Cyclosporin A

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Cyclosporin A in the Treatment of Refractory Adult Polymyositis/

Dermatomyositis: Population Based Experience in 6 Patients and

Literature Review

KHALID A. QUSHMAQ, ANDREW CHALMERS, and JOHN M. ESDAILE

ABSTRACT.

Objective. To evaluate the efficacy and toxicity of cyclosporin A (CSA)

in the treatment of refractory adult polymyositis/dermatomyositis

(PM/DM).

Methods. The province-wide British Columbia database for CSA use

for persons with rheumatic diseases at Pack Arthritis Centre was

reviewed to identify all patients with PM/DM for the period January 1991

through June 1998.

Also, a Medline search of English language literature was conducted for

this topic from 1976 until January 1999, using the terms

dermatomyositis, polymyositis, inflammatory myopathy, and cyclosporin A,

and the reference lists of all papers were screened to include articles

not identified by the Medline search.

Results. In British Columbia, 172 CSA users of whom 6 had PM/DM

were identified (4 PM, 2 DM). Previous therapy included high dose

prednisone (N = 6), methotrexate (N = 4), azathioprine (N = 4),

intravenous immunoglobulin (N = 3), and cyclophosphamide (N = 3). The

mean CSA dose was 3.5 mg/kg/day. All patients improved. Creatinine

kinase (CK) levels declined 52% from baseline. All 6 patients continued

CSA a median of 6 months (range 3-44 mo) after initiation of therapy.

Toxicity included an increase in serum creatinine > 30% of baseline in 3

patients and hypertension in one patient. The literature review

identified an additional 59 cases. Forty-eight (81%) had a reduction in

CK levels and improved clinically, 9/59 (15%) developed nephrotoxicity,

5/59 (8%) hypertension responsive to dose reduction, and 9/59 (15%) had

hypertrichosis, gingival hyperplasia, or tremor.

Conclusion. Our population based experience with 6 patients and

the 59 published cases suggests CSA is an effective therapy for

resistant PM/DM, and toxicity is possibly more than expected in other

rheumatic diseases. (J Rheumatol 2000;27:2855-9)