A 42-year-old man presented with intermittent hemoptysis. Chest computed tomography (CT) showed pulmonary embolism and thickening of the pulmonary artery walls (Fig. 1a). DVT was not noted on lower extremity CT but was confirmed by lower extremity ultrasound. The laboratory findings were as follows: white blood cells (WBC) 8,500 /muL with no eosinophilia, hemoglobin (Hb) 10.3 g/dL, C-reactive protein (CRP) 8.7 mg/dL, negative anti-nuclear antibodies, negative anti-cardiolipin IgG antibody, myeloperoxidase anti-neutrophil cytoplasmic antibody (MPO-ANCA) <0.5 IU/mL, proteinase 3 (PR3)-ANCA <0.5 IU/mL, and D-dimer 2.4 mug/mL. Pulmonary thromboembolism was diagnosed, and anti-coagulant therapy was started. During follow-up, the patient continued to have bloody sputum, CRP remained elevated at 3 to 6 mg/dL, and gradual expansion of the pulmonary arteries was noted on chest X-ray (Fig. 2).
Five months after starting anti-coagulant therapy, he was readmitted for massive hemoptysis. On arrival, his oxygen saturation was 76% breathing ambient air and 94% on 10 L/min of oxygen. The findings on his physical examination were normal, including lung sounds and lower extremity evaluation. Chest X-ray showed consolidation in the right lower lung field (Fig. 3, left). The laboratory findings were as follows: WBC 13,000 /muL, Hb 8.4 g/dL, CRP 6.3 mg/dL, erythrocyte sedimentation rate (ESR) 107 mm/hr, and D-dimer 7.9 mug/mL. Chest CT showed prominent aneurysms and thickening of the pulmonary artery walls (Fig. 1b) with consolidation predominantly located in the right middle lobe, findings which were all suggestive of hemorrhage. The results of echocardiography were normal with an estimated right ventricular systolic pressure of 27 mmHg, and right heart catheterization was not performed due to the risk associated with the procedure. Anti-coagulant therapy was immediately discontinued.
Further examinations revealed that the patient had had recurrent oral ulcers since his early 30s and a history of uveitis two years prior and erythema nodosum one year prior. The uveitis and erythema nodosum had been successfully treated with topical steroids. The patient was diagnosed with Behcet's disease. Human leukocyte antigen testing was positive for B51, and findings from an interferon gamma release assay for tuberculosis and serologic tests for syphilis were negative. Intravenous methylprednisolone (1 g/day) for 3 days was followed by oral prednisone 50 mg/day (1 mg/kg/day), which was subsequently tapered. Intravenous cyclophosphamide at a dose of 750 mg (15 mg/kg) was also given every 3 weeks for a total of 6 cycles, followed by oral azathioprine. Within two weeks, hemoptysis disappeared, his CRP values normalized, and his ESR decreased to 15 mm/hr. The pulmonary artery aneurysm and thrombosis gradually resolved, and nearly returned to normal state at the end of cyclophosphamide pulse therapy, which was five months after starting treatment (Figure 1c and d, 3).