Suzuki, Y. et al. Intravenous immunoglobulin therapy for refractory interstitial lung disease associated with polymyositis/dermatomyositis. Lung 187, 201–206 (2009).

Interstitial lung disease (ILD), especially in its acute forms, is a major prognostic determinant in patients with polymyositis and dermatomyositis that can cause increased morbidity and mortality, often despite intensive treatment with anti-inflammatory and immunosuppressive agents, such as ciclosporin and cyclophosphamide. A report by Yuzo Suzuki and colleagues suggests that investigating intravenous immunoglobulin (IVIG) as a salvage therapy might be warranted.

The investigators were prompted to look into the usefulness of IVIG in this context by reports that it is a highly effective and safe treatment for polymyositis-associated myopathy refractory to conventional therapy. A retrospective review identified five patients, one diagnosed with polymyositis and four with amyopathic dermatomyositis, who had been treated with IVIG for acute, refractory ILD between 1985 and 2007. Three patients died of acute respiratory failure within 3 months of admission, but two survived. Clinical characteristics and clinical course did not differ significantly between those who survived and those who did not. The treatment was well tolerated, with no adverse effects reported.

The results of this small study suggest that IVIG is a safe and effective salvage therapy for some, but not all, patients with ILD associated with polymyositis and dermatomyositis resistant to therapy with corticosteroids and immunosuppressive agents. Given the extremely poor prognosis of these patients, further investigations of the efficacy of IVIG for such cases could be warranted.