Neonatal Lupus is a rare disease, described in 1954, which only occurs in children of mothers with antibodies to specific antigens of Systemic Lupus Erythematosus (SLE). Such antigens pass, transplacentally during gestation and is characterized by cutaneous manifestations such as rash, erythematous macules, papules or plaques which tend to coalesce; less frequent are blood disorders including aplastic anemia, neutropenia, thrombocytopenia, hemolytic anemia, pancytopenia; liver disorders with elevated liver enzymes and cholestasis; central nervous system disease such as mielopaias, convulsions; pulmonary as pneumonitis, and gastrointestinal tract such as bloody diarrhea. Alopecia is a common symptom in SLE but has not been reported in the literature in neonatal presentation.
Case report: A 1 month and 4 day old infant female, who presented 3 days after birth with a persistent bloody diarrhea, without mucus also presents dehydration and metabolic acidosis that warrant intravenous correction. An infectious etiology is discarded and is referred to an allergist for study of a possible lactose intolerance which is discarded initially. However, the physical finding of hair loss is evident with areas of alopecia which together with the persistence of the diarrhea and rash is suspect of a possible immunological etiology. Therefore, it was decided to test Anti-Ro autoimmunity in the infant and his mother; given a positive results.
With the Anti-Ro test the presence of Neonatal Lupus is confirmed. A treatment with EV-dose methylprednisolone was initiated, which had little clinical response, meriting a treatment with azathioprine with a good clinical response, which improved and reduced Anti-Ro values.
The importance of clinical observation is evident when there are unusual features which allows for a rare diagnosis. This striking case, given the unusual presentation with alopecia which together with the clinical observations was indicative of this disease, and not of other syndromes characteristic of this age, like sepsis.
Two children with lupus erythematosus and myelopathy were studied. The first child developed the neonatal lupus erythematosus syndrome associated with transplacentally acquired anti-Ro/SSA antibodies. The cutaneous manifestations of neonatal lupus erythematosus disappeared but a residual myelopathy was confirmed at 16 months of age. The second child developed cutaneous lupus erythematosus at 3 months of age associated with a total deficiency of the Clr component of complement. A myelopathy and mesangial glomerulonephritis developed at 2 years of age which required treatment with corticosteroids. These two children with CNS lupus erythematosus, one associated with transplacentally acquired antibodies and the other associated with a complement deficiency, may suggest an immune-mediated mechanism for the pathogenesis of myelopathy in childhood lupus erythematosus.
The ninth column on Evidence-Based Behavioral Medicine is a synthesis of a recent systematic meta-review of multiple health behavior change (MHBC) interventions published by Prochaska and Prochaska in the American Journal of Lifestyle Medicine (Am J Life Med 5:208–221, 2011). Health risk behaviors are highly prevalent and increase the risk of developing and exacerbating chronic disease. The purpose of the meta-review was to examine the efficacy of MHBC interventions in a variety of populations and settings. The available literature was synthesized into three health behavior domains including energy-balance behaviors (physical activity and nutrition), addictive behaviors, and disease-related prevention. Twelve systematic reviews were identified that summarized more than 150 randomized clinical trials. Findings suggest that: (1) Physical activity and nutrition interventions are effective in producing weight loss among adults and female youth, (2) treating two addictive behaviors produces a higher long-term abstinence rate than treating a single behavior, and (3) although preventive interventions for cardiovascular disease and cancer significantly reduce health risk behaviors, reductions in disease incidence are yet to be demonstrated.
Multiple risk; Behavior change; Risk behavior; Lifestyle change; Primary prevention
In a recent article, Lund et al sought to compare maternal and neonatal outcomes of various treatment regimens for opioid dependence during pregnancy.1 In their background, discussion the authors state that “In the United States buprenorphine plus naloxone [Suboxone®] … has been the preferred form of prescribed buprenorphine due to its reduced abuse liability relative to buprenorphine alone [Subutex®].” This claim is certainly consistent with the view of the firm that has manufactured and sold both products, Reckitt Benckiser. In September of 2011, the company announced that it was “… discontinuing distribution and sale of Subutex® tablets as we believe that mono product (product containing buprenorphine alone with no naloxone) creates a greater risk of misuse, abuse and diversion …”.2 Supporting evidence for the alleged “reduced abuse liability” appears to be lacking, however, and evidence cannot be located in the two references cited by Dr. Lund and his co-authors, which in fact are silent on the subject of abuse potential.3,4 In contrast, it has been reported that the transition to buprenorphine/naloxone from the mono formulation has been associated with “… no reduction in injection risk behaviors among IDUs.”5
If evaluation of economic evidence is to be used increasingly in Saudi Arabia, a review of the published literature would be useful to inform policy decision-makers of the current state of research and plan future research agendas. The purpose of this paper is to provide a critical review of the state of health economic evaluation research within the Saudi context with regard to the number, characteristics, and quality of published articles.
A literature search was conducted on May 8, 2011 to identify health economic articles pertaining to Saudi Arabia in the PubMed, Embase, and EconLit databases, using the following terms alone or in combination: “cost*”, “economics”, “health economics”, “cost-effectiveness”, “cost-benefit”, “cost minimization”, “cost utility analysis”, and “Saudi”. Reference lists of the articles identified were also searched for further articles. The tables of contents of the Saudi Pharmaceutical Journal and the Saudi Medical Journal were reviewed for the previous 5 years.
The search identified 535 citations. Based on a reading of abstracts and titles, 477 papers were excluded. Upon reviewing the full text of the remaining 58 papers, 43 were excluded. Fifteen papers were included. Ten were categorized as full economic evaluations and five as partial economic evaluations. These articles were published between 1997 and 2010. The majority of the studies identified did not clearly state the perspective of their evaluation. There are many concerns about the methods used to collect outcome and costs data. Only one study used some sort of sensitivity analysis to assess the effects of uncertainty on the robustness of its conclusions.
This review highlights major flaws in the design, analysis, and reporting of the identified economic analyses. Such deficiencies mean that the local economic evidence available to decision-makers is not very useful. Thus, building research capability in health economics is warranted.
cost-effective analysis; pharmacoeconomics; economic evaluation; quality assessment; Saudi Arabia
Prolidase deficiency (PD) is a rare autosomal recessive disorder which may have a wide spectrum of clinical features. These features include a characteristic facies, cognitive impairment, rashes or skin ulceration, splenomegaly, recurrent infections involving mainly the respiratory system, and iminodipeptiduria. The disorder is caused by a mutation in the PEPD gene.
To describe a cohort of unrelated PD patients from Northern Israel whose inborn error of metabolism was associated with systemic lupus erythematosus (SLE) and to identify in the medical literature all PD cases mimicked by and/or associated with SLE.
Three patients with PD associated with SLE were clinically, biochemically and genetically investigated. These patients were from 3 unrelated consanguineous families residing in Northern Israel. A computer-assisted (PubMed) search of the medical literature from 1975 to 2011 was performed using the following key words: Prolidase deficiency, SLE, and systemic lupus erythematosus.
An association between PD and SLE was found in 10 PD patients. These 10 patients included three from our cohort of 23 PD patients, and seven out of just under 70 PD patients previously reported in the literature.
The present findings underscore the relatively high incidence of the association between SLE and PD, suggesting that this association may not be coincidental. The phenotypic similarities between SLE and PD might suggest that the PEPD gene constitutes a modifier gene or a genetic risk factor in the causation of SLE.
To review best practices for early recognition and treatment of conditions resulting in neonatal cholestasis, in order to improve long-term outcomes for affected infants.
QUALITY OF EVIDENCE
Studies, review articles, and meta-analyses pertaining to neonatal-onset cholestasis were sought via electronic databases. Reference lists of studies and review articles supplemented the electronic search. Studies were included if they examined the importance of early diagnosis and intervention for cholestatic jaundice of any cause, and mainly comprised Level II and Level III evidence.
Review of the relevant literature supports the recommendation that infants with jaundice at 2 weeks of age should be tested for cholestasis by quantifying the direct reacting bilirubin levels in their blood. Subsequent rapid investigation using a diagnostic algorithm enables early diagnosis of the specific cause and facilitates timely intervention for conditions whose outcomes are improved by early treatment.
Universal screening for neonatal cholestasis might help with early identification of cases and improve outcomes, although further study is required in the North American setting.
Maternal vitamin D deficiency is not uncommon. The lack of vitamin D during pregnancy may result in poor fetal growth and altered neonatal development that may persist into later life. Recognition of risk factors and early detection of vitamin D deficiency during pregnancy is important in order to prevent neonatal vitamin D deficiency and related complications. The aim of the current study is to assess the effect of maternal vitamin D status on the neonatal vitamin D stores. A total of 92 pregnant women at the end of the 3rd trimester and their newborns were recruited from Al Khafji Joint Operation Hospital, Saudi Arabia, during the year 2011. Maternal and cord blood samples were taken for determination of serum levels of circulating 25-hydroxyvitamin D3 [25(OH)D3] concentration, serum calcium (Ca++), phosphorus (PO4) and alkaline phosphatase (ALP). Compared with pregnant women with adequate vitamin D levels, women deficient in vitamin D had infants with vitamin D deficiency (X±SD 33.44±18.33 nmol/L vs 55.39±17.37 nmol/L, P=0.01). Maternal and neonatal serum 25(OH)D3 levels showed a positive correlation with serum Ca++ and negative correlation with serum PO4 and ALP. Neonatal 25(OH)D was related to maternal 3rd trimester levels (r=0.89, P=0.01). The newborn serum 25(OH)D3 concentrations rely on maternal vitamin D status. Poor maternal vitamin D status may adversely affect neonatal vitamin D status and, consequently, calcium homeostasis.
maternal vitamin D; neonatal 25-hydroxyvitamin D3; calcium homeostasis.
Neonatal lupus erythematosus is a rare autoimmune disease caused by transplacental passage of maternal autoantibodies against Ro/SS-A, La/SS-B, and U1-ribonucleoprotein. The clinical spectrum of neonatal lupus erythematosus comprises cutaneous, cardiac, and systemic abnormalities. Typical cutaneous manifestations include annular erythematous plaques with or without fine scale predominately on the scalp, neck, or face and less commonly on the trunk and extremities. The authors describe the case of a two-month-old male child who developed atypical, targetoid-like lesions involving the genitals and soles of the feet after brief sun exposure and was subsequently diagnosed with neonatal lupus erythematosus.
Recognition of neonatal liver disease has been heavily dependent on the occurrence of jaundice. In most instances the jaundice is related to specific disturbances in bilirubin transport and other tests of liver function are normal. In contrast, hepatitis and other liver diseases not specifically affecting bilirubin transport often go undetected unless jaundice occurs.
The development of practical methods for the estimation of bile acids in serum has permitted an evaluation of hepatic excretory function in neonates and children independent of bilirubin excretion. Since bile acid excretion by the liver each day greatly exceeds bilirubin excretion it was not surprising to find that elevations in serum bile acids occur regularly in anicteric hepatitis. Because the excretion of bile acids generates canalicular bile flow a reduction in the capacity to excrete bile acids intimates the presence of cholestasis. Early cholestasis is not associated with hyperbilirubinaemia but as cholestasis becomes more severe, jaundice occurs and the possibility of biliary atresia arises in neonates. Serum bile acid patterns in neonates being evaluated for biliary atresia indicate two distinctive patterns. Those infants with severe cholestasis and patent bile ducts usually have pre-dominantly cholic acid in serum. This observation is consistent with the bile acid patterns found in intrahepatic and extrahepatic cholestasis occurring in adult life. Infants found to have extrahepatic biliary atresia have marked elevations in the proportion of chenodeoxycholate in serum. Since elevations of chenodeoxycholate in serum are associated with hepatitis, the findings are consistent with the view that extrahepatic atresia is a rare sequalae of hepatitis.
Studies of bile acid metabolism and excretion give promise of providing further insight on the pathogenesis of cholestatic liver disease.
Neonatal lupus erythematosus is a rare disorder caused by the transplacental passage of maternal autoantibodies. The 52-kDa Ro/SSA antigen (Ro52) ribonucleoprotein represents an antigenic target strongly associated with the autoimmune response in mothers whose children have neonatal lupus and cardiac conduction disturbances, mainly congenital heart block. The objective of this study was to identify putative Ro52/60-kDa Ro/SSA antigen (Ro60) epitopes associated with neonatal lupus and congenital heart block. The reactivity of IgG antibodies present in the sera from mothers with systemic lupus erythematosus and Sjögren's syndrome and in the sera from asymptomatic mothers (a longitudinal study of 192 samples from 66 subjects) was investigated by ELISA using Ro52, Ro60 and 48-kDa La/SSB antigen proteins, as well as 45 synthetic peptides, 13–24 residues long, of Ro52/Ro60 proteins. One to 19 samples collected before, during and after pregnancy were available for each mother. Forty-three disease controls selected randomly and normal sera were tested in parallel. Although no differences were found between Sjögren's syndrome and asymptomatic mothers of group I, who had at least one infant with neonatal lupus, and of group II, who had healthy babies only, significant differences were observed between lupus mothers from both groups. In the former group of lupus mothers, a significantly higher frequency of antibodies to Ro52 peptides 107–122 and 277–292 was observed. Between 18 and 30 weeks of gestation, the period of risk, there was clearly an elevated level of antibodies reacting with Ro52 peptides 1–13, 277–292 and 365–382. Antibodies to Ro52 peptide 365–382 have been shown previously to cross-react with residues 165–185 of the heart 5-HT4 serotoninergic receptor, and might be pathologically important. The level of these Ro52 antibody subsets decreased at the end of pregnancy and after delivery. IgG antibodies to Ro52 peptides 1–13, 107–122, 277–292 and 365–382 may therefore represent important biomarkers to predict a complication in pregnant lupus women with Ro52 antibodies.
This study was undertaken to analyze the histopathological findings of oral discoid lupus erythematosus with conventional light microscopy for early diagnosis of the oral lesions that would aid in prompt treatment.
To find out the predominant age, sex, site and clinical features of oral discoid lupus erythematosus. To study the histopathological features of oral discoid lupus erythematosus. To study the alterations of basement membrane changes of oral discoid lupus erythematosus.
Materials and Methods:
Our study consisted 21 cases of diagnosed DLE with oral lesions. A detailed clinical proforma was used for thorough clinical examination and light microscopy was used for histopathological study of the incisional biopsy specimens.
Statistical Analysis Used:
The lesions were diagnosed on the histopathological criteria given by Gisslen et al. and was statistically analyzed using the Chi square test.
In the present study 9.52% patients had only oral lesions, while 90.47% patients had oral lesions along with skin lesions with the most common site of oral involvement being labial mucosa (76.19%), vermillion border (71.42%) and buccal mucosa (42.85%). On clinical examination, white spots were present in 28.6%, ulcers in 19% and central erythema in 52.4% lesions. Histopathologically, atrophy was observed in 66.66% cases, acanthosis in 66.66% and acanthosis alternating with atrophy in 33.33% cases along with the basement membrane appearing thin and homogenous in 66.7% and partially destroyed in 81% cases with Periodic Acid Schiff stain.
Thus, from this study it was found that a diagnosis of oral discoid lupus erythematosus was based on the combination of clinical and histopathological findings. Thus the dentist may be in an important position to establish the diagnosis with the aid of clinical and histopathological findings before the cutaneous lesions become apparent.
Discoid lupus erythematosus; hyperkeratotic surface; periodic acid schiff stain; photosensitivity
Of the various collagen vascular diseases seen in pediatric age group, discoid lupus erythematosus, systemic lupus erythematosus, neonatal lupus erythematosus, juvenile dermatomyositis and childhood scleroderma are common and of practical importance to clinicians. Various treatment modalities of these conditions have been discussed at length. Of these, some are conventional and routine,while others are used in challenging situations of these diseases. Autologous stem cell transplant, biological therapies, intravenous immunoglobulin and narrow band ultraviolet B are among the latest therapeutic options for these difficult-to-treat conditions in children.
Children; collagen vascular diseases; lupus erythematosus
A 46 year old white woman with active systemic lupus erythematosus developed a skin rash 8 hours after intravenous urography with the non-ionic contrast medium iopamidol. Severe Stevens-Johnson syndrome with erythema multiforme, intrahepatic cholestasis, pulmonary infiltrates and acute renal failure ensued, leading to her death. Although non-ionic contrast media are generally less toxic than traditional ionic agents, their use in patients with immunological disease may be hazardous.
The effects of maternal systemic lupus erythematosus (SLE) on neonatal prognosis were examined by comparing clinical features of full-term babies born to lupus mothers and age- and parity-matched controls.
Materials and Methods
From January 2000 to December 2005, 39 singletons were born to 37 SLE women. Excluding 11 cases of prematurity and preeclampsia, 28 full-term neonates formed the lupus group. The control group included 66 full-term babies. The retrospective study examined medical records and compared gestational age, birth weight, days of hospital stay, small for gestational age (SGA) frequency, Apgar scores < 7, and parity. Lupus neonates were tested for anti-nuclear antibody (ANA) and platelet count, and electrocardiogram was performed.
Average gestational age (38 vs. 39 weeks, p < 0.05) and birth weight (2,775 vs. 3,263 g, p < 0.05) were significantly different between the SLE and control groups. SGA frequency was higher in the SLE group (25% vs. 4.5%, p < 0.05). No significant difference was observed in Apgar score, birth weight, gestational age, SGA frequency, and platelet count between lupus subgroups formed based on anti-dsDNA antibody levels and antiphospholipid antibody status.
The association of maternal ANAs, antiphospholipid antibodies, and drug history with neonatal prognosis could not be elucidated. However, even in uncomplicated pregnancies, maternal lupus is disadvantageous for gestational age, birth weight, and SGA frequency.
Lupus erythematosus; systemic; newborn
Neonatal lupus erythematosus is an immune-mediated disease caused by transplacental passage of maternal autoantibodies, primarily anti-Ro (SSA) and anti-La (SSB). The major clinical manifestations are congenital heart block, cutaneous lupus lesions, and hematologic problems. Hepatic, pulmonary, and neurological involvements are rare. We report a 5-day-old male neonate, born to a clinically asymptomatic mother, presenting with conjugated hyperbilirubinemia, cutaneous lupus lesions, congenital heart block, and thrombocytopenia. Both the neonate and his mother had high titers of antinuclear antibodies (1:640), anti-Ro (SSA), and anti-La (SSB) antibodies. The thrombocytopenia improved with prednisolone (2 mg/kg/day) for 14 days. The skin lupus rashes and bilirubin resolved 2 months later, and liver enzymes were completely normal by 6 months.
We report the first case of Cremophor EL-induced cutaneous lupus erythematosus-like reaction in a 40-year-old female undergoing treatment for breast cancer. There have been four reported cases of paclitaxel- and four cases of docetaxel-induced cutaneous lupus reactions in the published literature [Dasanu and Alexandrescu: South Med J 2008;101:1161–1162; Adachi and Horikawa: J Dermatol 2007;34:473–476; Lortholary et al: Presse Med 2007;36:1207–1208; Chen et al: J Rheumatol 2004;31:818–820]. Our patient developed findings of a cutaneous lupus-like reaction with administration of paclitaxel which was subsequently discontinued. She was re-challenged with albumin-bound paclitaxel which has no Cremophor EL compound in its formulation. This administration of albumin-bound paclitaxel did not induce further reaction. She did not develop a cutaneous lupus erythematosus-like reaction with three other subsequent administrations of albumin-bound paclitaxel. The diagnosis of lupus-like reaction in our patient was made based on the development of a malar butterfly rash sparing the nasolabial folds, the appearance of this rash in context of recently receiving treatments with paclitaxel, resolution of the rash after discontinuing the paclitaxel, and the presence of autoimmune antibodies in the patient's serum which resolved with discontinuation of the paclitaxel. This is the first case demonstrating that the cause of the cutaneous lupus erythematosus-like reaction is not likely due to the taxane component of paclitaxel but the chemical composition of Cremophor EL. If the chemotherapeutic agent was causing the reaction then the same reaction should be seen by albumin-bound paclitaxel. We propose that previously reported lupus reactions may actually be due to Cremophor EL, which consists of polyoxyethylated castor oil, and not the chemotherapeutic agent itself.
Cremophor EL; Cutaneous lupus erythematosus; Taxol; Paclitaxel
Purpose of review
Systemic lupus erythematosus (SLE) is a heterogeneous human disease influenced by a complex interplay of necessary, but not individually sufficient, factors. Although many genetic and environmental factors are associated with SLE, this review will focus on the evolving evidence for key Epstein-Barr virus (EBV)-specific roles in SLE, focusing on new experimental studies published during 2009, 2010, and 2011.
SLE patients have a dysregulated immune response against EBV. EBV antigens exhibit structural molecular mimicry with common SLE antigens and functional molecular mimicry with critical immune-regulatory components. SLE patients, from a number of unique geographic regions, are shown to have higher rates of EBV seroconversion, especially against early EBV antigens, suggesting frequent viral reactivation. SLE patients also have increased EBV viral loads and impaired EBV-specific CD8+ cytotoxic T cells, with impaired cytokine responses to EBV in lupus patients. Irregular cytokine production in plasmacytoid dendritic cells and CD69+ CD4+ T cells after stimulation with EBV has also been demonstrated.
Recent advances demonstrate SLE-specific serologic responses, gene expression, viral load, T-cell responses, humoral fine specificity, and molecular mimicry with EBV, further supporting potential roles for EBV in lupus etiology and pathogenesis.
environmental factors; Epstein-Barr virus; etiology; systemic lupus erythematosus
BACKGROUND--High titres of serum antiphospholipid antibodies are a possible pathogenic factor for cardiac lesions in patients with systemic lupus erythematosus. OBJECTIVE--To test the hypothesis of a causal link between high titres of antiphospholipid antibodies in the serum and myocardial involvement in patients without systemic lupus erythematosus. PATIENTS AND DESIGN--18 patients with primary antiphospholipid syndrome (recurrent fetal loss, arterial and/or venous thrombosis, high titres of antiphospholipid antibodies, and no criteria for systemic lupus erythematosus) were prospectively studied by cross sectional, M mode, and pulsed Doppler echocardiography, and compared with 18 healthy controls. The pulsed Doppler indices of left ventricular diastolic function included isovolumic relaxation time and four mitral outflow indices: peak velocity of early flow, peak velocity of late flow, early to late peak flow velocity ratio, and rate of deceleration of early flow. Four computerised M mode indices were also measured: peak rate of left ventricular enlargement in diastole, peak rate of posterior wall thinning, peak velocity of lengthening of the posterior wall, and velocity of circumferential chamber lengthening. RESULTS--Compared with controls, patients with primary antiphospholipid syndrome had higher values for isovolumic relaxation time and peak velocity of late mitral outflow and lower values for early to late mitral peak outflow velocity ratio, rate of deceleration of early mitral outflow, peak rate of left ventricular enlargement in diastole, peak rate of posterior wall thinning, peak velocity of lengthening of the posterior wall and velocity of circumferential chamber lengthening. CONCLUSION--This abnormal pattern reflects an impairment of myocardial relaxation and filling dynamics of the left ventricle in patients with primary antiphospholipid syndrome who were free of any clinically detectable heart disease. These data suggest that high serum titres of antiphospholipid antibodies may be associated with subclinical myocardial damage.
Congenital complete heart block in utero has become diagnosed more frequently with the clinical use of fetal echocardiography. The fetus with complete heart block may remain asymptomatic or may develop congestive heart failure. Congenital complete heart block is more frequently seen in infants of mothers with systemic lupus erythematosus, both clinically manifested and subclinical systemic lupus erythematosus with positive antibodies (SS-A and SS-B antibodies). At birth, the neonate with complete heart block may remain asymptomatic and may not require a pacemaker to increase the heart rate. The indications for a pacemaker in neonates with complete heart block have been discussed. Both in-utero and neonatal management of congenital complete heart block are discussed to manage congestive heart failure in a fetus. Four patients with congenital complete heart block are presented covering a broad spectrum of clinical presentation, diagnosis, and management both in the fetal and neonatal period.
Tumid lupus erythematosus is a rare variant of chronic cutaneous lupus erythematosus that is characterized clinically by smooth, nonscarring, pink to violaceous papules or plaques without evidence of surface change. Histopathologic features include superficial and deep lymphocytic infiltration in a perivascular and periadnexal distribution, with dermal interstitial mucin deposition and focal or absent dermoepidermal junction involvement. These clinical and histopathologic features can be challenging to differentiate from other cutaneous diseases. This is particularly true because patients with tumid lupus erythematosus usually do not have other manifestations of systemic lupus erythematosus or cutaneous lupus erythematosus. We present two cases of tumid lupus erythematosus, one associated with concomitant systemic lupus erythematosus and the other occurring concurrently with discoid lupus erythematosus. Furthermore, we demonstrate the rare occurrence of a patient with tumid LE occurring below the waist at a photoprotected site.
tumid lupus erythematosus; systemic lupus erythematosus; discoid lupus erythematosus; chronic cutaneous lupus erythematosus
Lupus is a chronic inflammatory autoimmune disease with a wide range of clinical presentations resulting from its effect on multiple organ systems. There are four main types of lupus: neonatal, discoid, drug-induced, and systemic lupus erythematosus (SLE), the type that affects the majority of patients. Patients with lupus experience a loss of self-tolerance as a result of abnormal immunological function and the production of autoantibodies, which lead to the formation of immune complexes that may adversely affect healthy tissue.
Although the precise etiologic mechanism is unknown, genetic, hormonal, and environmental factors, as well as immune abnormalities, have been identified. Associations between lupus onset and age, sex, geography, and race have also been established. Management of this disease should be individualized and should include both pharmacological and nonpharmacological modalities for symptom relief and resolution as well as improved quality of life.
Neonatal lupus erythematosus (NLE) is an autoimmune disease affecting the fetus as a result of transplacental transfer of anti-Ro autoantibodies. Typically, it presents in the first few months of life with an annular form of subacute cutaneous lupus erythematosus. We report an unusual case of NLE presenting at birth with scaly erythematous telangiectatic patches and macules with skin atrophy involving the face, head, and upper trunk. Thrombocytopenia was discovered on laboratory investigations. Histopathology of skin biopsy was consistent with subacute cutaneous lupus. The mother was clinically free of disease and had no family history of autoimmune disease. Serology (extra-nuclear antigens) was positive in both the baby and the mother. This is a rare presentation of a rare disease.
Atrophic lesions; congenital lupus erythematosus; Saudi Arabia
Pulmonary arterial hypertension (PAH) is a serious and often fatal complication of systemic lupus erythematosus (SLE). Because the diagnosis of PAH often is made years after symptom onset, early diagnostic strategies are essential. Doppler echocardiography currently is considered the noninvasive screening test of choice for evaluating pulmonary hypertension.
Screening for asymptomatic pulmonary hypertension in systemic lupus erythematosus patients using Doppler echocardiography, and correlating it with inflammatory parameters of the disease.
Patients and methods:
Doppler echocardiography was performed in 74 patients with systemic lupus erythematosus over one year (66 adult and 8 juvenile), adult SLE included 57 patients with adult-onset and 9 patients with childhood-onset. Pulmonary hypertension was diagnosed if the peak systolic pressure gradient at the tricuspid valve was more than 30 mmHg. All patients were subjected to full history taking, rheumatological examination, laboratory studies and chest x-ray.
In seventy four SLE patients, the pulmonary hypertension was detected in 8 patients (10.8%), 7 adult-onset SLE patients (aged from 19 to 30 years) and 1 juvenile SLE (aged 12 years). The range of pulmonary artery systolic pressure was 34–61.2 mmHg (43.19 ± 9.28). No significant differences between patients with and those without pulmonary hypertension as regard clinical features. Significantly higher frequencies of rheumatoid factor and anti-cardiolipin antibodies were found in patients with pulmonary hypertension versus those without (P = 0.02, P = 0.008 respectively). Positive rheumatoid factor and ACL were significantly associated with occurrence of PAH in SLE (P = 0.007, P = 0.006 respectively). No significant correlations were found between pulmonary artery pressure, disease duration, SLE Disease Activity Index (SLEDAI), ESR, and anti-ds DNA.
Patients with SLE have an increased risk of pulmonary arterial hypertension. Echocardiography should be used as a screening tool in patients at high risk for development of pulmonary hypertension. Positive anti-cardiolipin antibodies and rheumatoid factor were significant predictors of pulmonary hypertension in our study.
systemic lupus erythematosus; pulmonary hypertension; echocardiography; screening
Treatment adherence is critical in the management of rheumatic diseases. Recent advances in therapy for rheumatoid arthritis (RA) and systemic lupus erythematosus (SLE) are promising, although the impact on quality of life may be limited due to nonadherence. Databases including Ovid Medline, Scopus and the Epub-ahead-of-print subset of PubMed were searched for the period of the last 10 years using combined keywords patient compliance, medication adherence, disease modifying antirheumatic drug (DMARD), rheumatoid arthritis and systemic lupus erythematosus. Additional references from retrieved papers were considered. Inclusion criteria were the following: identification of a quantitative measure of adherence to medications including DMARDs and biologics; inclusion of well-defined measures of adherence; and patients with RA or SLE. Studies in RA and SLE patients demonstrated overall inadequate treatment adherence. Adherence was measured using multiple methods including pharmacy records, electronic monitoring, self-report and physician report. The evidence for interventions to improve treatment adherence was limited and demonstrated various results. Future research should further explore determinants of nonadherence and continue to examine the efficacy of implementing various strategies to improve medication management in this patient population.
adherence; disease-modifying antirheumatic drugs; rheumatoid arthritis; systematic review; systemic lupus erythematosus