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

Intern Med. 2015;54(17):2267-72. doi: 10.2169/internalmedicine.54.3230. Epub 2015 Sep 1.


Multifocal fibrosclerosis is the term used to represent a combination of similar fibrous lesions occurring at different anatomical sites. We herein report a hypertrophic pachymeningitis patient with a soft tissue mass around the thoracic vertebral bodies. A histopathological analysis of the biopsied tissues from both lesions showed dense fibrosis and a marked infiltration of lymphocytes and plasma cells, which lead to the diagnosis of multifocal fibrosclerosis. This pathological condition closely resembles that of IgG4-related disease and is a very rare combination of manifestations. Our case suggests that hypertrophic pachymeningitis patients need to also undergo a whole body examination.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Aged
  • Anti-Inflammatory Agents / therapeutic use
  • Humans
  • Hypertrophy / diagnosis
  • Immunosuppressive Agents / therapeutic use
  • Male
  • Meningitis / complications*
  • Meningitis / diagnosis*
  • Meningitis / drug therapy
  • Meningitis / pathology
  • Methylprednisolone / therapeutic use
  • Plasma Cells / pathology
  • Retroperitoneal Fibrosis / complications
  • Retroperitoneal Fibrosis / congenital*
  • Retroperitoneal Fibrosis / diagnosis
  • Retroperitoneal Fibrosis / drug therapy
  • Retroperitoneal Fibrosis / pathology
  • Tacrolimus / therapeutic use
  • Thoracic Vertebrae / pathology
  • Treatment Outcome


  • Anti-Inflammatory Agents
  • Immunosuppressive Agents
  • Tacrolimus
  • Methylprednisolone

Supplementary concepts

  • Multifocal fibrosclerosis