AB050. Man 69 years old with persistent fever and hemoptysis

Abstract: The description of a case of granulomatosis with polyangiitis initially manifesting as lung cancer. A male, 69 years old, was admitted because of high fever (39 °C), cough, hoarseness, thoracic pain for the last fifteen days and hypoxia (PH 7.4, PO₂ 40, PCO₂ 41 mmHg) and was treated with broad-spectrum antibiotics. The chest CT showed extensive opacification with bronchogram in the right upper lobe, diffuse pneumonitis in the middle lobe and scattered pulmonary nodules bilaterally. Laboratory Results: CRP 25.7 mg/dL, ESR 111 mm and leukocytes 9,750/mcL (86% pmn). Bronchoscopy (twice) revealed a necrotic mass completely obstructing the right upper lobe. Histologically, areas of necrosis surrounded by atypical granulomas were observed. The patient started having hemoptysis. Pending further results the patient was put on anti-tuberculosis treatment. Meanwhile, in a new CT, most of the lesions had cavitated, he developed hematuria (90–100) and proteinuria (1.8 gr/24h) and C-ANCA were found + (>100 EU/mL). So a diagnosis of granulomatosis with polyangiitis (GPA) was reached. He was started on methylprednisolone and cyclophosphamide for 5 monthly cycles. While the respiratory failure improved (pO₂: 80 mmHg), he developed acute renal failure (creatinine 3.64 mg/dL). A kidney biopsy was performed and rapidly progressive glomerulonephritis was diagnosed. Gradually, renal function improved (creatinine 1.12 mg/dL), but he still has mild proteinuria (<1 gr/d). Recently cyclophosphamide was discontinued and he was switched to azathioprine. The usual radiographic image of the GPA are multiple nodules (40–70%). In our case the primary finding was an endobronchial lesion with a first diagnosis of malignancy. The delay in diagnosis (30 days) from the admission of the patient was due to the atypical bronchial localization (15–25%) of the GPA and the atypical histological picture in the lung. But the escalation of organ involvement, especially kidney failure pointed to the correct final diagnosis.

Keywords: Granulomatosis; polyangiitis; glomerulonephritis