title: Clinical and pathological features of Churg Strauss syndrome among a Japanese population: a case series of 18 patients.
abstract: BACKGROUND
  Many autoimmune diseases differ in individual of different races, but there has been very scarce information on the clinicopathological features of Churg Strauss syndrome (CSS) among Asians patients.
  
  
  OBJECTIVE
  To clarify the clinicopathological details of Japanese CSS patients.
  
  
  METHODS
  The medical records of CSS patients hospitalized in 1980-2007 were carefully reviewed.
  
  
  RESULTS
  Seventeen patients fulfilled the Japanese Ministry of Health, Labour and Welfare (MHLW) criteria and all 18 fulfilled the American College of Rheumatology criteria. Sixteen patients (89%) had the history of asthma. Frequently involved organs were peripheral nerves (PNS) (94%), skin (50%), gastrointestinal tract (33%), kidney (22%), and heart (17%). The mean (range) eosinophil count, C-reactive protein, and number of damaged organs was 18,108 (3,820-36,760)/microL, 51 (0-126) mg/L, and 2.7 (1-6), respectively. Four patients died, of whom three had heart involvement while only one without it died (100 versus 9%, respectively, p = 0.0088). Regarding the pathology, vasculitis was observed in six of seven skin but in only 2 of 10 PNS biopsies. Eosinophilia was found in all of the tissues except for PNS and muscle (40%). Granuloma was observed in only three of the total of 29 biopsies.
  
  
  CONCLUSIONS
  The usefulness of MHLW criteria was verified. The clinical features of Japanese CSS patients were mostly similar to those previously reported, except for lower asthma- and ANCA-positivity rates. Regarding the pathology, vasculitis and eosinophilia were much more frequently observed in skin.
authors.affiliation: Department of Allergy and Immunological Diseases, Tokyo Metropolitan Komagome Hospital, Tokyo, Japan. ytaki-tky@umin.ac.jp
authors.forename: Tatsunori
authors.initials: T
authors.lastname: Shimoi
authors.forename: Kensaku
authors.initials: K
authors.lastname: Shojima
authors.forename: Atsuko
authors.initials: A
authors.lastname: Murota
authors.forename: Yasunobu
authors.initials: Y
authors.lastname: Takizawa
authors.forename: Junko
authors.initials: J
authors.lastname: Maruyama
authors.forename: Keigo
authors.initials: K
authors.lastname: Setoguchi
mesh_terms: D000328:Adult; D000368:Aged; D044466:Asian People; D015267:Churg-Strauss Syndrome / Q000175:diagnosis* / Q000276:immunology; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged