CASE REPORT

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

V Cottin¹, P-Y Brillet², F Combarnous³, F Duperron², H Nunes⁴, J-F Cordier¹

¹ Hospices Civils de Lyon, Lyon I University, Louis Pradel Hospital, Department of Respiratory Diseases – Reference Center for Orphan Lung Disorders, Lyon, France
² Assistance Publique – Hôpitaux de Paris, Paris 13 University, Avicenne Hospital, Department of Imaging, Bobigny, France
³ Clinique du Tonkin, Department of Nephrology, Villeurbanne, France
⁴ Assistance Publique – Hôpitaux de Paris, Paris 13 University, Avicenne Hospital, Department of Respiratory Diseases, Bobigny, France

Correspondence to:
Pr V Cottin, Hôpital Louis Pradel, 28 avenue Lépine, 69677 Lyon (Bron) Cedex, France; vincent.cottin{at}chu-lyon.fr

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. These cases represent an unusual new presentation of pleural and retrosternal fibrosis extending beyond the anatomical structures and associated with retroperitoneal fibrosis.

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