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.

Prednisolone markedly reduced serum IgG4 levels along with the improvement of pituitary mass and anterior pituitary function in a patient with IgG4-related infundibulo-hypophysitis

“…It is also associated with idiopathic retroperitoneal fibrosis [34,35]. Some cases with infundibulo-neurohypophysitis were suspected as IgG4-related because they were associated with IgG4-related systemic diseases [36][37][38][39][40]. The first IgG4-related hypophysitis with high serum IgG4 level was reported in 2004 [4].…”

IgG4-Related Sclerosing Disease, an Emerging Entity: A Review of a Multi-System Disease

“…Idiopathic retroperitoneal fibrosis or mediastinal fibrosis may be associated with Riedel thyroiditis, orbital/lacrimal IgG4-RSD or IgG4-related sclerosing cholangitis 112. The fibrosis in these locations may extend to involve the kidney, ureter and bowel mesentery with varying response to steroid therapy which is ineffective in advanced fibrosis 113-118…”

Multifocal Fibrosclerosis with Hypertrophic Pachymeningitis and a Soft Tissue Mass around the Thoracic Vertebral Bodies: A Case Report with Review of the Literature

“…Multifocal fibrosclerosis is the term used to represent a combination of similar fibrous lesions occurring at different anatomical sites (4), and some conditions in this disease overlap with those of IgG4-related disease. There have been several reports on multifocal fibrosclerosis with hypertrophic pachymeningitis (5)(6)(7)(8). We herein report a hypertrophic pachymeningitis patient with a soft tissue mass around the thoracic vertebral bodies, paying special attention to the similarities and differences between this disease and IgG4-related disease.…”