Diagnostic Criteria For Systemic Lupus Erythematosus Research Articles

Cardiovascular Alterations Present in Systemic Lupus Erythematosus: Review of the Literature

Systemic lupus erythematosus (SLE) is a systemic inflammatory disease secondary to an autoimmune response that is characterized by the deposition of immune complexes that activate complement and cause tissue damage. The American College of Rheumatology (ACR, American College of Rheumatology) in 2012 established the new diagnostic criteria for SLE, identifying 17 criteria of which at least 4 must be met (at least one clinical, and one immunologic) or lupus nephritis as the sole criterion in the presence of antinuclear antibody (ANA) or double-stranded anti-DNA for diagnosis.1

81 Juvenile RHUPUS syndrome: a case reports

BackgroundRHUPUS syndrome is a rare association with rheumatoid arthritis and systemic lupus erythematosus (SLE) in adult patients. Its pediatric presentation is very rare and underdiagnosed. The mechanism is not well understood yet, but most theories accepted a real overlap between SLE and juvenile idiopathic arthritis. In children. Patients with Rhupus have clinical symptoms of SLE with positive ANA, anti-DNA or anti Sm associated with clinical symptoms of JIA.ObjectiveIllustrate an unusual presentation of lupus in childrenMethodswe present a case of juvenile rhupus syndrome; we describe the clinical presentation, the serological results, the diagnostic criteria for SLE (ACR 1997) and JIA (ILLA 2001) and the treatment installed.Case reportsWe recently diagnosed Rhupus syndrome in an 11-year-old girl who presented with polyarthritis deformans of the bilateral joints of the wrists, hands and feet for 24 months. she also had an onset of profound asthenia, recurrent oral aphtosis and massive hair loss over the past few months. Initial investigation showed anaemia, increased erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP). Radiographic examinations showed juxtra-articular osteopenia and chronic synovitis of the wrists. The autoimmune assessment was contributory with a positive rheumatoid factor (RF), antinuclear antibodies (ANA) (1/1000) and positive anti-Sm antibodies. Anticardiolipin and anti-RNP antibodies were negative. Our patient met >4 ACR criteria for SLE classification. She was treated with methotrexate and hydroxychloroquine, under close medical supervision in order to watch for the appearance of other organic damage to lupus disease, in particular renal and neurological. This type of joint damage is considered to be either lupus joint damage, lupus with chronic arthritis, or overlapping lupus with JIA. Children with Rhupus initially present with JIA and later develop lupus. Previous reports have shown female predominance, polyarticular involvement, non-erosive arthritis, and years of diagnostic wandering. Our patient had polyarthritis deformans with a two-year delay in diagnosis of SLE.ConclusionAlthough rare, the infantile Rhupus syndrome must be evoked in front of a deforming arthropathy.

Antinuclear antibodies (ANA) as a criterion for classification and diagnosis of systemic autoimmune diseases

The classification and diagnosis of systemic autoimmune diseases are frequently based on a collection of criteria composed of clinical, laboratory, imaging, and pathology elements that are strongly associated with the respective disease. Autoantibodies are a distinctive hallmark and have a prominent position in the classification criteria of many autoimmune diseases. The indirect immunofluorescence assay on HEp-2 cells (HEp-2 IFA), historically known as the antinuclear antibody test, is a method capable of detecting a wide spectrum of autoantibodies. A positive HEp-2 IFA test is part of the classification criteria for systemic lupus erythematosus (SLE) and juvenile idiopathic arthritis (JIA), as well as the diagnostic criteria for autoimmune hepatitis (AIH) and primary biliary cholangitis (PBC). A positive HEp-2 IFA test can appear as different morphological patterns that are indicative of the most probable autoantibody specificities in the sample. Only some of the HEp-2 IFA patterns are associated with the specific autoantibodies relevant to SLE, JIA, AIH, and PBC, whereas some other patterns occur mainly in non-related conditions and even in apparently healthy individuals. This paper provides a critical review on the subject and proposes that the classification and diagnostic criteria for SLE, JIA, AIH, and PBC could be improved by a modification on the HEp-2 IFA (ANA) criterion in that the staining patterns accepted for each of these diseases should be restricted according to the respective relevant autoantibody specificities.

Assessment of ovarian reserve by serum anti-Müllerian hormone in patients with systemic lupus erythematosus: a meta-analysis.

Systemic lupus erythematosus (SLE) is a chronic multisystem autoimmune and cyclophosphamide (CYC) is often used in the therapy of SLE. Anti-Müllerian hormone (AMH) is expressed in the ovarian granulosa cells and is a reliable biomarker for ovarian reserve. Recent studies have showed that SLE patients have lower serum AMH levels and CYC has a negative influence on ovarian reserve. But the results are conflicting in other studies. The objective of our study is to perform a systemic review and metaanalysis to confirm the relationship between SLE and ovarian reserve reflected by serum AMH levels as well as the effect of CYC on ovarian reserve of SLE patients. PubMed, Embase, Web of Science, CNKI, CHINESE WANFANG, China Science and Technology Database (VIP) databases were searched for eligible studies by two independent authors. Studies comparing serum AMH levels between SLE patients and healthy controls as well as serum AMH levels between SLE patients with and without the treatment of CYC were extracted. All statistical analyses were performed with STATA 12.0. Totally 19 studies including 1,272 SLE patients and 555 healthy controls were included in our study. In a comparison of serum AMH levels between SLE patients and healthy controls, the pooled SMD was -0.79 (95% CI, -1.41 to -0.18) (P<0.05), indicating a significantly lower serum level of AMH in SLE patients. The results were repeated in subgroup analyses by region, diagnostic criteria of SLE and AMH detection methods. The therapy of CYC in SLE patients had a negative influence on serum AMH levels with the pooled SMD of -0.58 (95% CI, -0.87 to -0.30) (P<0.05). SLE is related to increased risk of decreased ovarian reserve and the treatment of CYC can do harm to ovarian reserve.

Systemic lupus erythematosus: new horizons for diagnosis and therapy

Systemic lupus erythematosus (SLE) is a systemic autoimmune rheumatic disease of unknown etiology, characterized by the overproduction of organ-specific autoantibodies to various components of the cell nucleus and by the development of immune-mediated inflammatory damage to internal organs. SLE is the prototype of a systemic human autoimmune disease; it is an extremely heterogeneous disease in terms of both clinical manifestations and a genetic predisposition and pathogenetic mechanisms, which often complicates the early diagnosis of the diseases and makes it impossible to personify therapy. Despite the fact that in the 21st century, the 15-year survival rate for SLE has increased up to 85%, the inadequate control of disease activity and the need for almost constant intake of glucocorticoids and immunosuppressive drugs result in the accrual of irreversible damages to internal organs and, as a consequence, in decreases in quality of life, disability, social maladaptation, and premature death. The paper considers the new 2019 EULAR/ACR diagnostic criteria for SLE, the contemporary 2019 EULAR recommendations for the management of SLE, and new pharmacotherapy options for this condition, which are based on the deciphering of the mechanisms of SLE immunopathogenesis.

The Regulatory T Cell in Active Systemic Lupus Erythematosus Patients: A Systemic Review and Meta-Analysis.

Background: Regulatory T cells (Tregs) researches in systemic lupus erythematosus (SLE) have floundered over the years, reports on the numbers and function of Tregs in SLE present quite contradictory results. We therefore conducted a meta-analysis to verify the changes of Tregs in active SLE.Methods: We systematically searched PubMed, Embase, and ISI web of knowledge databases for eligible articles. In total, 628 active SLE patients and 601 controls from 18 studies were included. Due to a high degree of heterogeneity, a random effects model was used to assess the mean differences in Treg percentages, absolute numbers, and suppression capacities of Tregs between active SLE and controls. Further, subgroup analysis was performed to identify potential sources of heterogeneity.Results: The pooled percentages of Tregs in active SLE patients were found to be lower than those in controls (−0.864 ± 0.308, p = 0.005), with great heterogeneity (I2 = 95.01). The discrepancy of published results might result from the following differences among studies: gating strategies for Tregs, diagnostic criteria for SLE, and thresholds of SLEDAI chosen to differentiate between active and inactive SLE. In active SLE, Tregs gated based on CD25 alone showed lower pooled frequency than those gated by Foxp3+ or CD127low/∅. The percentages of Tregs in active SLE was significantly lower than that in controls when the enrolled SLE patients were diagnosed according to the 1997 modified criteria, whereas they were comparable to controls when diagnosed by the 1982 criteria; the higher threshold of SLEDAI score used to define active SLE tended to achieve a lower percentage of Tregs. The pooled absolute numbers of Tregs in active SLE were significantly decreased compared to those in controls (−1.328 ± 0.374, p < 0.001), but seemed to be unaffected by gating strategies. Suppression capacities of Tregs from active SLE patients showed no abnormalities based on the limited pooled data. Longitudinal monitoring of active SLE showed a significant decrease in Treg percentage at remission.Conclusions: This study implies that loss of Tregs may play a role in the pathogenesis of active SLE and help clarify contradictory Treg results in SLE.

Vaso-occlusive retinopathy and stage IV Lupus Nephritis; Primary manifestation of a 30 year old female

The diagnosis of systemic lupus erythematosus (SLE) depends on clinical evidence of renal, rheumatologic, cutaneous, and neurologic involvement, supported by serological markers. Ocular involvement is not included in diagnostic criteria of SLE. Vaso-occlusive retinopathy, as the primary manifestation of systemic lupus erythematosus (SLE), is relatively rare. We report a 30-year-old female, who suddenly suffered bilateral visual loss. Fundus examination revealed bilateral occlusive retinal vasculitis. She fulfilled the American College of Rheumatology criteria for diagnosing SLE; she had arthralgia, photosensitivity, positive ANA, high titre of anti-dsDNA, and proteinuria. A renal biopsy showed stage IV lupus nephritis. Treatment was initiated with pulse steroid and immunosuppressant. Best corrected visual acuity of both eye improved to finger counting after treatment. This case demonstrates that in patients with severe vaso-occlusive retinopathy, a generalized immunological disorder, like SLE, should be suspected.Bangladesh J Medicine Jan 2018; 29(1) : 36-40

Clinical presentation of human C1q deficiency: How much of a lupus?

Hereditary human C1q deficiency has been well described to be associated with high susceptibility for the development of systemic lupus erythematosus (SLE). The majority of subjects present a clinical syndrome closely related to SLE. However, limited information is available about the primary diagnosis and particular clinical manifestations of SLE in this specific subgroup of patients. In this review, we performed a comprehensive search of electronic databases up to November 2014 to identify and analyze reports on patients with C1q deficiency. We identified 71 C1q-deficient patients descending from 45 families that had been published. According to the American College of Rheumatology (ACR) diagnostic criteria for SLE 39/71 (55%) subjects could be classified as having SLE. Another 16/71 (22.5%) presented a SLE-like syndrome (defined as 3 positive ACR criteria) whereas in 16/71 (22.5%) no SLE could be diagnosed at time of publication. Symptoms began at a median age of 5 years, male and females being equally affected. Discoid rash (56% versus 10%, p<0.001) and oral ulcers (49% versus 24%, p<0.001) occurred significantly more frequent in C1q deficiency-associated SLE/SLE-like disease than in sporadic SLE, whereas arthritis (38% versus 84%, p<001) and anti-ds-DNA (18% versus 78%, p<0.001) occurred less frequently. Renal and neurological manifestations were found to occur similarly frequent. The severe course of disease in some patients seemed to be mostly due to severe infections at early ages and not in particular due to more aggressive SLE manifestations.

Autologous haematopoietic stem cell transplantation for systemic lupus erythematosus: data from the European Group for Blood and Marrow Transplantation registry

Patients with systemic lupus erythematosus (SLE) refractory to conventional immunosuppression suffer substantial morbidity and mortality due to active disease and treatment toxicity. Immunoablation followed by autologous stem cell transplantation (ASCT) is a novel therapeutic strategy that potentially offers new hope to these patients. This retrospective survey reviews the efficacy and safety of ASCT in 28 SLE patients from eight centres reported to the European Group for Blood and Marrow Transplantation (EBMT) registry between 2001 and 2008. Median disease duration before ASCT was 52 (nine to 396) months, 25/28 SLE patients (89%) were female, age 29 (16-48) years. At the time of ASCT, eight (one to 11) American College of Rheumatology (ACR) diagnostic criteria for SLE were present and 17 (60%) patients had nephritis. Peripheral blood stem cells were mobilized with cyclophosphamide and granulocyte-colony stimulating factor in 93% of patients, and ex vivo CD34 stem cell selection was performed in 36%. Conditioning regimens were employed with either low (n = 10) or intermediate (18) intensities. With a median follow-up of 38 (one to 110) months after ASCT, the five-year overall survival was 81 ± 8%, disease-free survival was 29 ± 9%, relapse incidence (RI) was 56 ± 11% and non-relapse mortality was 15 ± 7%. Graft manipulation by CD34+ selection was associated with a lower RI (p = 0.001) on univariate analysis. There were five deaths within two years after ASCT: three caused by infection, one by secondary autoimmune disease and one by progressive SLE. Our data further support the concept of immunoablation and ASCT to re-induce long-term clinical and serologic remissions in refractory SLE patients even in the absence of maintenance therapy. This study also suggests a beneficial effect of ex vivo graft manipulation on prevention of relapses post-transplantation in SLE.

Evaluation of autoantibody profiles in a cohort of new-onset pediatric systemic lupus erythematosus patients using autoantigen microarray technology

Purpose Systemic lupus erythematosus (SLE) is characterized by the production of autoantibodies directed against many highly-conserved nuclear antigens. 15% of SLE patients develop disease in childhood or adolescence, and pediatric (pSLE) patients often have more severe disease onset and organ system involvement. Antigen microarray technology allows the comprehensive analysis of autoantibodies directed against hundreds of antigens with minimal amounts of sera. The purpose of this study was to characterize autoantibody profiles in a prospective cohort of new-onset pSLE patients at onset and within 6-9 months of disease onset and to determine if autoantibody profiles differentiate patients with distinct clinical manifestations such as nephritis. Methods New-onset pediatric rheumatology patients meeting the revised ACR diagnostic criteria for SLE were eligible for this study. The study was approved by the Stanford University Institutional Review Board and informed consent was obtained prior to participation in the study. Sera from 51 pSLE patients and 17 healthy age- and sex-matched controls were evaluated using an 1128-feature antigen microarray manufactured with approximately 130 antigens. Demographic and clinical data from corresponding clinic visits were collected. Microarrays were probed with 1:200 dilutions of serum and a Cy5-conjugated goat-antihuman IgG secondary antibody, scanned with a GenePix 4000 scanner, and analyzed using GenePix 6.1 software to determine median fluorescence intensity minus background for each antigen. Significance Analysis of Microarrays (SAM) software was used to determine differences in autoantibody expression between pSLE patients and controls, within the pSLE group over time, and between pSLE patients with and without nephritis. Results SAM identified increased reactivity against more than 50 autoantigens in new-onset pSLE patients compared to controls, with a false discovery rate (FDR) of less than 1%. Reactivity against many of these antigens decreased significantly by 6-9 months from disease onset. Subgroup analysis comparing patients with class III or IV Iupus nephritis to patients without significant nephritis demonstrated increased reactivity against several autoantigens including double stranded DNA, C1q and several types of collagen in patients with nephritis. Conversely, patients without significant nephritis had increased reactivity against other antigens including heat shock proteins and desmoglein. Conclusion New-onset pSLE patients have a broad spectrum of antibodies directed against many autoantigens, including those not classically associated with SLE. In addition, preliminary subgroup analysis of pSLE patients with and without nephritis revealed distinct autoantibody profiles between the two groups. Further studies are currently underway to validate these findings and evaluate additional subgroups of patients with various clinical manifestations. Ultimately, autoantibody profiles may identify important biomarkers for more accurate diagnosis, prognostication, and treatment of pSLE patients.

Complete Remission of Human Parvovirus B19 Associated Symptoms by Loxoprofen in Patients with Atopic Predispositions

Two cases of women in their thirties with past histories of atopic dermatitis and allergic rhinitis developed a low grade fever, followed by a butterfly-shaped erythema, swelling of their fingers, and polyarthralgia. Despite such symptoms that overlap with those of systemic lupus erythematosus (SLE), the diagnostic criteria for SLE were not fulfilled. Due to positive results for human parvovirus B19 (HPV-B19) IgM antibodies in the serum, diagnoses of HPV-B19 infection were made in both cases. Although acetaminophen failed to improve their deteriorating symptoms, a nonsteroidal anti-inflammatory drug (NSAID), loxoprofen, completely removed the symptoms immediately after the administration. In those cases, since the patients were predisposed to atopic disorders, an increased immunological response based on the lymphocyte hypersensitivity was likely to be involved in the pathogenesis. The immunomodulatory property of NSAID was thought to repress such lymphocyte activity and thus provided a rapid and sustained remission of the disease.

The Association of IL-12b Polymorphisms with Systemic Lupus Erythematosus in Chinese Han Population

Background. Systemic lupus erythematosus (SLE) is a complex immune disease. The genetic variation in the IL-12b gene was found to associate with SLE in Caucasian population. In this study, we examined this association in Chinese Han population by a recently developed method, unlabeled probe-based high resolution melting analysis. Methods. A total of 297 SLE patients and 351 controls were recruited. Unlabeled probe-based high resolution melting analysis (HRMA) was used in genotyping. Results. Statistically significant differences were observed in both genotype and allele frequencies for rs6887695 in the SLE patients as compared with the controls. Minor allele (C) of rs6887695 (P = 0.031, OR 0.78, [95% CI 0.63-0.98]) was found to be protective against SLE. The association of SNP rs6887695 with the diagnostic criteria of SLE was also examined. Minor allele (C) exerts protective effect on the incidence of arthritis (P = 0.013, OR = 0.65, 95% CI = 0.47-0.92) and abnormalities of antinuclear antibody (P = 0.022, OR = 0.68, 95% CI = 0.49–0.95). IL-12b SNPs were irrelevant to other diagnostic criteria of SLE. Summary. Polymorphisms of rs6887695 in IL-12b gene were associated with disease risk, as well as arthritis and antinuclear antibody synthesis, of systemic lupus erythematosus in Chinese population.

Chorea as the First Sign in a Patient with Elderly-Onset Systemic Lupus Erythematosus

The case of an elderly patient who had chorea as an initial symptom of systemic lupus erythematosus (SLE) accompanied by antiphospholipid syndrome (APS) is reported. A 68-year-old woman suddenly developed chorea of her left arm and leg. Magnetic resonance imaging (MRI) of the brain demonstrated a focal lesion in the right caudate head, which showed hyperintensity on fluid-attenuated inversion recovery and diffusion-weighted imaging. This condition was thought to be a common form of vascular chorea, which is likely to occur in elderly individuals; however, the laboratory data of this patient finally fulfilled the diagnostic criteria of SLE and APS. Physicians should be careful in diagnosing elderly individuals simply as having a vascular chorea because this symptom can be the initial manifestation of SLE or APS.

Is there a link between Escherichia coli septicemia and the onset of systemic lupus erythematosus?

We read with great interest the contribution by Bazso et al. [1]. They reported the case of a 12-year-old girl with juvenile idiopathic arthritis (JIA) who developed systemic lupus erythematosus (SLE) after 10 years and speculated a few hypotheses of “shared autoimmunity,” the complete dysregulation of the immune system, and the side eVect of the anti-TNF therapy. However, we would like to suggest a diVerent and possible pathomechanism of the overlapping JIA and SLE case. The patient presented Escherichia coli (E. coli) septicemia at the age of 22 and subsequently developed clinical, immunological, and pathological manifestations fulWlling the diagnostic criteria of SLE within the following 8 weeks. SigniWcantly, Uthman et al. [2] also reported a case of SLE following E. coli sepsis in a 72-year-old woman, postulating three plausible pathomechanisms. Firstly, Robertson and Pisetsky [3] reported that Wve of the eight patients with E. coli bacteremia demonstrated increased levels of antibodies to single-stranded (ss) DNA from E. coli. Secondly, Gilkeson et al. [4] showed that normal mice immunized with ssDNA derived from E. coli developed an immune-mediated proliferative glomerulonephritis secondary to renal deposition of anti-DNA antibodies as in SLE. Thirdly, E. coli is known to produce heat shock protein (HSP), and Schultz et al. [5] suggested a role of HSP in the pathogenesis of rheumatic diseases like SLE. The host initially reacts to microbial infections with an enhanced cellular and humoral response to the microbial HSP, and cross-reactivity may occur with the HSP of the stressed host because of structural similarities to the microbial HSP [5]. Therefore, there is a possibility that E. coli septicemia might play a critical role as an inducer in the development of SLE in the Bazso et al.’s patient. However, further studies are necessary to elucidate the exact relationship and molecular pathogenic pathway between the JIA and SLE overlap in the future.

Aplicação dos critérios diagnósticos do lúpus eritematoso sistêmico em pacientes com hanseníase multibacilar

Systemic lupus erythematosus (SLE) is a chronic inflammatory disease that affects multiple organs or systems. There is no pathognomonic clinical or laboratory test sensitive and specific enough for a specific diagnosis. The criteria proposed by the American College of Rheumatology (ACR), as modified in 1997, are used for the diagnosis. The presence of four or more criteria presents sensitivity and specificity of 96%. However, these diagnostic criteria for SLE may have lower specificity in areas that are endemic for chronic infectious diseases, such as Brazil (endemic for leprosy), which may have similar clinical and laboratory manifestations. A prevalence study was conducted, applying the SLE criteria to patients with recently diagnosed multibacillary leprosy who were registered at the leprosy outpatient clinic, Department of Dermatology, Federal University of Pernambuco (UFPE), during the data gathering period. The specificity and the number of false positives in this group were calculated. One hundred patients were included. The prevalences of some of the SLE criteria were high. The criteria with the highest prevalence were malar erythema (44%), arthritis (23%), photosensitivity (29%), lymphopenia (19%) and presence of antiphospholipid antibodies, including immunological criteria (20%). The specificity found (84%) was lower than the specificity allocated to the criteria in 1997 by the ACR. Diseases in our setting, such as leprosy in multibacillary forms, mimic the clinical and laboratory characteristics of SLE, and thus physicians need to be aware of the realities of local infectious diseases before affirming a definitive diagnosis of SLE.

The association between single-nucleotide polymorphisms of NCF2 and systemic lupus erythematosus in Chinese mainland population

Systemic lupus erythematosus (SLE) is a complex immune disease. The genetic variation in the NCF2 gene was found to associate with SLE in US and European populations. However, the association of rs10911363 with SLE was not extensively studied in Chinese mainland population. A total of 488 SLE patients and 380 controls were recruited. Unlabeled probe-based high-resolution melting analysis (HRMA) was used in genotyping. HRMA with unlabeled probe successfully distinguished all genotypes. Neither genotype nor allele frequencies of single-nucleotide polymorphism (SNP) rs10911363 showed statistically significant differences between SLE patients and controls. The association of SNP rs10911363 with the diagnostic criteria of SLE was also examined. Minor allele (G) of rs10911363 was found to significantly associate with the incidence of arthritis (p = 0.024, odds ratio (OR) = 1.35, and 95% confidence interval (CI) = 1.04-1.75) and increased abnormalities of antinuclear antibody (p = 0.002, OR = 1.51, and 95%CI = 1.17-1.95) and anti-DNA (p = 0.013, OR = 1.40, and 95%CI = 1.07-1.82). Polymorphisms of rs13277113 in NCF2 gene were associated with arthritis and autoantibody production, but not disease risk, of SLE in Chinese population.

Polymorphisms of PXK are associated with autoantibody production, but not disease risk, of systemic lupus erythematosus in Chinese mainland population

Systemic lupus erythematosus (SLE) is a complex immune disease. The genetic variation in the PXK gene was found to associate with SLE in Caucasian populations. However, the association of rs6445975 with SLE has not been extensively studied in a Chinese mainland population. A total of 288 SLE patients and 357 controls were recruited. Unlabeled probe-based high-resolution melting analysis (HRMA) was used in genotyping. HRMA with unlabeled probe successfully distinguished all genotypes. Neither genotype nor allele frequencies of SNP rs6445975 showed statistically significant differences between SLE patients and controls. The association of SNP rs6445975 with the diagnostic criteria of SLE was also examined. No obvious association was observed between rs6445975 and the incidence of clinical symptoms. However, the minor allele (G) of rs6445975 was found to significantly associate with increased abnormalities of anti-Smith (p = 0.004, odds ratio (OR) = 1.95, 95% confidence interval (CI) = 1.22-3.09), anti-Ro (p = 0.015, OR = 1.69, 95% CI = 1.10-2.58), anti-La (p = 0.008, OR = 1.86, 95% CI = 1.17-2.93) and C3C4 (p = 0.007, OR = 1.79, 95% CI = 1.17-2.74). Polymorphisms of rs6445975 in the PXK gene were associated with autoantibody production, but not disease risk, of systemic lupus erythematosus in a Chinese population.

Reply

Editor, We would like to thank Dr Weekitt Kittisupamongkol (2009) for his comments regarding the diagnostic criteria for systemic lupus erythematosus (SLE) used in our paper (Lin et al. 2009). We agree that the diagnostic criteria were updated in 1997. The diagnoses of the underlying disease (SLE) in our patients were made before and after the optic neuritis attacks by the rheumatologists at our hospital. We reconfirmed the diagnoses of SLE in these patients with the rheumatologists. Four of our patients with optic neuritis were diagnosed with SLE before 1997, according to the 1982 revised criteria (Tan et al. 1982). The other four patients were diagnosed with SLE after 1997 using the 1997 update of the revised criteria (Hochberg 1997). The positive findings in our eight patients did not include the positive lupus erythematosus (LE) cell preparation or positive antiphospholipid antibody. The prevalence of SLE-associated optic neuritis in our study would not be changed by applying these two diagnostic criteria for SLE.

Pulmonary involvement of systemic lupus erythematosus: analysis of 90 necropsies

Pleuropulmonary manifestations of systemic lupus erythematosus (SLE) have been reported to be of variable prevalence, depending on the diagnostic methods used. The objective of this study was to determine the anatomopathological prevalence and the nature of lung involvement associated with SLE and to define if there were differences in the grade and type of pulmonary involvement in patients who had died at different time periods, before or after 1996. Complete autopsy studies of 90 patients with SLE diagnosis carried out between 1958 and 2006 and their clinical records were studied. All patients fulfilled the American College of Rheumathology (ACR) diagnostic criteria for SLE. Two groups of patients were analyzed: patients who had died before 1996 and those deceased in 1996-2006. Some pleuropulmonary involvement was detected in 97.8% of the autopsies. The most frequent findings were pleuritis (77.8%), bacterial infections (57.8%), primary and secondary alveolar haemorrhages (25.6%), followed by distal airway alterations (21.1%), opportunistic infections (14.4%) and pulmonary thromboembolism (7.8%), both acute and chronic. No cases of acute or chronic lupus pneumonitis were found. Opportunistic lung infections were invasive aspergillosis, disseminated strongyloidiasis, mucormicosis and Pneumocystis carinii. Only three of 23 patients with alveolar haemorrhage showed capillaritis. The four patients with primary pulmonary hypertension (PHT) had plexiform lesions. Deceased patients' age at death (46.09 +/- 11.01 vs 30.3 +/- 11.5 years, P < 0.0001) as well as survival time from diagnosis date (11.8 +/- 11.2 vs 4.4 +/- 4.9 years, P < 0.0001) in the second time period evaluated were significantly higher. However, there were no statistically significant differences in the prevalence of any of the pulmonary manifestations. Sepsis was considered the major cause of death without significant differences in both groups. Our results show that pulmonary manifestations directly caused by systemic lupus erythematosus are very uncommon and that their prevalence has not changed in the past 10 years. Pulmonary infection is still the most frequent affection, and it is an important cause of death in patients with lupus.

The Neurologic Manifestations of Systemic Lupus Erythematosus

Often patients with systemic lupus erythematosus (SLE) suffer from neurologic symptoms and at times the diagnosis of SLE is made after patients present with neurologic events. Although diagnostic criteria for SLE have included seizures and psychosis, there are 19 recognized neurologic manifestations of the disease. The various neurologic manifestations of SLE are reviewed as are the various treatment options. The pathophysiology of the major syndromes is reviewed. Neurologists need to be familiar with the various ways SLE can present and the various neurologic complications known lupus patients can experience. Because of the various treatment options available, rapid recognition of these syndromes is critical.