title: [Oral cyclophosphamide therapy for multifocal fibrosclerosis with hypertrophic intracranial pachymeningitis].
abstract: A 54-year-old man was admitted to our hospital because of a headache, dry cough, low grade fever and hearing loss sustained for 6 months. Physical and neurological examinations revealed bilateral conjunctival hyperemia, fine crackles in the lower lungs, cutaneous scars, horizontal gaze evoked nystagmus, bilateral moderate sensorineural deafness and mild hyperreflexia. Hypertrophic intracranial pachymeningitis (HIP) accompanied by episcleritis, pulmonary fibrosis, subcutaneous fibrosis of the trunk and upper limbs, bilateral chronic otitis media and sinusitis of the paranasal cavities were observed. Histopathological investigation of biopsied tissues from the dura matter, lung, skin and nasal mucosa showed marked fibrosis with lymphocyte and plasma cell infiltrations. The diagnosis of multifocal fibrosclerosis (MF) was made; this is a rare syndrome of unknown etiology characterized by fibrosis involving multiple organ systems. Although steroid pulse therapy and cyclophosphamide (CP) pulse therapy was not effective in his illnesses, the combination therapy of corticosteroid and oral CP was dramatically effective. We concluded that HIP can be a manifestation of MF, and additional oral CP should be considered as a treatment for steroid-resistant MF with HIP.
authors.affiliation: Deparment of Neurology, Brain research institute, Niigata University.
authors.forename: Masayoshi
authors.initials: M
authors.lastname: Tada
authors.forename: Osamu
authors.initials: O
authors.lastname: Onodera
authors.forename: Kenju
authors.initials: K
authors.lastname: Hara
authors.forename: Keiko
authors.initials: K
authors.lastname: Tanaka
authors.forename: Hitoshi
authors.initials: H
authors.lastname: Takahashi
authors.forename: Shoji
authors.initials: S
authors.lastname: Tsuji
authors.forename: Masatoyo
authors.initials: M
authors.lastname: Nishizawa
mesh_terms: D000284:Administration, Oral; D000893:Anti-Inflammatory Agents / Q000008:administration & dosage*; D002493:Central Nervous System Diseases / Q000150:complications; D003520:Cyclophosphamide / Q000008:administration & dosage*; D004359:Drug Therapy, Combination; D004388:Dura Mater / Q000473:pathology*; D005355:Fibrosis; D006801:Humans; D007249:Inflammation; D008279:Magnetic Resonance Imaging; D008297:Male; D008417:Mastoiditis / Q000150:complications; D008581:Meningitis / Q000150:complications*; D008875:Middle Aged; D010033:Otitis Media / Q000150:complications; D011239:Prednisolone / Q000008:administration & dosage; D011658:Pulmonary Fibrosis / Q000150:complications; D020551:Pulse Therapy, Drug; D012598:Sclerosis; D012852:Sinusitis / Q000150:complications
chemical_list: D000893:Anti-Inflammatory Agents; D003520:Cyclophosphamide; D011239:Prednisolone