Pulmonary-Cardiac Involvement in a Patient with IgA Vasculitis
- 1King Faisal Specialist Hospital & Research Center, Riyadh, Saudi Arabia
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.
Related
- Diagnostic value of hematological inflammatory indices for renal involvement in adult IgA vasculitis : A cross-sectional study
- Vasculitis Syndromes with Renal Involvement: A Review from a Case Series
- Comparison of Clinical, Hemodynamic and Imaging Profiles Between Isolated Pulmonary Vasculitis and Chronic Thromboembolic Pulmonary Hypertension
- Severe gastrointestinal involvement is associated with renal manifestations in pediatric IgA vasculitis: a retrospective cohort study
- Pulmonary Leukocytoclastic Vasculitis Associated with Myelodysplastic Syndrome
BibTeX
@article{almutlaq2026pulmonarycardiac,
title = {{Pulmonary-Cardiac Involvement in a Patient with IgA Vasculitis}},
author = {Malak Almutlaq and Hamad Alhumaidan and Mohannad K. Maghrabi and Ali Alenazy},
journal = {Methodist DeBakey Cardiovascular Journal},
year = {2026},
month = nov,
doi = {10.14797/mdcvj.1953},
url = {https://doi.org/10.14797/mdcvj.1953},
note = {CC BY-NC 4.0},
}
RIS
TY - JOUR
TI - Pulmonary-Cardiac Involvement in a Patient with IgA Vasculitis
AU - Malak Almutlaq
AU - Hamad Alhumaidan
AU - Mohannad K. Maghrabi
AU - Ali Alenazy
PY - 2026
DA - 2026/11/03
JO - Methodist DeBakey Cardiovascular Journal
DO - 10.14797/mdcvj.1953
UR - https://doi.org/10.14797/mdcvj.1953
AB - 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.
SN - 1947-6094
SN - 1947-6108
ER -