title: [Macrophagic myofasciitis. Study and Research Group on Acquired and Dysimmunity-related muscular diseases (GERMMAD)].
abstract: MACROPHAGIC MYOFASCIITIS: A most unusual inflammatory myopathy, first described by Germmad had been reported with increasing frequency since 1993 in the leading French myopathology centers. We present our experience with this new disease: macrophagic myofasciitis.
  
  
  METHODS
  By November 1999, 70 cases of macrophagic myofasciitis had been recorded since our first description. The first 22 patients (sex ratio M/F = 1:3) referred with the presumptive diagnosis of polymyositis (n = 11), polymyalgia rheumatica (n = 5), mitochondrial cytopathy (n = 4), and congenital myopathy or muscle dystrophy (n = 1 each). Symptoms included myalgia (91%), anthralgia (68%), marked asthenia (55%), muscle weakness (45%), and fever (32%).
  
  
  RESULTS
  Abnormal laboratory findings included elevated CK levels (50%), markedly increased erythrocyte sedimentation rate (37%), and myopathic EMG (35%). Muscle biopsy showed a unique myopathological pattern characterized by: i) centripetal infiltration of epimysium, perimysium and perifascicular endomysium by sheets of large cells of the monocyte/macrophage lineage (CD68+, CD1a-, S100-, with a PAS-positive content; ii) absence of necrosis, of both epithelioid and giant cells, and of mitotic figures; iii) presence of occasional CD8+ T-cells; iv) inconspicuous muscle fiber damage. The picture was easily distinguishable from sarcoid myopathy and fasciitis-panniculitis syndromes. The infectious diseases know to be associated with reactive histiocytes, including Whippleís disease, Mycobacterium avium intracellulare infection and malakoplakia, could not be documented. Patients improved under corticosteroid therapy and/or immunomodulatory therapeutic
  
  
  CONCLUSIONS
  A new inflammatory muscle disorder, characterized by a distinctive pathological pattern of macrophagic myofasciitis is emerging in France.
authors.affiliation: Service de Médecine Interne, Groupe Hospitalier Pitié-Salpêtrière, Paris. patrick.cherin@psl.ap-hop-paris.fr
authors.forename: P
authors.initials: P
authors.lastname: Chérin
authors.forename: P
authors.initials: P
authors.lastname: Laforêt
authors.forename: R K
authors.initials: RK
authors.lastname: Ghérardi
authors.forename: F J
authors.initials: FJ
authors.lastname: Authier
authors.forename: T
authors.initials: T
authors.lastname: Maisonobe
authors.forename: M
authors.initials: M
authors.lastname: Coquet
authors.forename: J M
authors.initials: JM
authors.lastname: Mussini
authors.forename: J F
authors.initials: JF
authors.lastname: Pellissier
authors.forename: B
authors.initials: B
authors.lastname: Eymard
authors.forename: S
authors.initials: S
authors.lastname: Herson
mesh_terms: D000328:Adult; D000368:Aged; D001706:Biopsy; D004796:Clinical Enzyme Tests; D003402:Creatine Kinase / Q000097:blood; D003937:Diagnosis, Differential; D005208:Fasciitis / Q000175:diagnosis / Q000209:etiology / Q000473:pathology; D005260:Female; D006801:Humans; D008264:Macrophages; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D018908:Muscle Weakness / Q000209:etiology; D018482:Muscle, Skeletal / Q000473:pathology; D009135:Muscular Diseases / Q000175:diagnosis* / Q000209:etiology / Q000473:pathology; D010146:Pain / Q000209:etiology
chemical_list: D003402:Creatine Kinase