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

Mehmet Ali Oktay; Selin Akyuz Oktay; Bahriye Uzun Kenan; Emine Nur Sunar Yayla; Betül Öğüt; Bahar Büyükkaragöz; Deniz Gezgin Yıldırım; Sevcan A. Bakkaloğlu

doi:10.28982/josam.7276

Authors

Mehmet Ali Oktay Gazi University, Department of Pediatrics, Ankara, Turkey https://orcid.org/0000-0002-7116-5828
Selin Akyuz Oktay Gazi University, Department of Pediatrics, Ankara, Turkey https://orcid.org/0000-0003-4324-6361
Bahriye Uzun Kenan Gazi University, Department of Pediatric Nephrology, Ankara, Turkey https://orcid.org/0000-0002-8248-0436
Emine Nur Sunar Yayla Gazi University, Department of Pediatric Rheumatology, Ankara, Turkey https://orcid.org/0000-0003-1646-2341
Betül Öğüt Gazi University, Department of Pathology, Ankara, Turkey https://orcid.org/0000-0003-1646-2341
Bahar Büyükkaragöz Gazi University, Department of Pediatric Nephrology, Ankara, Turkey https://orcid.org/0000-0002-6342-1975
Deniz Gezgin Yıldırım Gazi University, Department of Pediatric Rheumatology, Ankara, Turkey https://orcid.org/0000-0002-4823-2076
Sevcan A. Bakkaloğlu Gazi University, Department of Pediatric Rheumatology, Ankara, Turkey https://orcid.org/0000-0001-6530-9672

DOI:

https://doi.org/10.28982/josam.7276

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