Use of belimumab throughout pregnancy to treat active systemic lupus erythematosus—A case report

Background

Pregnancy can lead to flares in systemic lupus erythematosus (SLE), and the presence of SLE in pregnancy could lead to a poor outcome for the mother and the fetus.

Objective

To describe a patient whose active SLE (including lupus nephritis) was managed with the use of belimumab throughout pregnancy.

Methods

A case report and review of relevant literature is presented.

Results

A 38-year-old Caucasian woman with SLE was seen for advice regarding planning a pregnancy and management of her active lupus (cutaneous lupus, angioedema, lupus nephritis, leukopenia, and anti-phospholipid antibody syndrome) that could only be controlled by mycophenolate, a drug contraindicated in pregnancy. Azathioprine, hydroxychloroquine, rituximab, and moderate doses of prednisone were either unable to control her disease or led to unacceptable toxicity. After detailed discussions, she was treated with belimumab, which controlled her SLE and allowed withdrawal of mycophenolate. Belimumab was continued throughout the pregnancy, leading to well-controlled SLE and uneventful course, albeit with the presence of mild Ebstein?s anomaly in the baby.

Conclusion

To our knowledge, this is the first case report of belimumab use throughout pregnancy for controlling active SLE. Data from the belimumab pregnancy registry would be useful to confirm our findings and to further assess safety of this agent for use in pregnancy.     

Concomitant systemic lupus erythematosus and HIV: Case series and literature review

Objectives

To determine the effect of systemic lupus erythematosus (SLE) and human immunodeficiency virus (HIV) infection on the course of each other and to review the published reports on concomitant SLE and HIV infection.

Methods

We performed a retrospective review of the records of patients with SLE and HIV seen in the Department of Rheumatology at the Inkosi Albert Luthuli Central Hospital, Durban, South Africa. We used the terms “systemic lupus erythematosus” and “HIV” or “AIDS” to identify patients with SLE and HIV infection reported in the English medical literature.

Results

We identified 13 patients with SLE and HIV infection. All the patients were females and there were 11 African blacks and 2 Indians. SLE and HIV infection were diagnosed together in 6 patients. In this group, there were 5 lupus flares in 4 patients, and 2 of the flares followed highly active anti-retroviral treatment (HAART). Five patients developed HIV after the diagnosis of SLE. The 3 patients in whom follow-up data was available had inactive SLE, one of whom was on HAART. Two HIV-positive patients developed SLE after receiving HAART for 30 and 35 mo. Seven of our patients also had tuberculosis. Our literature search identified 58 previously reported patients with HIV and SLE.

Conclusion

Our case series and review of the literature show that there is a reduction in SLE disease activity in patients with concomitant SLE and HIV. However, when lupus flares occur in HIV-positive patients, they are unrelated to the use of HAART.     

La pancréatite lupique : une série de six cas

Purpose

The occurrence of acute pancreatitis in systemic lupus erythematosus (SLE) is known but rare, and is exceptionally the presenting manifestation. Its pathogenesis is multifactorial, and it is difficult to separate what belongs to vasculitis, thrombotic phenomena in the context of an associated antiphospholipid syndrome, or iatrogenic complications. We report on six cases of lupus pancreatitis.

Methods

This is a retrospective monocenter study of 110 patients with SLE. The diagnosis of lupus pancreatitis was established after exclusion of other causes of pancreatitis.

Results

Five women and one man (5.4%) with a mean age of 36.3 years presented with lupus pancreatitis. In four patients the pancreatitis was concurrent with the diagnosis of SLE and it occurred later during an exacerbation of the disease in the two remaining patients. In all patients, pancreatic manifestations were associated with other organ involvement. Clinical manifestations were: abdominal pain (n = 6), vomiting (n = 3), and fever (n = 3). Elevated pancreatic enzyme was noted in all cases. All patients were treated by high doses of glucocorticoids. The outcome was favorable in five patients, and one patient died.

Conclusion

Pancreatitis may be the presenting manifestation of SLE. Its pathogenesis is often multifactorial. The outcome is usually favorable with corticosteroids.     

A regulatory role for CD72 expression on B cells in systemic lupus erythematosus

Background

B regulatory cells and their regulatory products/markers, such us semaphorin 3A (sema3A) and its receptor NP-1, FcγIIB, IL-10, and others, act at the very base of self-tolerance, maintenance, and prevention of autoimmune disease development.

Objectives

The aim of the present study was to assess the involvement of CD72, a regulatory receptor on B cells, in systemic lupus erythematosus (SLE). In addition, the potential of soluble sema3A in enhancing the expression of CD72 on B cells of SLE patients was investigated.

Results

CD72 expression on activated B cells of SLE patients was significantly lower than that of normal controls. This lower expression of CD72 in SLE patients correlated inversely with SLE disease activity and was associated with lupus nephritis, the presence of anti-dsDNA antibodies, and low levels of complement. Co-culture of purified B cells from healthy controls with condition-media containing recombinant sema3A resulted in significant enhancement of CD72. Similar enhancement of CD72 on activated B cells from SLE patients, though significant, was still lower than in normal individuals.

Conclusions

The lower expression of CD72 on activated B cells from SLE patients correlates with SLE disease activity, lupus nephritis, the presence of anti-dsDNA antibodies, and low levels of complement. The improvement of CD72 expression following the addition of soluble semaphorin 3A suggests that CD72 may be useful as a biomarker to be followed during the treatment of SLE.     

Do current arterial hypertension treatment guidelines apply to systemic lupus erythematosus patients? A critical appraisal

Objective

Arterial hypertension (HTN) is reported to burden up to 74% of systemic lupus erythematosus (SLE) patients and contributes significantly to accelerated atherosclerosis and increased cardiovascular (CV) risk. Current HTN treatment guidelines have not incorporated lupus patients in their recommendations; whether these guidelines can be fully implemented in SLE is doubtful.

Methods

A critical appraisal of the existing HTN guidelines in regard to SLE is presented in this review, based upon clinical and experimental data. Particular issues addressed are the time of antihypertensive therapy initiation, the optimal blood pressure level, the antihypertensive agent of first-choice and the need for reduction of the total cardiovascular risk in SLE.

Results

Antihypertensive therapy should be recommended at levels of 140/90 mmHg (systolic and diastolic BP, respectively) in newly diagnosed lupus patients without overt target organ involvement. In the case of lupus nephritis (LN) or diabetes mellitus (DM), therapy should be implemented at lower levels, such as 130/80 mmHg. Hypertensive lupus patients should be considered at high or very high CV risk and, consequently, the optimal BP level should be less than 130/80 mmHg. Angiotensin converting enzyme inhibitors (ACEIs) and angiotensin receptor blockers (ARBs) seem to be a safe and efficacious first-choice antihypertensive treatment in lupus patients. Total CV risk should be considered and co-morbidities (dyslipidemia, antiphospholipid syndrome, etc.) should be managed promptly.

Conclusions

Current HTN therapeutic guidelines, lacking data from large-scale clinical trials, may not adequately apply to SLE patients. The assessment of the aforementioned recommendations in randomized clinical trials is expected to confirm their value in reducing CV risk in SLE.     

The epidemiology of atherosclerotic cardiovascular disease among patients with SLE: A systematic review

Objective

To perform a systematic review of the literature regarding the epidemiology of the association between systemic lupus erythematosus (SLE) and atherosclerotic cardiovascular disease (CVD), including the increased risk for CVD, as well as the risk factors responsible for development of CVD in patients with SLE.

Methods

We followed the PRISMA guidelines to systematically search the PubMed database from inception to June 2012. Studies were selected using predefined eligibility criteria, and 2 authors independently extracted data. The risk of bias was measured for each study using a domain-based assessment.

Results

We report on 28 studies that met criteria for inclusion in our analysis. We found strong epidemiologic evidence that SLE patients have an increased relative risk of CVD compared to controls. There is limited information regarding relative CVD mortality risks among SLE patients. Traditional CVD risk factors, including age, male sex, hyperlipidemia, smoking, hypertension, and CRP, are associated with CVD risk among SLE patients. Several SLE-specific factors, including disease activity and duration, and possibly specific manifestations and therapies, further increase risk. Several risk factors, such as disease activity and glucocorticoid use, are closely associated, making it difficult to disentangle their effects.

Conclusions

CVD risk among SLE patients compared to the general population is at least doubled. While older SLE patients appear to have the highest absolute risks of CVD, young women have alarmingly high relative risks, given the rarity of CVD in the comparison general population. Both traditional and SLE-specific risk factors are important, although there are discrepancies within the literature.     

Are RASopathies new monogenic predisposing conditions to the development of systemic lupus erythematosus? Case report and systematic review of the literature

Objective

RASopathies (Noonan syndrome (NS) and Noonan-related syndromes) are neurodevelopmental syndromes resulting from germline mutations in genes that participate in the rat sarcoma/mitogen-activated protein kinases (RAS/MAPK) pathway (PTPN11, SOS1, RAF, KRAS or NRAS, and SHOC2). Some monogenic conditions are associated with the development of systemic lupus erythematosus (SLE), and a few reports described the association of SLE with NS. We aim to search for a relationship between RASopathy and the development of SLE.

Methods

We reported for the first time a case of 13-year-old boy with NS with loose anagen hair (NSLAH) resulting from mutation in SHOC2 who developed an autoimmune disorder that fulfilled four American College of Rheumatology (ACR) criteria for the classification of SLE (polyarthritis, pericarditis, antinuclear antibodies, and anti-DNA antibodies). The case report then prompted a literature review by a systematic search for English and French articles on the subjects of RASopathies and SLE that had English abstracts in PubMed from 1966 to 2012.

Results

We identified seven additional patients with RASopathy and SLE. The male-to-female ratio was 1:1 and age at onset of SLE ranged from 5 to 32 years. The most common features were polyarthritis (7/8 patients), autoimmune cytopenia (4/8 patients), and pericarditis (4/8 patients) while only one patient presented with skin involvement.

Conclusion

The association of two rare diseases in eight patients suggests that RASopathies may be associated with the development of SLE, which is characterized by a higher male-to-female ratio, a lower rate of skin involvement, and a higher rate of pericarditis than “classic” SLE.     

Coexistence of systemic lupus erythematosus and multiple sclerosis: Prevalence,clinical characteristics,and natural history

Objectives

The coexistence of systemic lupus erythematosus (SLE) and multiple sclerosis (MS) in the same individual has rarely been described. Our objective was to report on the prevalence, clinical characteristics, and prognosis of cases fulfilling the criteria for both SLE and MS.

Methods

We utilized existing patient cohorts from the Departments of Rheumatology and Neurology, University of Crete, and screened patients diagnosed with either SLE (n = 728) or MS (n = 819) for features of both diseases. The clinical, laboratory, and neuroimaging findings were assessed.

Results

We identified nine patients who fulfilled the diagnostic criteria for both SLE and MS, corresponding to a prevalence rate of 1.0–1.2% in each cohort. All patients were women, with an average age at SLE diagnosis of 42.1 years (range: 34–56 years). The diagnosis of SLE preceded the development of MS in five patients, with a time lag ≤5 years in four of them. Initial presentation of MS included spinal symptoms in seven patients. All patients had features of mild SLE with predominantly cutaneous, mucosal, and musculoskeletal manifestations. Accordingly, therapeutic decisions were mainly guided by the severity of the neurological syndrome. During the median follow-up of 4 years (range: 1–10 years), three patients remained stable and the remaining experienced gradual deterioration in their neurological status. SLE remained quiescent in all patients while on standard immunomodulatory MS therapy.

Conclusions

Occurrence of both diseases in the same individual is rare, corroborating data that suggest distinct molecular signatures. SLE and MS coexistence was not associated with a severe phenotype for either entity.     

L’entérite : une manifestation peu fréquente et corticosensible du lupus érythémateux systémique

Introduction

. Lupus enteritis is a rare manifestation of systemic lupus erythematosus. The clinical manifestations are variable including abdominal pain, diarrhea, nausea and vomiting. Lupus enteritis is thought to be related to vasculitis.

Case reports

. We report here three new cases. All three patients aged of 45, 24 and 43 years (two females and one male) were admitted for abdominal pain, vomiting and diarrhea, and fulfilled the ACR criteria of systemic lupus erythematosus. The diagnosis of lupus enteritis was retained on the CT scan findings and the favorable outcome on corticosteroids after infectious etiologies were excluded.

Conclusion

. Lupus enteritis is thought to be one of the most common causes of acute abdominal pain in systemic lupus erythematosus. The diagnosis is based on clinical, radiological and biological findings. A good response to corticosteroids is usually reported.     

The Systemic Lupus International Collaborating Clinics (SLICC) damage index: A systematic literature review

Objectives

We performed a systematic literature review to determine factors that influence damage and damage progression in SLE patients and how damage relates to mortality in this population.

Methods

A search of Medline, Embase and Web of Science was performed, with papers included if they met the requirements of containing keywords relating to SLE and damage assessment using the SDI, published between 1990 and October 2012.

Results

A total of 358 articles were identified, with 50 included in this review. From 17 studies reporting damage at more than 2 time points, damage progressed over time, but the rate of damage accrual reported was variable across studies. Demographic factors that influence the accrual of damage in several reports include male gender, older age, longer disease duration, Afro-Caribbean and Indo-Asian ethnicity. Patients with higher disease activity at a single time point or over time accrue greater damage. Certain organ system involvement also predicts damage accrual, in particular renal and neuropsychiatric involvement. Corticosteroids, cyclophosphamide and azathioprine all show an association with damage accrual, while hydroxychloroquine appears to have a “protective” effect. Four studies, which examined prognosis, all demonstrated that damage is a predictor of future mortality.

Conclusions

Damage in SLE patients increases over time and predicts future mortality. Patients at risk of damage can be identified from demographics factors and the pattern of clinical involvement. Disease activity, corticosteroids and immunosuppressive therapy are also associated with future damage but further studies are needed to separate the mechanisms of these associations from the problem of residual confounding.     

Primary preventive services in patients with systemic lupus erythematosus: Study from a population-based sample in Southeast U.S.

Objectives

Systemic lupus erythematosus (SLE) patients are at risk for complications that can be mitigated by appropriate preventive care. We examined the receipt of immunizations, cancer screening, and cardiovascular risk preventive services in a predominantly Black cohort of SLE patients from the Southeast U.S. To identify gaps in primary preventive services (PPS) that might be specific to SLE as opposed to local health system factors, we used as reference a population-based sample from the same area.

Methods

A cross-sectional design was used to characterize the percentage of PPS received by 751 SLE patients from Atlanta, GA, and 9040 subjects from the same community, of whom 938 had diabetes. Factors associated with the receipt of PPS were examined with multivariable analysis of variance.

Results

Approximately 65% of recommended PPS were provided to the SLE, overall community (OC), and diabetes samples. However, only 22.5%, 45.7%, and 27.6% of SLE, OC, and diabetes subjects, respectively, received all recommended services. Factors associated with a higher percentage of PPS received by SLE patients included older age (63.6% if age ≥65 years, 45.8% if age between 18 and 35 years), having medical insurance (61.1% for insured, 49.7% for uninsured), having a primary care physician (PCP) (59.0% if patient had PCP, 51.8% if patient did not have PCP), and being a non-smoker (61.9% for non-smokers, 49.9% for smokers).

Conclusions

Less than one-quarter of SLE patients from a southeast U.S. community received all the recommended services that were studied. Further research is warranted to unravel the barriers that prevent SLE patients from reaching appropriate standards of preventive care.     

Evolution of cardiac dysfunction in patients with antiphospholipid antibodies and/or antiphospholipid syndrome: A 10-year follow-up study

Objectives

To describe the evolution of valve involvement and myocardial dysfunction over time in patients with systemic lupus erythematosus (SLE) with or without antiphospholipid antibodies (aPL) and/or antiphospholipid syndrome (APS).

Methods

From an initial cohort of 150 patients assessed by transthoracic echocardiography 10 years ago, 17 patients with primary APS (PAPS), 23 with SLE-associated APS (SLE/APS), 19 with SLE positive for aPL without APS, and 23 with SLE negative for aPL were re-evaluated in the present echocardiography study.

Results

Valvulopathy was detected in 65% of PAPS and 62% of SLE patients with or without aPL. Disease duration [odds ratio (OR), 1.63; 95% confidence interval (CI), 1.13–2.36; p = 0.009 for every 5 years of increase] and presence of SLE/APS (OR, 3.51; 95% CI, 1.27–9.67; p = 0.015) were the only factors associated with the progression of valvular disease in univariate and multivariate analyses. Left ventricular diastolic dysfunction similarly progressed over time, with deceleration time (DT) and isovolumic relaxation time (IVRT) being equally prolonged in each of the four groups (p < 0.05). Right ventricular DT was significantly prolonged in each of the three SLE patient groups (p < 0.001), whereas IVRT increased only in SLE/APS patients (p = 0.040).

Conclusions

Among patients with APS and SLE (with or without aPL), SLE/APS and disease duration were independent factors for valvular disease progression in the present 10-year follow-up echocardiography study. Anticoagulation did not arrest valvular disease progression. Ventricular diastolic dysfunction, primarily of the left ventricle, also progressed over the 10-year period.     

Long-term prognosis and causes of death in systemic lupus erythematosus

Purpose

We studied survival rate, prognostic factors, and causes of death in patients with systemic lupus erythematosus (SLE), particularly focusing on the influence of disease severity.

Patients and Methods

A cohort of 207 consecutive Italian patients with SLE were prospectively studied. All prominent clinical and serologic parameters were evaluated and considered as prognostic risk factors. Causes of death were defined on the basis of clinical data and, when available, postmortem examination. Survival was calculated from the time of diagnosis by Kaplan-Meier method.

Results

A total of 17 of 207 patients died; causes of death were active disease manifestations in 35.3% of cases and complication of the disease or its treatment in 64.7% of cases. The survival rates at 5, 10, and 15 years after the diagnosis were 96%, 93% and 76%, respectively. By multivariate analysis of the risk factors, a predictive model consisting of male gender, positive lupus anticoagulant, and “severe” SLE was identified. The survival curve of the patients with severe disease was similar to that of patients with mild disease until 10 to 15 years from the diagnosis. Thereafter the two curves tended to diverge, showing a clear survival decline in patients with severe disease.

Conclusions

Our study confirms the increase of short- and medium-term survival in patients with SLE, but long-term prognosis remains poor in patients with severe SLE manifestations.     

Polyradiculonévrite aiguë révélant un lupus érythémateux systémique : une présentation inhabituelle d’évolution fatale

Introduction

Neurological manifestations of systemic lupus erythematosus are common and numerous. They mainly involve the central nervous system, peripheral involvement being rare. Acute polyradiculoneuropathy is very uncommon.

Case report

We report a 44-year-old man, who presented with acute polyradiculoneuropathy revealing systemic lupus erythematosus. Outcome was fatal despite treatment with corticosteroids and immunoglobulin.

Conclusion

Acute polyradiculoneuropathy is a very rare manifestation of systemic lupus erythematosus and can compromise functional and life prognosis. Early diagnosis and management are crucial.     

Lymphomes spléniques de la zone marginale et auto-immunité : à propos de six cas

Introduction

Autoimmune manifestations are common in splenic marginal zone lymphoma (SMZL) and are sometimes the presenting feature of the disease. Autoimmune cytopenia (anemia, thrombocytopenia) are the most frequently reported autoimmune conditions. However, other immunological manifestations may be associated with SMZL.

Méthodes

We report a retrospective case series of six patients with SMZL associated with autoimmunity.

Results

Auto-immune manifestations were the presenting feature of lymphoma in four cases. Auto-immune manifestations included auto-immune cytopenia in three cases (two hemolytic anemia and one pancytopenia), thyroiditis in two cases, systemic lupus and Still's disease in one case each. Antinuclear antibodies were detected with a titre of 1/250 in three cases, and with a titre of 1/32,000 in the patient with systemic lupus. Testing for DNA antibodies was negative in all cases. Two patients had a circulating lupus anticoagulant, with portal venous thrombosis following splenectomy in one case. One patient had hypogammaglobulinemia. A monoclonal gammopathy was detected in three patients. All patients had spleen enlargement. Immunophenotyping of blood peripheral lymphocyte was typical in five out of the six cases. Bone marrow was infiltrated in five out of the six cases. Diagnosis was obtained by the combination of immunophenotyping and bone marrow histopathology in five cases, and by splenic histopathology in the remaining case. Hepatitis C virus serology was negative in all patient.

Conclusion

Autoimmune disease as systemic lupus or Still's disease may be associated with SMZL before its tumoral manifestations are evident. In this mode of presentation, spleen enlargement, hypogammaglobulinemia, monoclonal gammopathy, and multiple autoimmune diseases, should alert the physician.     

Évaluation des attentes des patients pour un programme d’éducation thérapeutique au cours du lupus systémique

Purpose

The aim of this study was to collect information to design a patient education program (PEP) for patients with systemic lupus erythematosus (SLE), based as much as possible on their expectations.

Patients and methods

Three different approaches were used for addressing patients’ needs: 1) A questionnaire on their expectations in terms of a PEP was sent to the members of SLE associations and offered to patients at the French reference center for SLE, 2) A patients’ focus group was conducted, and 3) After the teaching sessions, satisfaction questionnaires were also evaluated.

Results

The patients who answered the expectation questionnaire (n = 422, women/men sex-ratio: 12.6) indicated a major interest in the PEP (70.4%). Their expectations were broad, and covered the topics of pregnancy (90% of the women under the age of 40), the outcome of the disease (80.8%), the respective roles of the different treatments (70.4%), and also the management of everyday symptoms: fatigue and pain (66.4%). The focus group (eight people) highlighted the need for improving how the diagnosis of the disease was delivered, and also revealed the loneliness and the guilty feeling experienced by some patients toward their relatives. Satisfaction questionnaires confirmed these expectations for the PEP, and even extended them to new topics: the mechanisms behind SLE, travel and leisure, and possible accommodations in the workplace.

Conclusions

The direct consultation of patients with SLE targeted by a specific PEP program allowed us to confirm and adapt the topics and the content of a program designed by medical staff.     

Autism spectrum disorders in babies born to mothers with antiphospholipid syndrome

Objectives

To evaluate the outcomes of babies born to mothers with primary antiphospholipid syndrome and to compare to the outcomes of babies of mothers with systemic lupus erythematosus.

Methods

A retrospective study from 2003 to 2010 assessing the clinical characteristics and psychomotor development, as well as the immunological data, of children born to mothers with antiphospholipid syndrome (APS) (group 1) and systemic lupus erythematosus (group 2).

Results

Group 1 consisted of 36 children born to mothers (n = 26) with a primary APS. Autism spectrum disorders occurred in 3 children from group 1 and all of them had persistent anti-β2GP1 IgG antibodies.Group 2 consisted of 12 children born to mothers (n = 9) with lupus erythematosus. Three children experienced cutaneous neonatal lupus, but there were no neurodevelopmental disorders. Comparing children of groups 1 and 2, no significant difference was found with regard to the parameters at birth or during follow-up. The children in group 2 had antinuclear antibodies more frequently (p < 0.05).

Conclusion

Autism spectrum disorders could be observed in babies born to mothers with antiphospholipid syndrome, but there is no risk of thrombosis.

Key messages

Neonatal lupus is well-known complication in children born to mothers with systemic lupus erythematosus, but there is no risk of thrombosis in APS-exposed children.In children of APS mothers the rate of prematurity and small-for-gestational age weight remain high even in treated pregnancy.The presence of several cases of autism spectrum disorders in APS-exposed children could be related to mother's antibodies exposition, but need to be confirmed.     

Choc hémorragique révélant des micro-anévrismes digestifs au cours du lupus : cas clinique et revue de la littérature

Introduction

The vascular disorders in systemic lupus erythematosus (SLE) result from various mechanisms and presentations (inflammatory disease or vasculitis, atherosclerosis).

Prévalence et valeur diagnostique des anticorps antinucléaires de spécificité antigénique indéterminée : étude rétrospective à propos d’une série de 90 patients

Purpose

The objective of this study was to determine the clinical relevance and the diagnostic significance of positive antinuclear antibodies (ANA) without identified antigenic target by the usual characterization technique.

Patients and methods

Retrospective study conducted in the Laboratory of Immunology of Habib Bourguiba Hospital (Sfax, Tunisia) during 18 months. The inclusion criteria were the presence of an ANA titer greater or equal to 1/320 with negative characterization result. ANA screening was performed by indirect immunofluorescence (IIF) on Hep2 cells. Each positive serum was tested by IIF on Crithidia luciliae (anti-native DNA) and by immunodot (anti-nucleosome, anti-histone, anti-Sm, anti-RNP, anti-SSA, anti-SSB, anti-Scl 70, anti-PM-Scl, anti-Jo1, anti-PCNA and anti-ribosomal protein). Sera of systemic lupus erythematosus (SLE), myositis, and scleroderma patients were tested for anti-Ku, anti-PL7, anti-PL12 and anti-Ro-52 using dot myositis.

Results

Sera of 90 patients were studied: 18 men and 72 women (average age: 44 years). Drug-induced ANA was found in eight patients. The most frequent clinical symptoms were joint (56.7%), cutaneous (54.4%) and constitutional symptoms (45.6%). The diagnosis of an autoimmune disease was suspected in 49 patients (54.5%) and confirmed in 30 (33.3%) including 20 cases of connective tissue disease: myositis (n = 6), scleroderma (n = 5), Sjögren's syndrome (n = 3), SLE (n = 4), rheumatoid arthritis (n = 6) and antiphospholipid syndrome (n = 4). Other autoimmune diseases were less frequent. The anti-Ku antibody was detected in the majority of patients with connective tissue disease. The diagnosis of non-autoimmune diseases was established in 25.5% of patients. Eighteen patients (20%) had no diagnosis orientation.

Conclusion

Our study demonstrated the diagnostic value of the presence of ANA even in the absence of known antigenic target, confirmed the role of the IIF as “gold standard” test for ANA screening, and suggested the usefulness of the addition of Ku antigen in the immunodot classic profile.     

The Representation of Gender and Race/Ethnic Groups in Randomized Clinical Trials of Individuals with Systemic Lupus Erythematosus

Purpose of Review

This review evaluated gender and race/ethnic representation in randomized controlled trials (RCTs) of patients with systemic lupus erythematosus (SLE).

Recent Findings

Whites comprise 33% of prevalent SLE cases and comprised 51% of RCT enrollees. Blacks encompass 43% of prevalent SLE cases, but only represented 14% of RCT enrollees. Hispanics comprise 16% of prevalent SLE cases and 21% of RCT enrollees, while Asians comprise 13% of prevalent SLE cases and 10% of RCT enrollees. Males encompass 9% of SLE cases and 7% of RCT enrollees. The reporting and representation of males have remained stable over time, although their representation in RCTs is slighter lower than the prevalence of SLE in males. The representation of Hispanics, Asians, and Native Americans increased over time. However, the representation of blacks among RCT participants has decreased since 2006–2011.

Summary

RCTs among SLE patients need larger sample sizes in order to evaluate heterogeneity in outcomes among racial subgroups. It is imperative that novel strategies be developed to recruit racial minorities with SLE by identifying and improving barriers to RCT enrollment in order to better understand the disease’s diverse population.