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IgA Vasculitis Presenting as Pulmonary-Renal Syndrome
José Mário Bastos1, Joana Medeiros1, Catarina Oliveira Silva1
1Nephrology, Unidade Local de Saúde de Braga, Braga, PRT.
Cureus
|February 10, 2025
Summary
Immunoglobulin A (IgA) vasculitis (IgAV) can rarely cause pulmonary-renal syndrome in adults. This case highlights the need for high suspicion and multidisciplinary care for severe IgAV presentations.
Area of Science:
- Nephrology
- Rheumatology
- Pulmonology
Background:
- Immunoglobulin A (IgA) vasculitis (IgAV), previously Henoch-Schönlein purpura, is a small-vessel vasculitis.
- Adult-onset IgAV typically has a more severe course and higher risk of kidney complications than pediatric cases.
- Pulmonary-renal syndrome is a rare but severe IgAV manifestation.
Observation:
- A 33-year-old male presented with hemoptysis, fatigue, and myalgias, lacking typical purpura.
- Diagnostic workup revealed anemia, acute kidney injury, proteinuria, hematuria, and bilateral ground-glass opacities on CT.
- Negative serologies for ANCA, anti-GBM, and other autoimmune markers were noted.
Findings:
- Bronchofibroscopy confirmed diffuse alveolar hemorrhage.
- Renal biopsy showed IgA vasculitis nephritis with characteristic IgA deposition.
- The patient was diagnosed with pulmonary-renal syndrome secondary to IgAV.
Implications:
- This case emphasizes the importance of considering IgAV in atypical presentations of pulmonary-renal syndrome.
- A multidisciplinary approach and high index of suspicion are crucial for diagnosis.
- Prompt immunosuppressive therapy, including corticosteroids and plasma exchange, led to favorable outcomes and remission.
Keywords:
alveolar hemorrhageglomerulonephritisiga vasculitisplasmapheresispulmonary-renal syndromesmall-vessel vasculitisMore Related Videos
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