Kameda H et al. (2005) Combination therapy with corticosteroids, cyclosporine A, and intravenous pulse cyclophosphamide for acute/subacute interstitial pneumonia in patients with dermatomyositis. J Rheumatol 32: 1719–1726

Patients with polymyositis or dermatomyositis are at risk of developing acute or subacute interstitial pneumonia (A/SIP), which can be fatal despite high-dose prednisolone therapy.

Kameda and colleagues undertook a pilot trial of combined immunosuppressive therapy (high-dose prednisolone, 10–30 mg/kg intravenous pulse cyclophosphamide every 3–4 weeks and 2–4 mg/kg/day cyclosporine A) for A/SIP in patients with polymyositis or dermatomyositis. In the groups included in this study, all patients who went on to develop A/SIP had dermatomyositis. Analysis of the outcomes of patients with A/SIP treated with conventional regimens prior to the trial showed that 75% died despite immunosuppressive therapy (single or sequential). Use of the pilot combined regimen in 10 patients with A/SIP doubled the survival rate to 50%: 5 patients survived and are doing well 2 years later; 5 patients died of respiratory failure within 3 months. Analysis of clinical findings in those patients who developed A/SIP showed characteristic features of mild myositis, palmar papule, fever and negative or low titer of antinuclear antibodies. Of these, the authors considered palmar papules to be a likely indication of the presence or development of A/SIP and potential fatal outcome.

The immediate use of combined immunosuppressive therapy improved outcome in patients with A/SIP and dermatomyositis, although early and aggressive treatment was still unsuccessful in around half of such patients.