Multifocal fibrosclerosis: retroperitoneal fibrosis associated with a suprasellar tumor and pachymeningitis.

Abstract

A 42 year-old man with lumbago and fever had hydronephrosis due to a mass surrounding the abdominal aorta, associated with pachymeningitis and a suprasellar tumor. He was given a diagnosis of multifocal fibrosclerosis, involving both retroperitoneal and intracranial fibrosis. He responded to treatment with corticosteroid and cyclophosphamide. The intracranial lesions seemed to be rare, but important manifestations of the systemic disease, and were clearly visualized by magnetic resonance imaging. The phenotype of the infiltrated lymphocytes was CD4+, supporting the hypothesis that autoimmunity was involved pathogenetically. We propose a comprehensive category termed "inflammatory fibrotic disease" including both systemic and localized diseases.

• Authors: Wataru Fukuda, Mizuho Kimura, Teruaki Akaogi, Masami Sako, Kaiyo Ohiwa, Yasumasa Yamamoto, Genichi Kato, Hideo Hayashi, Toshikazu Yoshikawa
• Journal: Internal medicine (Tokyo, Japan) (Intern Med) Vol. 42 Issue 10 Pg. 1006-10 (Oct 2003) ISSN: 0918-2918 [Print] Japan
• PMID: 14606716 (Publication Type: Case Reports, Journal Article, Review)

Topics

• Adult
• Brain Neoplasms (complications, diagnosis, therapy)
• Humans
• Male
• Meningitis (complications, diagnosis, therapy)
• Retroperitoneal Fibrosis (complications, diagnosis, therapy)