity is primarily thought to mediate the pathogenesis of sarcoidosis, the presence of immunoglobulins, immune complexes and complement suggests that processes of humoral immunity may contribute to immunopathology in sarcoid lesions. To test this hypothesis, the distribution of B lymphocytes in paraffin embedded sarcoid lesions in mediastinal lymph nodes and open lung biopsy specimens was investigated. METHODS: Paraffin sections from eight open lung and 21 lymph node biopsies from sarcoid patients and five normal and five tuberculous lymph nodes from patients with tuberculosis were stained with a panel of monoclonal antibodies by means of avidin/biotin enhanced immunocytochemistry. RESULTS: Immunohistochemical analysis of the 29 biopsy specimens from the sarcoid patients revealed large numbers of B cells in the intergranulomatous regions. Further investigations in the open lung biopsy specimens indicated that these B cells were often organised into discrete circular or oval shaped aggregates with no germinal centre morphology, in which a few CD45RO memory T lymphocytes were scattered. The B cells were polyclonal, and a few plasma cells (IgM+, IgA+, IgG+) were identified. CONCLUSIONS: The finding of large numbers of B lymphocytes in sarcoid pulmonary lesions is in contrast to bronchoalveolar lavage studies, which have demonstrated proportions of 5% or less of B cells as a total of all immune cells, and therefore indicates that bronchoalveolar lavage may not correctly sample the immune cells of lung interstitial tissue in pulmonary sarcoidosis. The B cells at these sites are the possible origin of some of the humoral changes in the serum and lesions of sarcoid patients. They may also influence the pathogenesis of the disorder by presenting antigen(s) and forming immune complexes at sites of disease activity.
Images
PMCID: PMC464115  PMID: 1465757
15.  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
16.  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
17.  Annular lesion of the lung in sarcoidosis. 
Thorax  1989;44(9):756-757.
A 20 year old man presented with eight thin walled, ring shaped shadows, 1-3 cm in diameter, on a chest radiograph. Pulmonary sarcoidosis was diagnosed on the basis of histopathological examination of a cervical lymph node, transbronchial lung biopsy, and increased activity of angiotensin converting enzyme and lysozyme in serum. The lesions disappeared within six months of his starting corticosteroid treatment.
Images
PMCID: PMC462060  PMID: 2588213
18.  Cardiac Sarcoidosis Culminating in Severe Biventricular Failure 
Case Reports in Medicine  2009;2009:856785.
A 59-year-old woman with a history of lung sarcoidosis developed general edema and exertional dyspnea. An electrocardiogram showed first-degree atrioventricular block with complete right bundle branch block. Chest X-ray showed cardiomegaly. Echocardiography showed diffuse and severe hypokinesis of the left ventricle (LV) and biventricular enlargement with severe tricuspid regurgitation. Myocardial scintigraphy disclosed a perfusion defect at the ventricular septum and hypoperfusion at the posterior wall and the apex. On cardiac catheterization, pulmonary capillary wedge pressure, right ventricular, and right atrial pressures were elevated. Coronary angiograms were normal. Myocardial biopsy of the right ventricle histologically revealed epithelioid cell granuloma with infiltration of fibrous cells. The patient's symptom and LV function were improved with conventional medical therapy for heart failure. This is a rare case of cardiac sarcoidosis resulting in biventricular failure.
doi:10.1155/2009/856785
PMCID: PMC2738866  PMID: 19746177
19.  Sarcoidosis Presenting as Löfgren's Syndrome with Myopathy 
Case Reports in Rheumatology  2013;2013:125251.
A 34-year-old female patient, who had proximal muscle weakness for 8 months, presented with erythema nodosum lesions on the pretibial region in addition to pain, swelling, and movement restriction in both ankles for the last one month. Thoracic CT demonstrated hilar and mediastinal lymphadenopathy. She underwent mediastinoscopic lymph node biopsy; biopsy result was consistent with noncaseating granuloma. Serum angiotensin converting enzyme level and muscle enzymes have been elevated. Muscular MRI and EMG findings were consistent with myositis. Muscle biopsy was done, and myopathy was found. The patient was diagnosed with sarcoidosis, Löfgren's syndrome, and sarcoid myopathy. The patient displayed remarkable clinical and radiological regression after 6-month corticosteroid and MTX therapy.
doi:10.1155/2013/125251
PMCID: PMC3638518  PMID: 23691415
20.  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
21.  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
22.  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
23.  Extensive Systemic Sarcoidosis with Testicular Involvement Mimicking Metastatic Testicular Cancer 
Korean Journal of Urology  2011;52(4):295-297.
Sarcoidosis is an idiopathic, multisystem disease that rarely involves the genitourinary tract. Here we present an unusual case of testicular sarcoidosis with extensive lymphadenopathy that mimicked a metastatic testicular tumor. A 27-year-old male presented with a palpable right testicular mass accompanied by multiple palpable inguinal lymph nodes. The scrotal ultrasound showed a hypoechoic lesion at the inferior portion of the right testis. Extensive enlarged lymph nodes were noted in multiple areas on the abdominal computed tomography. Preoperative testicular tumor markers were within the normal range. Exploration of the right testis with a frozen section analysis of the right testicular mass and of a palpable right inguinal lymph node showed granulomatous inflammation. The testis was salvaged and the final pathological diagnosis was sarcoidosis. Treatment with high-dose corticosteroids resulted in complete resolution of the intratesticular mass and a significant decrease in the extent of the lymphadenopathy.
doi:10.4111/kju.2011.52.4.295
PMCID: PMC3085626  PMID: 21556220
Lymphatic diseases; Sarcoidosis; Steroids; Testicular neoplasms; Testis
24.  Clinical characteristics and computed tomography findings in Arab patients diagnosed with pulmonary sarcoidosis 
Annals of Saudi Medicine  2009;29(6):454-459.
BACKGROUND AND OBJECTIVE:
Sarcoidosis is prevalent worldwide with significant heterogeneity across different ethnic groups. We aimed To describe the clinical characteristics and computed tomography findings among Arab patients with pulmonary sarcoidosis.
METHODS:
A retrospective study of patient demographics, symptoms, co-morbid illness, sarcoidosis stage, treatment, pulmonary function and CT results.
RESULTS:
Of 104 patients, most (77%) were 40 years of age or older at diagnosis, and females in this category (≥40 years) significantly outnumbered male patients (69/104 (66.3%) vs. 35/104 (33.7%), P=.003). The most common complaints were dyspnea (76%), cough (72.1%) and weight loss (32.7%). The majority of patients displayed impairment in lung function parameters at presentation. However, significant impairment in forced vital capacity, percentage predicted (FVC%) (<50%) was present in only 17% of patients. The most frequent CT finding was mediastinal lymph node enlargement in 49 patients (73.1%). Parenchymal abnormalities indicating lung fibrosis were noted in 31 patients (46.3%), and traction bronchiectasis was the most common (35.8%) fibrotic pattern detected on CT scans.
CONCLUSION:
At presentation, clinical manifestations of sarcoidosis among this sample of Arab patients were similar to reports from other nations. Further studies are needed to explore the effects of race and ethnicity on disease severity in the Middle East.
doi:10.4103/0256-4947.57168
PMCID: PMC2881433  PMID: 19847083
25.  Evidence for abnormal regulation of circulating 1 alpha,25-dihydroxyvitamin D in patients with sarcoidosis and normal calcium metabolism. 
Journal of Clinical Investigation  1980;66(4):852-855.
The effects of vitamin D, 2.5 mg (100,000 U)/d for 4 d, on serum calcium, serum 25-hydroxyvitamin D (25-OHD), and serum 1 alpha,25-dihydroxyvitamin D [1 alpha,25(OH)2D] were compared in 17 normal subjects and 6 patients with sarcoidosis who had normocalcemia and no history of hypercalcemia. The diagnosis was confirmed histologically in each of them. Vitamin D increased mean serum 25-PHD from 30 +/- 4 to 99 +/- 15 ng/ml (P < 0.001) and did not change mean serum 1 alpha,25(OH)2D (32 +/- 3 vs. 29 +/- 3 pg/ml) or mean serum calcium (9.5 +/- 0.1 vs. 9.6 +/- 0.1 mg/dl) in the normal subjects. In contrast, vitamin D increased mean serum 25-OHD from 19 +/- 3 to 65 +/- 19 ng/ml (p < 0.05), increased mean serum 1 alpha,25(OH)2D threefold from 40 +/- 7 to 120 +/- 24 pg/ml, and increased mean serum calcium from 9.4 +/- 0.2 to 9.8 +/- 0.2 mg/dl (P < 0.01). There was a significant positive correlation between the serum 1 alpha,25(OH)2D and serum calcium in these individuals (r = 0.663, P < 0.01) but not in the normal subjects. The results (a) provide further evidence for abnormal regulation of circulating 1 alpha,25(OH)2D in sarcoidosis and (b) indicate that the abnormality may exist in patients with normal calcium metabolism. Thus, the defect in vitamin D metabolism in sarcoid apparently is more common than was previously recognized.
PMCID: PMC371661  PMID: 7419722

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