Positive Lupus Erythematosus Research Articles

Clinical features and outcomes of patients with antineutrophil cytoplasmic antibody-positive systemic lupus erythematosus

Purpose To investigate the clinical characteristics, pathological features, and outcomes of patients with antineutrophil cytoplasmic antibody (ANCA)-positive systemic lupus erythematosus (SLE) in northwest China. Methods This retrospective study included 491 patients with SLE tested for ANCA antibodies and 171 patients with ANCA-associated vasculitis (AAV) as controls. Subgroup analysis limited to those with renal involvement, and by ANCA antibody subtype (PR3 vs MPO). To compare the proteinuria remission rates between ANCA-positive and ANCA-negative lupus nephritis (LN) groups, a logistic regression model was used for propensity score matching based on age, hemoglobin, and baseline estimated glomerular filtration rate (eGFR). Results Compared to ANCA-negative SLE (n = 442), ANCA-positive SLE (n = 46) occur in older patients; however, these patients were younger than those with AAV (n = 167). The eGFR of patients with ANCA-positive LN (n = 25) was higher than that of patients having AAV with renal involvement (n = 56) but lower than that of patients with ANCA-negative LN (n = 163). Patients with SLE who had MPO-ANCA (n = 16) had higher levels of serum creatinine compared to those with PR3-ANCA (n = 30) (156.5 µmol/L vs. 45.5 µmol/L, p = 0.005). During the follow-up period, the remission rate of proteinuria in patients with ANCA-positive LN was lower than that of patients with ANCA-negative LN (50% vs. 75%, p = 0.008). Conclusion Patients with ANCA-positive LN may have worse baseline renal function and lower protein remission rates compared to patients with ANCA-negative LN. ANCA titers should be regularly monitored throughout the follow-up period in patients with SLE, especially in cases of renal involvement.

E081 Effectiveness of intravenous belimumab in patients with systemic lupus erythematosus at a UK tertiary referral centre

Abstract Background/Aims Belimumab was approved in the United Kingdom (UK) in 2016 as add-on therapy for patients with auto-antibody positive systemic lupus erythematosus (SLE) and high disease activity refractory to standard treatment. Data regarding the effectiveness of belimumab outside clinical trials has been published in worldwide SLE cohorts but not the UK. Here, we describe the efficacy of add-on belimumab therapy in SLE patients from a large London tertiary centre. Methods We performed a retrospective, intention-to-treat analysis of clinical and biochemical outcomes, at 6 and 12 months, post receipt of at least 1 infusion of belimumab in SLE patients attending Guy’s and St Thomas’ NHS Trust (April 2013-November 2021). Outcomes assessed included: the Safety of Estrogen in Lupus Erythematosus National Assessment-SLE Disease Activity Index (SELENA-SLEDAI), anti-dsDNA ELISA titre, complement C3 and C4 titres, urine protein:creatinine ratio (uPCR) and daily prednisolone dose. Analyses performed using SPSS statistics 27. Results 63 patients (56 female [88.9%], median age 38 years, self-reported race/ ethnicity; 24 Black [38.1%], 24 White [38.1%], 13 Asian [20.6%]) with SLE as defined by the 1997 ACR criteria were included. An 8-point reduction in median SELENA-SLEDAI was evident at 6 months after commencing belimumab, which increased to 9 points at 12 months (n = 28, p < 0.001)(Table 1). Improvements in C3/ C4 titres were also observed at 6 months (0.13-point and 0.04-point increase in median C3/ C4 titres respectively, n = 45, p < 0.001). At 12 months, significant reductions in median anti-dsDNA titre (16.50-point reduction, n = 42, p < 0.001) and median daily prednisolone dose were evident (2.50mg reduction, n = 51, p < 0.001). No change in uPCR was noted (n = 23, p = 0.956). 40/47 (85%) patients with a SELENA-SLEDAI score recorded at 6 months achieved the National Institute for Health and Care Excellence (NICE) defined target for a >/= 4-point reduction in SELENA-SLEDAI. No significant associations between baseline demographic variables and SELENA-SLEDAI score at 6 months, or prednisolone dose at 12 months, were evident. Conclusion We observed a significant improvement in SLE disease activity as determined by SELENA-SLEDAI score measured at 6 and 12 months after starting belimumab, and a significant steroid-sparing effect at 12 months. These findings are similar to published worldwide data. Disclosure G. Mallett: None. C. Hession: None. M. Fernando: Other; MF has received speaker fees from GlaxoSmithKline (GSK) and Union Chimique Belge (UCB), as well as conference fees from GSK.

Efficacy of belimumab combined with rituximab in severe systemic lupus erythematosus: study protocol for the phase 3, multicenter, randomized, open-label Synbiose 2 trial

BackgroundBelimumab, an anti-B-cell activating factor antibody, is approved for the treatment of auto-antibody positive systemic lupus erythematosus with a high degree of disease activity. Anti-CD20 B cell depletion with rituximab is used in refractory SLE as well, although with variable responses. We hypothesized that incomplete B cell depletion, related to a surge in BAFF levels following rituximab treatment, can cause ongoing disease activity and flares. The Synbiose 1 study primarily focused on immunological effects and shows the preliminary clinical benefit of combined rituximab and belimumab in SLE. The Synbiose 2 study will evaluate the clinical efficacy of combining belimumab with rituximab in patients with severe SLE, allowing the tapering of prednisolone and mycophenolate.MethodsSynbiose 2 is a phase 3, multicenter, randomized, controlled, open-label 2-year clinical trial. Seventy adults with severe SLE including lupus nephritis will be randomized 1:1 to receive either standard of care consisting of prednisolone and mycophenolate as induction and maintenance treatment, or belimumab and rituximab combined with standard of care as induction treatment, followed by prednisolone and belimumab as maintenance treatment. The primary objective is to assess whether combined B cell therapy will lead to a reduction of treatment failure. Secondary endpoints are complete and partial clinical and renal response and the improvement of SLE-specific autoimmune phenomena. Safety endpoints include the incidence of adverse events, with a special interest in infections.DiscussionThe Synbiose 2 trial is the first multicenter phase 3 clinical trial investigating combined B cell targeted therapy in SLE, including lupus nephritis. The outcome of this study will provide further evidence for the clinical efficacy of this new treatment strategy in severe SLE.Trial registrationClinicalTrials.gov NCT03747159. Registered on 20 November 2018.

Demodex positive discoid lupus erythematosus: Is it a separate entity or an overlap syndrome?

Discoid lupus erythematosus (DLE) is a chronic inflammatory erythematous skin disease that can be triggered by several factors. Rosacea is another skin disease that causes facial redness and tenderness. Demodex mites have been reported in rosacea and DLE patients commonly in the literature. These two diseases can be seen concomitant, mimic each other clinically and share common possible etiologic factors. To assess demodex mite infestation in both clinical and histopathological findings in DLE patients. We retrospectively evaluated the files of 42 patients with DLE who had been diagnosed DLE based on clinical and histopathological findings between August 2018 and August 2019. Demodex positivity was detected 50% of patients (n = 21). Neutrophile percentages in the dermal and perivascular area were higher in the demodex positive patients (4.43%) than in the Demodex negative patients (2.19%). The intensity of demodex mites correlated positively with dermal neutrophile percentages. ANA was negative in 29 patients (69%) and positive in 13 patients (31%). Anti-dsDNA was negative in serology and follicular plugging was positive in histopathology in all 42 patients (100%). This was a retrospective study. DLE and rosacea share common features in etiopathogenesis and clinical presentation. Inflammation and exacerbations caused by the demodex mites may increase the clinical severity of DLE. Although the position of demodex mites in DLE etiopathogenesis is not known exactly, the presence of high demodex in DLE patients has been determined. Standard skin surface biopsy can be a routine procedure for the evaluation of DLE patients in daily clinical practice.

RAGING REGURGITATION RAISES SPECTER OF MYSTERIOUS ENDOCARDITIS

Libman-Sacks endocarditis is a nonbacterial thrombotic endocarditis (NBTE) associated with anti-cardiolipin positive systemic lupus erythematosus and anti-phospholipid antibody syndrome. Lesions typically affect the mitral and aortic valves leading to regurgitation. A 44-year-old woman with Crohn's

Apoptosis in patients with primary antiphospholipid antibody syndrome.

Dysregulated apoptosis has been implicated in autoimmune diseases. In the present study, we investigated the apoptosis-related cytokines and apoptosis in patients with primary antiphospholipid syndrome (pAPS). We prospectively recruited 12 pAPS patients, 17 antiphospholipid antibody (APA)-positive systemic lupus erythematosus (SLE) patients without APS manifestations (APA+ SLE), 13 SLE patients with secondary APS (APS+ SLE) and 10 healthy controls (HCs). Plasma levels of soluble apoptosis-inducing ligands and cytokines, and the expression levels of apoptosis-inducing ligands in peripheral blood mononuclear cells, were determined. In addition, blood lymphocytes/monocytes apoptosis were determined in six pAPS patients and six HCs, using flow cytometric analysis of caspase 3, 8 and 9 activities. There was a trend toward higher plasma levels of soluble tumor necrosis factor (TNF)-related apoptosis-inducing ligand (sTRAIL), interleukin-10 (IL-10) and TNF-α in pAPS patients when compared with HCs. We also observed higher plasma levels of IL-10 and TNF α in APA+ SLE and APS+ SLE patients when compared with HCs. However, there was no significant difference in blood lymphocytes/monocytes apoptosis between pAPS patients and HCs. There was a trend toward elevated plasma levels of sTRAIL, IL-10 and TNF-α, but no evidence for dysregulated apoptosis in pAPS patients.

A Case of MPO-ANCA Positive Systemic Lupus Erythematosus Developed into Acute Kidney Injury

During the course of treatment for rheumatoid arthritis, anti-DNA antibody was detected, and systemic lupus erythematosus was diagnosed, who was subsequently treated with abatacept—and remission was achieved. Rebound of elevated anti-DNA antibody and urinary abnormalities were noted with MPO-ANCA positivity. Renal biopsy revealed lupus nephritis; followed by the development of rapidly progressive glomerulonephritis, which required apheresis (double filtration plasmapheresis) therapy, although no response was achieved. However, plasma exchange therapy was highly effective. These results suggest that MPO-ANCA-positive lupus nephritis can become severe and require intensive treatment in accordance with the treatment for severe vasculitis.

Type I Immune Response Induces Keratinocyte Necroptosis and Is Associated with Interface Dermatitis

Interface dermatitis is a characteristic histological pattern that occurs in autoimmune and chronic inflammatory skin diseases. It is unknown whether a common mechanism orchestrates this distinct type of skin inflammation. Here we investigated the overlap of two different interface dermatitis positive skin diseases, lichen planus and lupus erythematosus. The shared transcriptome signature pointed toward a strong type I immune response, and biopsy-derived T cells were dominated by IFN-γ and tumor necrosis factor alpha (TNF-α) positive cells. The transcriptome of keratinocytes stimulated with IFN-γ and TNF-α correlated significantly with the shared gene regulations of lichen planus and lupus erythematosus. IFN-γ, TNF-α, or mixed supernatant of lesional T cells induced signs of keratinocyte cell death in three-dimensional skin equivalents. We detected a significantly enhanced epidermal expression of receptor-interacting-protein-kinase 3, a key regulator of necroptosis, in interface dermatitis. Phosphorylation of receptor-interacting-protein-kinase 3 and mixed lineage kinase domain like pseudokinase was induced in keratinocytes on stimulation with T-cell supernatant-an effect that was dependent on the presence of either IFN-γ or TNF-α in the T-cell supernatant. Small hairpin RNA knockdown of receptor-interacting-protein-kinase 3 prevented cell death of keratinocytes on stimulation with IFN-γ or TNF-α. In conclusion, type I immunity is associated with lichen planus and lupus erythematosus and induces keratinocyte necroptosis. These two mechanisms are potentially involved in interface dermatitis.

Antiphospholipid antibodies and non-thrombotic manifestations of systemic lupus erythematosus

Objectives The aim of this study was to investigate the association between antiphospholipid antibodies and non-thrombotic and non-gestational manifestations of systemic lupus erythematosus. Methods Systemic lupus erythematosus patients with persistently positive antiphospholipid antibodies or lupus anticoagulant were identified and grouped as systemic lupus erythematosus with antiphospholipid syndrome (SLE-APS), systemic lupus erythematosus with positive antiphospholipid antibodies/lupus anticoagulant without antiphospholipid syndrome (SLE-aPL), and systemic lupus erythematosus with negative aPLs (SLE-No aPL). Groups were compared in terms of non-thrombotic systemic lupus erythematosus manifestations and laboratory features retrospectively. Results A total of 150 systemic lupus erythematosus patients, 26 with SLE-APS, 25 with SLE-aPL, and 99 with SLE-No aPL, were identified. Livedo reticularis, neurologic involvement, and thrombocytopenia were more common in antiphospholipid antibody positive systemic lupus erythematosus cases. Malar rash, arthritis, and pleuritis were more common in the SLE-No aPL, SLE-APS, and SLE-aPL groups, respectively. Positivity rates and titers of specific antiphospholipid antibodies did not differ between the SLE-APS and SLE-aPL groups. Conclusions Presence of antiphospholipid syndrome or persistent antiphospholipid antibodies may be related to non-thrombotic and non-gestational systemic lupus erythematosus manifestations. Patients with systemic lupus erythematosus plus antiphospholipid syndrome and persistent antiphospholipid antibodies without antiphospholipid syndrome also differ in terms of systemic lupus erythematosus manifestations.

Medicine Based Evidence for Individualized Decision Making: Case Study of Systemic Lupus Erythematosus

BackgroundTo guide management decisions for an index patient, evidence is required from comparisons between approximate matches to the profile of the index case, where some matches contain responses to treatment and others act as controls. MethodsWe describe a method for constructing clinically relevant histories/profiles using data collected but unreported from 2 recent phase 3 randomized controlled trials assessing belimumab in subjects with clinically active and serologically positive systemic lupus erythematosus. Outcome was the Systemic lupus erythematosus Responder Index (SRI) measured at 52 weeks. ResultsAmong 1175 subjects, we constructed an algorithm utilizing 11 trajectory variables including 4 biological, 2 clinical, and 5 social/behavioral. Across all biological and social/behavioral variables, the proportion of responders based on the SRI whose value indicated clinical worsening or no improvement ranged from 27.5% to 42.3%. Kappa values suggested poor agreement, indicating that each biological and patient-reported outcome provides different information than gleaned from the SRI. ConclusionThe richly detailed patient profiles needed to guide decision-making in clinical practice are sharply at odds with the limited information utilized in conventional randomized controlled trial analyses.

Association between rs2431697 T allele on 5q33.3 and systemic lupus erythematosus: case-control study and meta-analysis.

rs2431697 is located on 5q33.3, between pituitary tumor-transforming gene 1 and miR-146a. Several studies have estimated the association between rs2431697 and systemic lupus erythematosus risk. However, the results were inconsistent. A case-control study was carried out to explore the association between rs2431697 and systemic lupus erythematosus risk in a central Chinese population. Meta-analyses combining present with previous studies were conducted to further explore the association. Our case-control study included 322 cases and 353 controls. rs2431697 T allele was associated with increased risk of systemic lupus erythematosus (odds ratios (ORs) = 1.461, 95 % confidence intervals (CI) 1.091–1.957, P = 0.011). The association was stronger between T allele and the risk of anti-double-stranded DNA (dsDNA)-positive systemic lupus erythematosus (OR = 2.510, 95 % CI 1.545–4.077, P < 0.001). The meta-analyses included 8648 systemic lupus erythematosus patients and 10947 controls. rs2431697 T allele had an overall OR of 1.262 (95 % CI 1.205–1.323, P < 0.001) under fixed-effects model. After stratified by ethnicity, I2 reduced from 24.3 to 0 %. T allele had an OR of 1.213 (95 % CI 1.145–1.284, P < 0.001) in European descendant and 1.365 (95 % CI 1.259–1.480, P < 0.001) in Asian under fixed-effects model. Data on women were also extracted, and T allele had an OR of 1.337 (95 % CI 1.162–1.539, P < 0.001) under random-effects model. The pooled ORs were not influenced by each study in sensitivity analyses. There were no publication biases observed in these analyses. The results from our case-control study and the meta-analyses indicate that rs2431697 T allele significantly associates with the increased risk of systemic lupus erythematosus.Electronic supplementary materialThe online version of this article (doi:10.1007/s10067-015-3045-4) contains supplementary material, which is available to authorized users.

Endocardite por Streptococcus gallolyticus em portadora de lúpus eritematoso sistêmico: avaliação pelo ecocardiograma tridimensional

ABSTRACTA 42 year-old woman was referred to our hospital with a history of fever and poor general status for the last 30 days. She presented tachycardia and a systolic apical murmur. Laboratory tests revealed leukocytosis of 13,100/mL, hemoglobin of 8.4g/dL and positive systemic lupus erythematosus antibodies (anti-Ro/SSA, anti-La/SSB, anticardiolipin, and antinuclear antibodies); blood culture was positive for Streptococcus gallolyticus. Three-dimensional transesophageal echocardiography was performed and revealed multiple mitral valve vegetations, with leaflet perforation and important mitral regurgitation, as well as large aortic vegetation, with cusp perforation and severe regurgitation. Additionally, a small vegetation was observed on the tricuspid valve, which presented moderate regurgitation. Three-dimensional transesophageal echocardiography provides appropriate visualization of complications resulting from infectious endocarditis.

Belimumab for the management of systemic lupus erythematosus

Introduction: In 2011, Belimumab, a fully humanized monoclonal antibody against B lymphocyte stimulator, became the first biological agent to be licensed by the United States Food and Drug Administration (FDA) and the European Medicines Evaluation Agency (EMEA) for the use in auto-antibody positive adult Systemic Lupus Erythematosus (SLE).Areas covered: An overview of the clinical trial data, review of the medical and scientific literature following a MEDLINE search forms the basis of this expert opinion on biological therapy review. The Belimumab International SLE Study Phase III randomized placebo-controlled trials, BLISS-52 and BLISS-76, met the primary endpoint based on the SLE responder index (SRI) at week 52. The trials reported that belimumab 10 mg/kg infusions with standard therapy significantly reduced SLE disease activity compared with placebo with standard therapy.Expert opinion: The clinical efficacy, safety and tolerability of belimumab indicates a potential role for this drug in achieving disease control, reducing severity of recurrent flares, reducing morbidity and in the long-term achieving a positive long-term impact on quality of life. Belimumab is now being introduced in clinical practice and over the next 5 years data on its use outside the clinical trials will determine its place in SLE management.

Pediatric Systemic Lupus Erythematosus: More Than a Positive Antinuclear Antibody

Based on strong research evidence and consensus, the most common disease manifestations at diagnosis of pSLE are constitutional symptoms, arthritis, and malar rash. Based on some research evidence and consensus, patients with pSLE tend to have major organ system involvement (renal/central nervous system) and a greater disease burden compared with adults. Despite these findings, mortality is low. Based on some research evidence and consensus, the diagnosis of pSLE is unlikely if the ANA is negative, and most patients with SLE have a positive ANA at a titer ≥1:160. Based on strong research evidence, both MMF and cyclophosphamide can be used for induction therapy in class III and IV lupus nephritis. Based on strong research evidence, patients with SLE and anticardiolipin antibodies or LA have a two and six times greater risk of venous thrombosis, respectively, compared with patients with SLE without antiphospholipid antibodies. Based on strong research evidence, patients with pSLE have a higher risk for subclinical atherosclerosis when there is weight-adjusted prednisone use, azathioprine use, increasing age, male gender, high BMI, abnormal creatinine clearance, and elevated lipoprotein(a) levels.

Pericarditis as initial clinical manifestation of systemic lupus erythematosus in a girl

The most common diagnostic features of systemic lupus erythematosus (SLE) include mucocutaneous lesions, nephritis, arthritis and haematological disorder. Serositis in the form of pericarditis is an uncommon first-line clinical manifestation. We report on an 11-year-old Nigerian girl who presented recurrently with pericarditis as the initial clinical manifestation of SLE. Other diagnostic clinical features, namely malar rash and polyarthritis, evolved sequentially over time. Diagnostic laboratory features were lymphopenic leukopenia, a positive lupus erythematosus cell preparation and positive lupus anticoagulant tests. She responded well to non-steroidal anti-inflammatory and immunosuppressive therapy. Unexplained pericarditis in any child should warrant immediate screening for SLE.

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.

Lupus: improving long-term prognosis

Over recent decades short- and medium-term survival has greatly improved in patients affected with systemic lupus erythematosus, but long-term prognosis still remains poor mainly due to complications of the disease and/or its treatment. To improve long-term prognosis in systemic lupus erythematosus, we should try to adopt, early in the disease course, strategies that can contribute to reducing long-term complications, including screening for and prophylaxis against infections, control of risk factors for atherosclerosis, and cancer surveillance. However, in patients with systemic lupus erythematosus all these preventive strategies are often not sufficient. Indeed, two important systemic lupus erythematosus-related factors play a relevant role in all these complications: severe disease manifestations, such as glomerulonephritis and central nervous system involvement, and corticosteroid and cyclophosphamide use. Therefore, to prevent long-term complications, we should try to control disease activity and severity using the lowest effective dosage of these drugs. Moreover, strategies directed at preventing clinical manifestations in asymptomatic antinuclear antibody-positive individuals or in antiphospholipid antibody-positive systemic lupus erythematosus patients, as well as at preventing severe manifestations in patients with mild systemic lupus erythematosus at the time of the diagnosis should be considered.

Plasma cell hepatitis in liver allografts: Variant of rejection or autoimmune hepatitis?

Plasma cell hepatitis in liver allografts: Variant of rejection or autoimmune hepatitis?

Padrões de imunofluorescência do fator antinuclear (FAN) em células HEp-2 de soros reagentes para anti-SSA/Ro

OBJECTIVE: to evaluate the pattern at immunofluorescence of the antinuclear antibodies (ANA) detected by the indirect immunofluorescence (IIF) technique in positive samples for anti-SSA/Ro autoantibody and the clinical associations. METHODS: a retrospective transversal study was performed in a period of two years where the all the solicitations of testing for the presence of anti-extractable nuclear antigen (anti-ENA) antibodies delivered to the SPC/HCPA were analyzed. We selected the positive samples and identified which autoantibodies were involved (anti-SSA/RO, anti-SSB/La, anti-RNP, anti-Sm and anti-Scl70) as well as the immunofluorescence patterns by ANA testing and the clinical associations found in the patients presenting anti-SSA/Ro positive serum. IIF was used for ANA using HEp-2 cells and hemagglutination for anti-ENA antibodies detection. RESULTS: 90 out of the 392 solicitations analyzed were anti-ENA positive, with a predominance of women (86/91 - 94%) and the mean age was 42 years old. The most frequent autoantibody was anti-SSA/Ro (61/90 - 67.8%) and all samples that were anti-SSA/Ro positive were also ANA positive. Speckled nuclear immunofluorescence was the most frequent ANA pattern (42/61 - 68.9%) among the anti-SSA/Ro positive samples and systemic lupus erythematosus was the most common clinical diagnosis (31/61 - 50.8%). CONCLUSION: ANA testing by IIF using HEp-2 cells proved to be a good screening test for the detection of anti-SSA/Ro antibodies, that showed a strong positive association to the speckled nuclear IIF pattern. As opposed to what has been described in the literature, there was no ANA negative among the anti-SSA/Ro positive samples. At least in our experience, these data question the cost-effectiveness of performing routine screening for anti-SSA/Ro antibodies in ANA negative samples by IIF testing.

False positive VDRL (BFP-STS) and systemic lupus erythematosus; new data in clinico-laboratory associations.

To investigate the relationships between clinical features and serological parameters in systemic lupus erythematosus (SLE) patients with false positive venereal disease research laboratory (VDRL) test (BFP-STS) and those without. The records of 74 patients with SLE were reviewed. These were divided on the basis of the presence of a biologically false positive serological test (BFP-STS) (n = 18) or its absence (n = 56). The clinical features and serological parameters were subsequently evaluated in each group. A biologically false positive serological test was found in 18 patients (24.5%). Clinically, they had a significantly higher frequency of malar rash (P < 0.02), hemolytic anaemia (P < 0.02) and SLE-related antiphospholipid (APL) syndrome (P < 0.02). Neuropsychiatric lupus was more frequent in the group without BFP-STS. Moreover, subacute cutaneous LE (SCLE), Raynaud's phenomenon, livedo reticularis (LR) and cardiopulmonary complications were present only in the latter group. There was no significant association between BFP-STS and other autoantibodies including Extractable nuclear antigen (ENAs), however, there was a significant association with C3 hypocomplementemia (P < 0.05). Half of the patients with BFP-STS were positive for anticardiolipin antibodies (aCL Abs) vs. 28% in the negative group (P = NS). The study suggests that the clinical and serological relevance of BFP-STS in lupus patients may vary from those described in relation to other antiphospholipid determinants. Our patients appeared to have sparing of certain major organ systems, yet there was a greater tendency to developing Antiphospholipid (APL) syndrome and hemolytic anaemia.