A 21-year-old Korean woman had suffered from dyspnea and cough for the past 3 months. She was healthy until 2 years ago, when she first noticed both knee joints to swell and subside spontaneously. She also experienced intermittent both lower leg pain. Two months ago, chest P-A showed heterogenous, hazy, streaky densities on both lower lung fields and a well-marginated homogenous squared shape of mass-like density on the right middle lung (Fig. 1). She was treated with isoniazid, rifampin and pyrazinamide under the diagnosis of pulmonary tuberculosis, which was not confirmed by microbiologic study, at a private clinic. After 2 months of anti-tuberculous drugs, the chest X-ray revealed much improved infiltrations on both lower lung fields and right middle lung, but with newly developed lobulated, round homogenous density, with 4x4 cm in diameter, at the left hilar region (Fig. 2). She was transferred to our hospital for the evaluation of the left hilar lung mass.
The patient was single, non-smoker and non-drinker. There was no history of pulmonary tuberculosis. She denied fever, sweats, hemoptysis, headache, otalgia and otorrhea, and bladder or bowel dysfunction.
The temperature was 36.8  C, the pulse was 68 and the respiration was 18. The blood pressure was 130/80 mmHg.
On physical examination, she appeared pale and chronically ill. She had multiple ulcerations on lower lip and oral cavity near right premolar teeth, but not on genital area. The ophthalmologic study showed no abnormal findings. The lung, heart and abdomen were normal. The skin examination revealed erythematous, tender nodules on wrist and both pretibial areas, which were pathologically proven as erythema nodosum. A gynecologist notified the aphthous ulceration of labia minora. The remaining physical examination was normal.
The urine was normal. The hematocrit was 27 percent; the white-cell count was 9,000/mm3, with 68 percent neutrophils, 29 percent lymphocytes, 2 percent monocytes and 1 percent eosinophils. The ESR was 28 mm/hour. The shape of RBC was microcytic and hypochromic. The urea nitrogen was 18 mg%, the glucose 80 mg%, the serum alanine aminotransferase 20 IU, the alkaline phosphatase 56 U. The VDRL was negative. The arterial blood gas analysis showed PH; 7.40, PaCO2; 41 mmHg, PaO2; 96 mmHg. An electrocardiogram was normal.
The perfusion scan showed lobar perfusion defect of left lower lobe and focal segmental perfusion defect of right lower lobe. The ventilation scan showed round single defect in left hilar area. The computerized tomogram of thorax revealed 5x5cm-sized, round, mass with surrounding low density, suggesting PAA with thrombosis in left lung (Fig. 3). Ascending venogram of lower leg showed diffuse obstruction of right femoral vein. The digital subtraction pulmonary angiogram revealed huge dilatation of left main pulmonary artery, proximal to the obstructed pulmonary artery (Fig. 4).
Ten days after admission, blood-tinged sputum occurred abruptly at early morning. The patient was treated with steroids for 14 days without improvement of hemoptysis. During the next 6 weeks, although she was given steroids persistently, the left hilar lesions grew to large mass with diameter of 5 cm. We decided to excise the aneurysmal mass. Macroscopic examination of the excised lobe showed saccular dilation of pulmonary artery surrounded by the underventilated pulmonary parenchyma. On section, the aneurysmal dilatation was 5 cm in diameter and lined with laminated old blood clots. The main histopathological findings were located in the arterial walls. The arterial wall was markedly destructed with minimal fibrous thickening, medial destruction, marked infiltration of inflammatory cells, predominantly neutrophils. A large area of aneurysmal wall was necrotic, ulcerated and attached by thrombus. On special stain, elastic lamina was marked destructed, fragmented and frequently lost. There were multifocal infarctions, both in parenchymal and subpleural portion.
She was treated with maintenance prednisolone during 1 year after lobectomy, but she noted sudden massive hemoptysis and eventually died.
The computerized tomogram of thorax reveals 5x5 cm-sized, round, mass with surrounding low density suggesting pulmonary artery aneurysm with thrombosis in left lung.