@article {Cavagna484, author = {Lorenzo Cavagna and Roberto Caporali and Lul Abd{\`\i}-Al{\`\i} and Roberto Dore and Federica Meloni and Carlomaurizio Montecucco}, title = {Cyclosporine in Anti-Jo1-positive Patients with Corticosteroid-refractory Interstitial Lung Disease}, volume = {40}, number = {4}, pages = {484--492}, year = {2013}, doi = {10.3899/jrheum.121026}, publisher = {The Journal of Rheumatology}, 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{\textendash}120 mo]. Upon starting CYC, median forced vital capacity (FVC) was 60\% (IQR 56\%{\textendash}70\%), median DLCO 60\% (IQR 50\%{\textendash}62.75\%), and median Kazerooni score 16 (IQR 7{\textendash}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 CYC was reduced in 3 cases and withdrawn in 4.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. Conclusion. 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.}, issn = {0315-162X}, URL = {https://www.jrheum.org/content/40/4/484}, eprint = {https://www.jrheum.org/content/40/4/484.full.pdf}, journal = {The Journal of Rheumatology} }