title: Pre-treatment ferritin level and alveolar-arterial oxygen gradient can predict mortality rate due to acute/subacute interstitial pneumonia in dermatomyositis treated by cyclosporine a/glucocorticosteroid combination therapy: a case control study [corrected].
abstract: BACKGROUND
  Acute/subacute interstitial pneumonia in dermatomyositis (DM-A/SIP) is a disease associated with a poor prognosis that resists treatment with glucocorticosteroids (GC) and progresses rapidly in a period of weeks to months to death. We retrospectively studied outcomes, prognostic factors, and their relations with survival rate in patients with DM-A/SIP treated with early cyclosporine A (CSA)/GC combination therapy and 2-hour postdose blood concentration monitoring.
  
  
  METHODS
  This study comprised 32 DM-A/SIP patients who were simultaneously treated with CSA and prednisolone. Clinical and laboratory findings were compared between those who died due to DM-A/SIP and those surviving 24 weeks after beginning of therapy. Prognostic factors were extracted, and their relations with the survival rate were evaluated.
  
  
  RESULTS
  Of the 32 DM-A/SIP patients, 25 survived, 5 died of DM-A/SIP, and 2 died of infections. In those who died due to DM-A/SIP, ferritin level and the alveolar-arterial oxygen gradient were significantly increased compared with the survivors (P<0.001 and P = 0.002, respectively). Multivariate analyses showed that ferritin and alveolar-arterial oxygen gradient were independent prognostic factors of poor outcome. The survival rate 24 weeks after beginning of treatment was significantly lower in those with a ferritin level of ≥ 600 ng/ml and alveolar-arterial oxygen gradient of ≥ 45 Torr (P<0.001 and P<0.001, respectively). All patients with both prognostic factors died, and the outcome was significantly poorer in these patients than in those with one or neither of the prognostic factors (P<0.001).
  
  
  CONCLUSIONS
  We identified pre-treatment high serum ferritin level and high alveolar-arterial oxygen gradient as poor prognostic factors in DM-A/SIP patients undergoing early CSA/GC combination therapy and showed that the outcomes were poor in patients with both factors.
authors.affiliation: Department of Internal Medicine (I), Osaka Medical College, Takatsuki, Osaka, Japan.
authors.forename: Kentaro
authors.initials: K
authors.lastname: Isoda
authors.affiliation: Department of Internal Medicine (I), Osaka Medical College, Takatsuki, Osaka, Japan.
authors.forename: Tohru
authors.initials: T
authors.lastname: Takeuchi
authors.affiliation: Department of Internal Medicine (I), Osaka Medical College, Takatsuki, Osaka, Japan.
authors.forename: Takuya
authors.initials: T
authors.lastname: Kotani
authors.affiliation: Department of Internal Medicine (I), Osaka Medical College, Takatsuki, Osaka, Japan.
authors.forename: Kenichiro
authors.initials: K
authors.lastname: Hata
authors.affiliation: Department of Internal Medicine (I), Osaka Medical College, Takatsuki, Osaka, Japan.
authors.forename: Takeshi
authors.initials: T
authors.lastname: Shoda
authors.affiliation: Department of Internal Medicine (I), Osaka Medical College, Takatsuki, Osaka, Japan.
authors.forename: Takaaki
authors.initials: T
authors.lastname: Ishida
authors.affiliation: Department of Internal Medicine (I), Osaka Medical College, Takatsuki, Osaka, Japan.
authors.forename: Shuzo
authors.initials: S
authors.lastname: Yoshida
authors.affiliation: Department of Internal Medicine (I), Osaka Medical College, Takatsuki, Osaka, Japan.
authors.forename: Yuko
authors.initials: Y
authors.lastname: Kimura
authors.affiliation: Department of Internal Medicine (I), Osaka Medical College, Takatsuki, Osaka, Japan.
authors.forename: Shigeki
authors.initials: S
authors.lastname: Makino
authors.affiliation: Department of Internal Medicine (I), Osaka Medical College, Takatsuki, Osaka, Japan.
authors.forename: Toshiaki
authors.initials: T
authors.lastname: Hanafusa
mesh_terms: D000368:Aged; D015415:Biomarkers / Q000097:blood; D016022:Case-Control Studies; D016572:Cyclosporine / Q000627:therapeutic use*; D003882:Dermatomyositis / Q000097:blood* / Q000401:mortality; D004359:Drug Therapy, Combination; D005260:Female; D005293:Ferritins / Q000097:blood*; D005938:Glucocorticoids / Q000627:therapeutic use*; D006801:Humans; D053208:Kaplan-Meier Estimate; D017563:Lung Diseases, Interstitial / Q000097:blood* / Q000188:drug therapy / Q000401:mortality; D008297:Male; D008875:Middle Aged; D018396:Mucin-1 / Q000097:blood; D010100:Oxygen / Q000097:blood*; D011239:Prednisolone / Q000627:therapeutic use*; D011379:Prognosis; D016016:Proportional Hazards Models; D011650:Pulmonary Alveoli / Q000098:blood supply; D012189:Retrospective Studies
chemical_list: D015415:Biomarkers; D005938:Glucocorticoids; C060192:MUC1 protein, human; D018396:Mucin-1; D016572:Cyclosporine; D005293:Ferritins; D011239:Prednisolone; D010100:Oxygen