• Hirahito Endo.
• Division of Rheumatology and Infectious Disease, Department of Internal Medicine, Kitasato University School of Medicine.
• Nippon Rinsho. 2009 Mar 1; 67 (3): 536-41.

AbstractPulmonary involvement is a common future in patient with collagen diseases. Some of the pulmonary involvements are a resistant of current available treatment and a fetal condition. These conditions include interstitial pneumonitis in scleroderma, acute diffuse alveolar damage (DAD) in polymyositis/dermatomyositis (PM/DM), alveolar hemorrhage, and pulmonary hypertension in collagen disease. Intensive immunosuppressive treatment in early stage of interstitial pneumonitis may be effective for an intractable condition. Oral cyclophosphamide therapy is effective for non specific interstitial pneumonitis of scleroderma in a randomized control trial. Acute interstitial pneumonitis, so called DAD in patients with PM/DM is a resistant of steroid therapy and a poor prognostic complication. Combination therapy with high dose corticosteroids and immunosuppressive drugs (cyclosporin A, cyclophosphamide) is sometimes available therapy. Diffuse alveolar hemorrhage sometimes occurred in collagen disease including antineutrophilic cytoplasmic antibody (ANCA) associated vasculitis, systemic lupus erythematosus. Intensive treatment need to inhibit hemorrhage.

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