Abstract: PUB177
Overlap Syndrome of Rheumatoid Arthritis, Systemic Lupus Erythematosus, and ANCA-Associated Vasculitis: A Case Report
Session Information
Category: Glomerular Diseases
- 1402 Glomerular Diseases: Clinical, Outcomes, and Therapeutics
Authors
- Victorio, Milagros, Hospital Nivel IV Guillermo Almenara Irigoyen, Lima, Lima Region, Peru
- Plaza, Stephanie, Hospital Nivel IV Guillermo Almenara Irigoyen, Lima, Lima Region, Peru
- Vasquez, Edwin, Hospital Nivel IV Guillermo Almenara Irigoyen, Lima, Lima Region, Peru
- Castillo Velarde, Edwin Rolando, Hospital Nivel IV Guillermo Almenara Irigoyen, Lima, Lima Region, Peru
Introduction
“Overlap” refers to the coexistence of features from more than one autoimmune disease.Renal involvement may lead to rapidly progressive glomerulonephritis(RPGN).While RA–SLE overlap(“Rhupus”) is uncommon,triple overlap with ANCA-associated vasculitis(AAV) is exceptionally rare.
Case Description
A 62-year-old woman with longstanding rheumatoid arthritis treated with leflunomide,methotrexate,and infliximab presented with progressive edema and gross hematuria.Examination revealed pretibial purpura,ulnar deviation with rheumatoid nodules,and edema.Urinalysis showed nephrotic-range proteinuria,dysmorphic hematuria,and casts.Laboratory tests demonstrated acute kidney injury(creatinine 1.45→3.15 mg/dL),hypoalbuminemia,ANA 1:320,and MPO-ANCA >200;anti-dsDNA and ENA were negative.Renal biopsy demonstrated class IV lupus nephritis with wire-loop lesions,cellular crescents,and fibrinoid necrosis,with a “full-house” immunofluorescence pattern.Skin biopsy revealed leukocytoclastic vasculitis,supporting RA–SLE–ANCA overlap syndrome.Treatment with methylprednisolone and cyclophosphamide initially improved renal function;however,the patient was readmitted with sepsis,pancytopenia,respiratory failure,and shock resulting in death.
Discussion
This case highlights the rare coexistence of rheumatoid arthritis,systemic lupus erythematosus,and ANCA-associated vasculitis with severe renal involvement.Patients with SLE and ANCA positivity may develop aggressive necrotizing crescentic glomerulonephritis,creating diagnostic challenges between lupus nephritis and pauci-immune vasculitis.Our patient presented with rapidly progressive glomerulonephritis,ANA/MPO-ANCA positivity,and biopsy findings of wire-loop lesions,fibrinoid necrosis,and cellular crescents,supporting overlap syndrome and polyautoimmunity.Despite initial improvement with immunosuppressive therapy,the patient developed fatal infections.Triple overlap involving RA,SLE,and AAV is exceptionally rare and underscores the importance of early recognition and multidisciplinary management overall.
Image A:Endocapillary hypercellularity with wire-loop lesions.
Image B:Cellular crescent with fibrinoid necrosis and glomerular tuft collapse.