Systemic lupus erythematosus and antineutrophil cytoplasmic antibody–associated vasculitis: An emerging overlap syndrome with cutaneous manifestations

AbstractSystemic lupus erythematosus (SLE) and antineutrophil cytoplasmic antibody-associated vasculitis (AAV) are autoimmune diseases that often cause rapidly progressive glomerulonephritis, with neutrophil extracellular traps (NETs) involved in their pathogenesis. However, the involvement of NETs in the renal damage caused by SLE/AAV overlap syndrome has not been clarified yet. In this study, we detected renal deposition of NETs in a patient with SLE/AAV overlap syndrome. In addition, a significantly increased level of NET-inducing activity was observed in the patient’s serum, which improved with treatment. On the other hand, a markedly lower level of NET degradation was observed in the patient’s serum as compared to healthy subjects’ sera, without any posttreatment changes. These findings suggest that NETs may play a role in the pathogenesis of renal injury associated with SLE/AAV overlap syndrome.

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Systemic Lupus Erythematosus (SLE) and Antineutrophil Cytoplasmic Antibody-Associated Vasculitis (AAV) Overlap Syndrome: Case Report and Review of the Literature

Case Reports in Rheumatology ◽

10.1155/2019/5013904 ◽

2019 ◽

Vol 2019 ◽

pp. 1-5 ◽

Cited By ~ 1

Author(s):

Sathish Itikyala ◽

Debendra Pattanaik ◽

Syed Raza

Keyword(s):

Systemic Lupus Erythematosus ◽

Lupus Erythematosus ◽

Microscopic Polyangiitis ◽

Antineutrophil Cytoplasmic Antibody ◽

Overlap Syndrome ◽

Review Of The Literature ◽

Systemic Lupus ◽

Class V ◽

First Case ◽

Granulomatous Polyangiitis

We report here the first case of systemic lupus erythematosus (SLE) and antineutrophil cytoplasmic antibody-associated vasculitis overlap syndrome (SLE/AAV) who had granulomatous polyangiitis (GPA) as the initial presentation. SLE/AAV overlap syndrome is an uncommon entity recently described in the literature. Prior reported patients with SLE/AAV overlap syndrome presented with SLE and microscopic polyangiitis (MPA). Our patient initially presented with granulomatous gastric ulcer and later developed respiratory failure. She was diagnosed with GPA. While on maintenance treatment with azathioprine 150 mg/day, she developed hematuria and proteinuria which turned out to be from class V lupus nephritis instead of relapse of vasculitis. Currently, the patient is doing well after treatment with rituximab. Although rare, this entity should be recognized and need to be treated appropriately.

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A Case of Systemic Lupus Erythematosus/Antineutrophil Cytoplasmic Antibody-Associated Vasculitis Overlap Syndrome with Dissociated Pathological and Immunological Findings

Case Reports in Nephrology ◽

10.1155/2020/5698708 ◽

2020 ◽

Vol 2020 ◽

pp. 1-7

Author(s):

Kazuhiko Kato ◽

Tetsuya Kawamura ◽

Risa Terashima ◽

Yukiko Tsuchiya ◽

Yasuhito Takahashi ◽

...

Keyword(s):

Systemic Lupus Erythematosus ◽

Renal Function ◽

Lupus Erythematosus ◽

Pathological Condition ◽

Antineutrophil Cytoplasmic Antibody ◽

Consensus Conference ◽

Overlap Syndrome ◽

Pathological Findings ◽

Systemic Lupus ◽

American College Of Rheumatology

Systemic lupus erythematosus/antineutrophil cytoplasmic antibody-associated vasculitis overlap syndrome (SLE/AAV OS) describes a pathological condition that presents with overlapping features of two diseases. There have been few reports of SLE/AAV OS and none from Japan. We present the case of a 59-year-old woman admitted with chief complaints of fever and decreased renal function. SLE was suspected due to the identification of four items from the diagnostic criteria of the American College of Rheumatology, including positivity for anti-ds-DNA and antinuclear antibodies. However, pathological findings from the kidney biopsy suggested pauci-immune crescentic glomerulonephritis. She was also diagnosed with AAV according to the Chapel Hill Consensus Conference (CHCC) 2012 definitions and the classification algorithm of AAV. SLE/AAV OS was suspected, we started immunosuppressant therapy, and subsequently her renal function improved. In previous reports, initial immunological and pathological findings generally concur. In cases where clinical and pathological features appear to conflict, as in the present case, a treatment strategy decision should be based on pathological and immunological findings to improve the prognosis of OS.

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AB0013 ASSOCIATION BETWEEN STAT4 POLYMORPHISM AND MANIFESTATIONS OF SLE

Annals of the Rheumatic Diseases ◽

10.1136/annrheumdis-2021-eular.3470 ◽

2021 ◽

Vol 80 (Suppl 1) ◽

pp. 1041.1-1041

Author(s):

V. Agarwal ◽

S. Kakati ◽

P. Debbaruah

Keyword(s):

Systemic Lupus Erythematosus ◽

Lupus Erythematosus ◽

Indian Population ◽

Association Studies ◽

Statistical Significance ◽

Elevated Serum ◽

Systemic Lupus ◽

Cutaneous Manifestations ◽

Genotype Group ◽

Dsdna Antibody

Background:SNP rs7574865, located within the third intron of STAT4 gene at chromosome 2, has been associated with susceptibility to SLE among different ethnic groups.1,2 Interestingly, we recently have documented an association between this gene and susceptibility to systemic lupus erythematosus (SLE) in Indian population.3Objectives:To determine whether the STAT4 (rs7574865) SNP is associated with clinical and immunological manifestations in SLE.Methods:The study was carried out on 100 unrelated SLE (SLICC criteria 2012) patients from North-East India. Genotyping of STAT4 rs7574865 SNP was done using Taqman probe and Real-Time Polymerase chain reaction. An association study was performed between the alleles and genotypes of STAT4 rs7574865 with the clinical and immunological manifestations included in the SLE SLICC classification criteria. For all analysis, the statistical significance was fixed at 5% level of significance (p < 0.05).Results:The mean duration of illness was 2.69±2.55 years. Cases and Controls remained in Hardy-Weinberg equilibrium.The occurrence of Photosensitivity and hyperpigmentation was significantly higher in TT genotype group (97.22% and 77.77%, respectively) with p <0.001 in each case.SLE patients with nephritis (Albuminuria >500mg/24 hours) and elevated serum creatinine were both significantly higher in TT genotype group as compared to GT and GG (p< 0.001 and p=0.001 respectively).The Anti-dsDNA antibody was significantly associated with TT genotype (p <0.001).Conclusion:Our study provides evidence regarding the association between STAT4 rs7574865 gene polymorphism is risk factor for cutaneous manifestations, Lupus nephritis and Anti ds-DNA positivity in SLE. So, our findings reinforce the need for further association studies including prospective studies with larger subjects in order to replicate such findings.References:[1]Graham RR, Ph D, Hom G, Ph D, Behrens TW, Bakker PIW De, et al. and the Risk of Rheumatoid Arthritis and Systemic Lupus Erythematosus. N Engl J Med. 2007;357(10):977–86.[2]Yuan H, Feng JB, Pan HF, Qiu LX, Li LH, Zhang N, et al. A meta-analysis of the association of STAT4 polymorphism with systemic lupus erythematosus. Mod Rheumatol. 2010;20(3):257–62.[3]Gupta V, Kumar S, Pratap A, Singh R, Kumari R, Kumar S, et al. Association of ITGAM, TNFSF4, TNFAIP3 and STAT4 gene polymorphisms with risk of systemic lupus erythematosus in a North Indian population. Lupus. 2018;27(12):1973–9.Disclosure of Interests:None declared

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