Abstract Idiopathic pulmonary hemosiderosis (IPH) is a rare cause of diffuse alveolar hemorrhage characterized by recurrent hemoptysis, pulmonary infiltrates, and iron deficiency anemia. Its pathogenesis remains unclear, though autoimmune, allergic, genetic, and environmental factors have been proposed. Diagnosis requires exclusion of other causes of alveolar hemorrhage, with histology showing hemosiderin-laden macrophages without vasculitis or immune complex deposition. We present a case of a 64-year-old male who has had multiple episodes of recurrent hemoptysis since 2020, with diffuse ground glass abnormalities on Computed tomography (CT) scans and acute hypoxic respiratory failure requiring supplemental oxygen. He responded well to steroids with resolution of his symptoms and CT findings. He has had multiple bronchoscopies, which showed diffuse alveolar hemorrhage at the time of his flare. His transbronchial biopsies were nonrevealing and had hemosiderin laden macrophages. His autoimmune work was negative, including Antinuclear Antibody (ANA), Anti-double-stranded DNA (anti-dsDNA), Antineutrophil Cytoplasmic Antibodies (ANCA) by Immunofluorescence Assay (IFA), Proteinase 3 (PR-3), myeloperoxidase (MPO), anti-Glomerular Basement Membrane (GBM), Antiphospholipid Antibody (APLA) panel and Complement C3, C4. He subsequently had a right Video-Assisted Thoracoscopic Surgery wedge resection of right middle and lower lobes. The biopsy showed numerous hemosiderin laden macrophages that were intra-alveolar and interstitial, but without significant intra-alveolar hemorrhage. It only showed a very focal alveolar septal acute inflammation. Given the clinical picture, negative autoimmune workup and predominant biopsy findings the consensus diagnosis favored IPH and he started steroid sparing immunosuppression with azathioprine. He tolerated it well and had significant reduction in his recurrent episodes. Fig. 1 shows his CT scans on one of his presentations- initial CT and rapid clearance of the ground glass opacities within a few days of initiating steroids. He has been relatively stable and continues to follow closely with the pulmonary team. IPH is a rare disease in adults and is a diagnosis of exclusion. A high level of suspicion is needed when initial workup remains negative. A surgical biopsy is often required for diagnosis. Early initiation of immunosuppressive therapy is the cornerstone of management IPH, while recognizing that well-defined treatment protocols and prognostic factors remain lacking. Long term monitoring and careful follow up is essential due to frequent relapses. This abstract is funded by: None
Saeed et al. (Fri,) studied this question.