Pediatric ANCA-associated vasculitis presented with various clinical findings mimicking IgA Vasculitis and IgG4-related disease: Two cases

Pediatric ANCA-associated vasculitis

Authors

Keywords:

ANCA, vasculitis, IgG4, IgA

Abstract

Granulomatous polyangiitis (GPA) is the most common anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV), characterized by necrotizing inflammation of small and medium-sized vessels. It can affect various organs, particularly the lung, kidneys, upper respiratory tract, ears, and skin. Diagnosis of AAV poses significant challenges due to its diverse clinical features. This report presents two interesting cases of GPA: one with rare ocular involvement, who subsequently developed end-stage kidney disease (ESKD), and the other with palpable purpura mimicking immunoglobulin A vasculitis, who relapsed with mastoiditis while in renal remission. Early and effective treatment can improve patient prognosis, highlighting the importance of increasing disease awareness during initial diagnosis and in pediatric AAV patients experiencing relapses.

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Author Biographies

Bahriye Uzun Kenan, Gazi University, Department of Pediatric Nephrology, Ankara, Turkey

Department of Pediatric Nephrology

Emine Nur Sunar Yayla, Gazi University, Department of Pediatric Rheumatology, Ankara, Turkey

Department of Pediatric Rheumatology

Betül Öğüt, Gazi University, Department of Pathology, Ankara, Turkey

Department of Pathology

Bahar Büyükkaragöz, Gazi University, Department of Pediatric Nephrology, Ankara, Turkey

Department of Pediatric Nephrology

Deniz Gezgin Yıldırım, Gazi University, Department of Pediatric Rheumatology, Ankara, Turkey

Department of Rheumatology

Sevcan A. Bakkaloğlu , Gazi University, Department of Pediatric Rheumatology, Ankara, Turkey

Department of Pediatric Nephrology 

Department of Pediatric Rheumatology

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Published

2023-08-05

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Section

Case Report

How to Cite

1.
Oktay MA, Akyuz Oktay S, Uzun Kenan B, Sunar Yayla EN, Öğüt B, Büyükkaragöz B, Gezgin Yıldırım D, Bakkaloğlu SA. Pediatric ANCA-associated vasculitis presented with various clinical findings mimicking IgA Vasculitis and IgG4-related disease: Two cases: Pediatric ANCA-associated vasculitis. J Surg Med [Internet]. 2023 Aug. 5 [cited 2024 Jul. 15];7(8):521-3. Available from: https://jsurgmed.com/article/view/7276