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During the congress, E-Posters will be accessible to all participants on the congress website 24/7, as well as in the E-poster stations in the congress center.
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Please review the E-Poster format requirements carefully when preparing your E-Poster. Should your E-Poster not meet the mentioned requirements, it may not be displayed as described above.
E-Poster Submission Deadline
Please prepare and upload your E-Poster no later than March 14, 2026 11.59PM CET. After this date, you will no longer be able to prepare and upload your E-poster and it will not be displayed and accessible on the congress website.
Please follow the instructions below to input your abstract title.
Abstract titles should be brief and reflect the content of the abstract.
The coexistence of ANCA-associated vasculitis (AAV) and IgG4-related disease (IgG4-RD) presents diagnostic and therapeutic challenges. AAV, linked to necrotizing vasculitis and ANCAs, affects small to medium vessels, while IgG4-RD involves elevated IgG4 levels and plasma cell infiltration. A 58-year-old male with fever, positive anti-PR3 ANCA, elevated IgG4, and renal histopathology showing necrotizing glomerulonephritis and storiform fibrosis improved with corticosteroids and cyclophosphamide. This case highlights the need for integrated diagnostic approaches.
A 58M office clerk was admitted with a persistent low-grade fever lasting four months and significant unintentional weight loss (6-10 kg). He reported intermittent right loin pain but denied other symptoms. Despite extensive evaluation, his symptoms remained undiagnosed.
On examination, he was normotensive, with tachycardia and a temperature of 100°F. Initial labs, including CBC, liver and kidney function tests, procalcitonin, blood and urine cultures, serum beta-D-glucan, and ESR, were normal, ruling out common infections. Elevated CRP (300 ng/dl) and ferritin (1100 ng/dl) indicated inflammation. Autoimmune testing showed normal ANA but elevated C-ANCA (110 IU/ML), suggesting ANCA-associated vasculitis, potentially Granulomatosis with Polyangiitis (GPA). Infectious screening, including GeneXpert for tuberculosis and urine AFB, was negative.
During the hospital stay, he developed sudden shortness of breath, bradycardia, and hypotension. ECG revealed complete heart block, leading to a Stokes-Adams attack. A temporary pacemaker was inserted, later replaced by a permanent one. He had rapid clinical deterioration, marked by decreased oxygen saturation, reduced urine output, a drop in hemoglobin, and a rise in serum creatinine from 0.61 to 4.62 mg/dl. Urine analysis showed 3+ proteinuria with RBCs, suggestive of glomerulonephritis. Imaging revealed pleural effusion and a kidney lesion. Elevated serum IgG4 levels (3.28 g/L) indicated possible IgG4-related disease.
Kidney biopsy showed crescentic glomerulonephritis and tubulointerstitial nephritis with storiform fibrosis and significant IgG4 plasma cell infiltration. Diagnosed with overlap syndrome of ANCA vasculitis and IgG4-related disease, he received immunosuppressive therapy, antibiotics, and antifungal prophylaxis. He improved with increased urine output and decreased creatinine, achieving full recovery. This case highlights the importance of a multifaceted approach in managing complex autoimmune conditions.
AAV involves necrotizing vasculitis with ANCAs, affecting small to medium vessels, and manifests with glomerulonephritis and systemic symptoms. IgG4-RD is marked by fibro-inflammatory lesions with IgG4-positive plasma cells and storiform fibrosis. Renal involvement in IgG4-RD often presents as tubulointerstitial nephritis. Our patient showed features of both conditions, confirmed by biopsy. Treatment with corticosteroids and cyclophosphamide improved renal function and systemic symptoms, highlighting the need for integrated diagnosis and therapy in overlap syndromes.
The overlap of ANCA-associated vasculitis and IgG4-related disease is rare but crucial to recognize for optimal treatment. Research into shared mechanisms will improve understanding and management of autoimmune diseases.
