cyclosporine a in the treatment of interstitial pneumonitis associated with sjogren's syndrome

Conditions: Sjogren's Syndrome

Summary: the purpose of this large multicenter, randomized, double-blinded, controlled clinical study is to investigate the efficacy and safety of cyclosporin a for primary sjogren's syndrome associated pneumonitis(pss-ip), which has important implications for the establishment of standardized diagnosis and treatment of pss-ip.


Eligibility criteria:
inclusion criteria: * patients meeting the 2002 or 2012 pss criteria; * patients meeting the diagnostic criteria of interstitial pneumonitis(ip); * patients with exertional dyspnea consistent with grade 2 on the magnitude of task component of the mahler modified dyspnea index; * pulmonary function test: patients with percentages of forced vital capacity (fvc) to predicted values≥45%, percentage of diffusing capacity of carbon monoxide (dlco) to predicted values≥30%, forced expiratory volume in one second (fev1) / fvc\> 65%; * for patients who received oral glucocorticoid, the doses should be no more than 10 mg/d (or equivalent amount of other types of glucocorticoids); * patients who had not received any prior treatment with immunosuppressants (including but not limited to cyc, csa, azathioprine(aza), tacrolimus(fk-506), methotrexate, leflunomide, etc.) or had discontinued the therapy above for at least 3 months; for patients who received hydrochloroquine(hcq), the doses should be stabilized for at least 3 months; * patients who had not received any prior treatment with biological agents, including but not limited to rituximab, infliximab, adalimumab, etanercept, etc., or had discontinued therapy for at least three months; * for patients who had prior treatment with n-acetylcysteine, the doses of above drugs should be stabilized for at least 3 months; * the women of reproductive age who had a negative urine pregnancy test. the women and men of reproductive age must receive effective contraceptive measures from the screening period to last administration of drugs; * patients who were able to read, to understand and to sign informed consent. exclusion criteria: patients who met any of the following criteria will not participate in this study. * patients with acute exacerbation of ip(aeip); * arterial blood gas analysis showed respiratory failure; * patients with lung diseases other than ip: 1. patients with severe pulmonary hypertension who require specific treatments assessed by the rheumatology and immunology experts in various clinical centers; 2. patients with a history of smoking within the last 6 months or current smokers; 3. patients with other serious lung diseases, such as lung tumor or active pulmonary infection; 4. lung biopsy, alveolar lavage or high-resolution computerized tomography (hrct) suggested serious lung diseases other than ip; * patients with other rheumatic autoimmune diseases, including but not limited to rheumatoid arthritis, systemic lupus erythematosus, inflammatory myopathy, systemic sclerosis, primary biliary cirrhosis, etc.; * patients with serious heart, liver, kidney diseases, hematologic and/ or endocrine diseases: 1. heart diseases: decompensated heart failure or refractory hypertension; clinically important abnormal ecg that may lead to unacceptable risks to enrolled patients at screening; 2. liver function: alanine aminotransferase (alt) and/or aspartate aminotransferase (ast) ≥2 times the upper limit of normal (uln); 3. renal function: renal tubular and/or interstitial diseases, renal insufficiency: serum creatinine≥2 uln or glomerular filtration rate (egfr) \<90 ml/min/1.73 m2; 4. white blood cell (wbc) count \<3 ×109/l and/or hemoglobin (hb) \<100 g/l and/or platelet (plt) count \<80×109 /l; 5. other serious diseases: such as cancer, etc.; * patients with active infection or other diseases which will be aggravated with treatment of glucocorticoid and immunosuppressive therapy; * patients positive for hbsag or hepatitis c antibody; * women during pregnancy or lactation, or cannot guarantee effective contraception; * patients who did not cooperate with treatment for mental illness or other reasons; * patients who had allergic constitution or were allergic to many drugs; * patients who were allergic or intolerant to csa, cyc, or glucocorticoid.