Year: 2026
Type: article
Study design: Case Report
Abstract: Immunoglobulin A (IgA) vasculitis (IgAV), formerly known as Henoch-Schönlein purpura, is a systemic small-vessel vasculitis caused by IgA immune-complex deposition; cardiac involvement is exceedingly rare and incompletely characterized. We describe a 41-year-old woman with biopsy-proven IgA nephropathy who presented with progressive dyspnea, hemoptysis, and a painful purpuric rash and was found to have severe multisystem IgAV with simultaneous cardiac, pulmonary, renal, and cutaneous involvement. High-sensitivity troponin T peaked at 20,208 ng/L and N-terminal pro-B-type natriuretic peptide at 49,428 pg/mL. Cardiac magnetic resonance imaging showed a left ventricular ejection fraction of 25%, diffuse mid-wall and epicardial late gadolinium enhancement with abnormal T1 and T2 values, and a 25 × 15 mm apical thrombus. Skin biopsy confirmed leukocytoclastic vasculitis and bronchoscopy confirmed diffuse alveolar hemorrhage, while multimodality imaging and a negative serologic work-up excluded cardiac sarcoidosis, antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis, and anti-glomerular basement membrane disease. The patient was treated with pulse methylprednisolone, plasma exchange, rituximab, and cautious anticoagulation, with resolution of alveolar hemorrhage, a progressive decline in cardiac biomarkers, resolution of the left ventricular thrombus, and partial recovery of left ventricular systolic function. This case expands the recognized spectrum of cardiovascular involvement in adult IgAV and highlights the value of multimodality imaging and multidisciplinary management in establishing the diagnosis and guiding therapy.
DOI: 10.14797/mdcvj.1953
Institution: King Faisal Specialist Hospital & Research Centre
Language: English
Subfield: Rheumatology
Field: Medicine
Domain: Health Sciences
Open Access status: diamond
OpenAlex ID: W7225180455