title: Cyclosporine in anti-Jo1-positive patients with corticosteroid-refractory interstitial lung disease.
abstract: OBJECTIVE
  To describe the longterm effectiveness and safety of cyclosporine (CYC) in patients with anti-Jo1-positive antisynthetase syndrome with corticosteroid-refractory interstitial lung disease (ILD).
  
  
  METHODS
  All patients with anti-Jo1 antisynthetase syndrome referred to our division between June 1991 and February 2010 were retrospectively evaluated for ILD. ILD was assessed using pulmonary function tests (PFT) and/or high-resolution computed tomography (HRCT). Kazerooni score was used to evaluate the HRCT extent of ILD. Prednisone was the first-line treatment in all cases (1 mg/kg/day orally, then tapering). Patients with corticosteroid-refractory or relapsing ILD were then included in this retrospective study. All patients started CYC (3 mg/kg/day) without increasing prednisone dosage. Both PFT and chest HRCT were regularly reassessed during followup.
  
  
  RESULTS
  Over the period of study we evaluated 18 patients with antisynthetase syndrome; 17 had ILD (13 women; median age at ILD onset 57 yrs); all patients failed prednisone within 12 months of ILD onset and subsequently started CYC. The median followup on CYC was 96 months [interquartile range (IQR) 57-120 mo]. Upon starting CYC, median forced vital capacity (FVC) was 60% (IQR 56%-70%), median DLCO 60% (IQR 50%-62.75%), and median Kazerooni score 16 (IQR 7-18). After 1 year of CYC, FVC (p = 0.0006), DLCO (p = 0.0010), and total Kazerooni score (p = 0.0002) improved and prednisone was tapered (median reduced from 25 mg/day to 2.5 mg/day; p < 0.0001). The results were substantially maintained including at last available followup. CYC side effects were hypertension (5 patients) and creatinine increase (6 patients). CYC was reduced in 3 cases and withdrawn in 4. Three out of 4 patients who interrupted CYC experienced ILD relapse; 2 patients recommenced low-dose CYC with subsequent ILD control. One patient refused re-treatment and subsequently died.
  
  
  CONCLUSIONS
  CYC is effective and substantially safe in patients with anti-Jo1 antisynthetase syndrome with corticosteroid-refractory ILD. CYC withdrawal may be associated with ILD relapse, and low-dose CYC was effective in ILD control.
authors: Division of Rheumatology, University and IRCCS Foundation Policlinico S. Matteo, Pavia, Italy. lorenzo.cavagna@unipv.it
authors: Lorenzo
authors: L
authors: Cavagna
authors: Roberto
authors: R
authors: Caporali
authors: Lul
authors: L
authors: Abdì-Alì
authors: Roberto
authors: R
authors: Dore
authors: Federica
authors: F
authors: Meloni
authors: Carlomaurizio
authors: C
authors: Montecucco
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use*; D000328:Adult; D000368:Aged; D000974:Antibodies, Antinuclear / Q000276:immunology*; D016572:Cyclosporine / Q000627:therapeutic use*; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D017563:Lung Diseases, Interstitial / Q000188:drug therapy* / Q000276:immunology; D008297:Male; D008875:Middle Aged; D009220:Myositis / Q000188:drug therapy* / Q000276:immunology; D011241:Prednisone / Q000627:therapeutic use*; D012129:Respiratory Function Tests; D019233:Retreatment; D016896:Treatment Outcome; D014797:Vital Capacity
chemical_list: D000305:Adrenal Cortex Hormones; D000974:Antibodies, Antinuclear; D007166:Immunosuppressive Agents; C046750:Jo-1 antibody; D016572:Cyclosporine; D011241:Prednisone