Pulmonary Amyloidosis with Pulmonary Arteriovenous Fistula
Amyloid deposition has been reported to make vessels fragile, and some cases of hemorrhage caused by amyloidosis have been reported. We report herein a case of pulmonary amyloidosis suspected of causing a pulmonary AV fistula. read only
A 77-year-old man was admitted for close examination of abnormal shadows on his chest roentgenogram, which was taken during a routine check-up. His medical history revealed a gastric ulcer and cerebral hemorrhage. His family history was unremarkable. At the age of 62, his chest roentgenogram showed two tumorous shadows in the left upper lobe, and an open biopsy disclosed “benign lung tumors” of unknown origin. Since then he had received no treatment or follow-up examination. On admission, he was asymptomatic and his physical examination disclosed bruit on the right chest wall audible only on inspiration. No telangiectasia was seen on his skin or in his oral cavity. Laboratory data revealed increased ESR, CRP and CEA. Blood gas analysis showed mild hypoxia (PaOs of 62 mm Hg, PaC02 of 33 mm Hg) while the results of a lung (unction test were unremarkable. Sputum examination did not suggest malignancy or tuberculosis. Chest roentgenogram revealed bilateral multiple nodular calcified shadows (Fig 1).
Tomography revealed two vessel-like shadows connected to one nodular lesion in the right lower lobe, but no such shadows were found in the other nodular lesions. Pulmonary angiography showed an AV fistula in the right lower lobe, while the other nodules were unrelated to the pulmonary arteries (Fig 2). These nodules were thought to be benign because of their slow rate of growth. A right lower lobectomy was performed for resection of the AV fistula and biopsy of the other tumors. Pathologic examination showed the nodular lesions in the right lower lobe to be a solitary AV fistula with amyloid deposition. AO the other nodules were amyloidomas. Massive amyloid deposits were seen in the vascular walls of the fistula (Fig 3), and the amyloid was found to be A X type. Further examinations (including rectal biopsy, gastroscopy, fiberoptic bronchoscopy, bone marrow aspiration) were performed, but no amyloid deposits were detected in other organs. No specific cause of amyloidosis was found. Re-examination of the old specimens disclosed that the first two tumors were also amyloidomas. The final diagnosis was primary amyloidosis of the lungs with a solitary AV fistula. After the operation, hypoxia disappeared and the patient has been well with no special treatment.
Figure 1. Chest roentgenogram taken on admission; multiple nodular shadows, some of which were calcified, were evident.
Figure 2. Pulmonary angiography showing an AV fistula in the right lower lobe. Note that no other nodules were related to the pulmonary arteries.
Figure 3. From the lobectomy specimen showing the AV fistula with mural amyloid deposition, Congo-Red stain, original magnifications X 40 (upper) and x 200 (lower).