Douglas, William W.

Abstract We report findings in 70 patients with both diffuse interstitial lung disease and either polymyositis (PM) or dermatomyositis (DM). Initial presentations were most commonly either musculoskeletal (arthralgias, myalgias, and weakness) or pulmonary (cough, dyspnea, and fever) symptoms alone; in only 15 patients (21.4%) did both occur simultaneously. Pulmonary disease usually took the form of acute to subacute antibiotic-resistant community-acquired pneumonia. Chest radiographs and computed tomography most commonly demonstrated bilateral irregular linear opacities involving the lung bases; occasionally consolidation was present. Jo-1 antibody was present in 19 (38%) of 50 patients tested. Synchronous associated malignancy was present in 4 of 70 patients (5.7%). Surgical lung biopsies disclosed nonspecific interstitial pneumonia (NSIP) in 18 of 22 patients (81.8%), organizing diffuse alveolar damage (DAD) in 2, bronchiolitis obliterans organizing pneumonia (BOOP) in 1, and usual interstitial pneumonia (UIP) in 1. Treatment usually included prednisone in 40–60 mg/d dosages for initial control, followed by lower dose prednisone plus an immunosuppressive agent such as azathioprine or methotrexate for disease suppression. Survival was significantly better than that observed for historical control subjects with idiopathic UIP, and was more consistent with survival previously reported in idiopathic NSIP. There was no difference in survival between Jo-1 positive and Jo-1 negative groups.

Abstract The clinical records of 487 patients seen at Mayo Clinic Rochester (MCR) during 1994 through 1996 who were diagnosed as having idiopathic pulmonary fibrosis of the usual interstitial pneumonia type (IPF/UIP) were reviewed. Computed tomography (CT) had been done in 94.7% of patients and surgical open lung biopsy (OLB) in 20.3%. Median survival was 3.2 yr. We compared survival as a function of treatment program, based on intent to treat at the time of the initial contact within the study window. Treatment advised included colchicine alone in 167, no therapy in 157, prednisone at maintenance dosages alone in 54, colchicine plus prednisone in 71, and other programs in 38. Oxygen therapy was advised in 133 patients. By univariate analysis, worse survival was associated with prednisone therapy compared with no therapy, and with oxygen therapy compared with no oxygen therapy. However, on multivariate analysis, worse survival was associated with older age, male gender, lower diffusing capacity for carbon monoxide (DlCO), lower alveolar volume (Va), and a history of worsening pulmonary function. When adjustment was made for these factors, there was no significant difference in survival between those patients treated with colchicine or prednisone and those on no therapy, and no difference between those on oxygen therapy and those without oxygen.