Syndrome of pleural and retrosternal "bridging" fibrosis and retroperitoneal fibrosis in patients with asbestos exposure

Abstract

Two case histories are described of pleural and anterior mediastinal fibrosis presenting as a continuous fibrotic process with thick parietal pleural plaques extending from one pleura to the contralateral pleura through the retrosternal area, and with retroperitoneal fibrosis. Follow-up over 4 years in one case demonstrated rapid progression of disease, with pleural fibrosis preceding retrosternal and retroperitoneal fibrosis. Histopathological analysis in both cases showed non-tumoral fibrosis with broad fibrous bundles surrounding fibroblasts (and lymphocytes in one case). Possible causes such as infections and exposure to ergot derivatives were excluded. Both patients had been slightly or moderately exposed to asbestos.

Figure 1. Retrosternal and pleural fibrosis in patient 1.

(A) Anterior posterior chest radiograph showing reduced lung volumes, pleural opacity of the right lung apex and axillary pleural plaques. (B) Lateral view of the chest radiograph showing enlargement of the retrosternal area. (C) CT scan of the chest showing a dense fibrous lesion extending from one pleura to the contralateral pleura through the retrosternal area (“bridging fibrosis”).

Figure 2. Retrosternal, pleural and retroperitoneal fibrosis in patient 2.

(A) CT scan of the chest in 2001 showing a moderate left pleural effusion and bilateral pleural thickening predominating on the anterior parietal pleura. (B) CT scan of the chest in 2005 showing dramatic worsening of the bilateral pleural thickening with left circumferential pleural thickening and a thick calcified retrosternal mass in continuity with the pleura on both sides (“bridging fibrosis”). (C) CT scan of the abdomen in 2005 showing periaortic retroperitoneal fibrosis.