[Nation-wide survey for the treatment with cyclosporin A of interstitial pneumonia associated with collagen diseases].
OBJECTIVE
This study was performed to investigate the efficacy and safety of cyclosporin A (CsA) for the treatment of interstitial pneumonia (IP) associated with collagen diseases in Japan.


METHODS
Questionnaires were sent to 36 hospitals specializing in collagen diseases.


RESULTS
Fifty-eight patients (7 polymyositis (PM), 19 dermatomyositis (DM), 7 systemic sclerosis (SSc), 7 rheumatoid arthritis (RA), 2 mixed connective tissue disease (MCTD), 1 systemic lupus erythematosus (SLE) and 1 Sjögren's syndrome (SS), 1 RA + SSc, 2 PM + SSc, 1 DM + SLE, and 10 idiopathic interstitial pneumonia (IIP) with IP were treated with CsA at 14 hospitals. IP was classified into the acute or chronic type. In the PM/DM group (7 PM, 19 DM, 2 PM + SSC, 1 DM + SLE), 65.5% were the acute type. In the other collagen disease group (7 SSc, 7 RA, 2 MCTD, 1 SLE, 1 SS, and 1 RA + SSc) and IIP group, 36.8% and 50% were the acute type, respectively. Mean dosages of CsA were 3.7 +/- 1.3 mg/kg/day for the PM/DM group, 3.0 +/- 1.0 for the other collagen disease group, and 3.8 +/- 4.8 for the IIP group. Oral corticosteroids were administered in combination with CsA in 100, 73.7, and 70% of the patients with PM/DM, other collagen disease, and IIP groups, respectively. CsA was effective for 72.2, 33.3, and 25% of the acute IP cases in the PM/DM, other collagen disease, and IIP groups, respectively. CsA was effective for 50.0, 50.0, and 60.0% of chronic IP cases in the PM/DM, other collagen disease, and IIP groups, respectively. Twenty-three adverse effects were observed, but most of them ameliorated upon withdrawal or reduction of the CsA dose.


CONCLUSIONS
CsA is effective for the treatment of acute type IP associated with collagen diseases, especially PM/DM. To perform a prospective multi-center trial, standards for the recruitment of patients, efficacy assessments, and trial course and treatment should be determined carefully.
Harigai
M
M
Institute of Rheumatology, Tokyo Women's Medical University, Kawada-cho, Shinjuku-ku.
Hara
M
M
Kamatani
N
N
Kashiwazaki
S
S
D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D003095:Collagen Diseases / Q000150:complications*; D016572:Cyclosporine / Q000627:therapeutic use*; D003882:Dermatomyositis / Q000150:complications; D004359:Drug Therapy, Combination; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D017563:Lung Diseases, Interstitial / Q000150:complications / Q000188:drug therapy*; D008297:Male; D017285:Polymyositis / Q000150:complications; D011239:Prednisolone / Q000627:therapeutic use; D011446:Prospective Studies; D011795:Surveys and Questionnaires; D016896:Treatment Outcome
D000893:Anti-Inflammatory Agents; D007166:Immunosuppressive Agents; D016572:Cyclosporine; D011239:Prednisolone