Despite initial immunosuppressive treatment, the patient was lost to follow-up, returned two years later with multi-organ complications including heart failure, and died of respiratory failure from diffuse alveolar hemorrhage.
F1000Research · 16 authors, 17 centres
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Despite initial immunosuppressive treatment, the patient was lost to follow-up, returned two years later with multi-organ complications including heart failure, and died of respiratory failure from diffuse alveolar hemorrhage.
Laboratory testing revealed elevated erythrocyte sedimentation rate at 100 mm/h, C-reactive protein at 66 mg/L, and elevated alkaline phosphatase. CT imaging showed sinusitis, pulmonary nodules, and hepatomegaly. Biopsy of skin, nasal mucosa, and liver demonstrated chronic inflammatory granulomatous inflammation with necrosis, and serum anti-PR3 ANCA was positive, confirming GPA diagnosis. Two years later, he presented with heart failure, skin ulceration, nasal septum perforation, and pulmonary hypertension. Despite re-initiation of immunosuppressive therapy, he developed diffuse alveolar hemorrhage and died of respiratory failure three days later.