Interstitial lung disease in polymyositis and dermatomyositis

Abstract

Objectives. To assess prevalence, characteristics, and long-term outcome of interstitial lung disease (ILD) in polymyositis (PM) and dermatomyositis (DM). To determine predictive variables of ILD course in PM/DM, and to define both clinical and biochemical features associated with ILD onset in PM/DM. Methods. The medical records of 156 consecutive PM/DM patients in 3 medical centers were reviewed. Results. Thirty-six PM/DM patients (23.1%) developed ILD. We observed that 19.4% of patients with ILD had resolution of pulmonary disorders, whereas 25% experienced ILD deterioration. Morbidity and mortality rates were as high as 13.9% and 36.4%, respectively, in PM/DM patients with ILD. Parameters of PM/DM that related to ILD poor outcome were identified as follows: Hamman-Rich-like pattern, initial diffusing capacity of carbon monoxide <45%, neutrophil alveolitis, and histologic usual interstitial pneumonia. Additionally, for the group with ILD, polyarthritis, higher values of erythrocyte sedimentation rate and C-reactive protein, presence of anti-Jo-1 antibody, and characteristic microangiopathy were significantly more frequent. Conclusion. Our series underlines the high frequency of ILD in PM/DM patients, resulting in increased morbidity and mortality rates. It also indicates that PM/DM patients should routinely be screened for ILD, even those patients without anti-Jo-1 antibody, because 69% of our ILD patients were seronegative for the anti-Jo-1 antibody. Our findings further suggest that PM/DM patients presenting with factors predictive of ILD poor outcome may require more aggressive therapy.

Opportunistic infections in polymyositis and dermatomyositis

“…PM/DM has been reported to be associated with increased mortality, with mortality rates ranging from 4% to 50% (1)(2)(3)(4)(5). Malignancy, pulmonary and cardiac involvement, and infections are generally cited as the most common causes of death in PM/DM patients (1)(2)(3)(4)(5).…”

“…Polymyositis (PM) and dermatomyositis (DM) are systemic inflammatory disorders affecting skeletal muscles and other organs (1)(2)(3)(4)(5). PM and DM are considered to be associated with high morbidity and mortality rates, in some cases as high as 50%, primarily related to life-threatening muscle weakness, cardiac and lung impairment, as well as infectious manifestations (1)(2)(3)(4)(5).…”

“…PM and DM are considered to be associated with high morbidity and mortality rates, in some cases as high as 50%, primarily related to life-threatening muscle weakness, cardiac and lung impairment, as well as infectious manifestations (1)(2)(3)(4)(5). In clinical series of patients with PM/DM, infectious complications have been described in up to 26% of patients (1,2,4,6).…”

Clinical manifestations of dermatomyositis and clinically amyopathic dermatomyositis patients with positive expression of anti–melanoma differentiation–associated gene 5 antibody

“…We tend to use the term "dermatomyositis-like skin syndrome" to describe those patients who have distinctive or predominantly cutaneous manifestations of DM without clinical evidence of myositis, since patients diagnosed with this syndrome are at a higher risk for developing interstitial lung disease (ILD), malignancy, and/or delayed onset of myositis (2). Patients with DM/CADM showed a lower 5-year survival rate than patients with PM (3)(4)(5). The direct cause of a lower survival rate is due to further development of ILD from DM/CADM, excluding malignant diseases.…”

Anti–Jo‐1 antibody levels correlate with disease activity in idiopathic inflammatory myopathy

“…Initially thought to be a marker of inflammatory myopathy alone, anti-Jo-1 antibody is now associated with a distinct clinical entity known as the antisynthetase syndrome, which includes fever, myositis, interstitial lung disease (ILD), nonerosive arthropathy, mechanic's hands, and Raynaud's phenomenon (5). ILD is especially prevalent in the antisynthetase syndrome, occurring in ϳ75% of patients with anti-Jo-1 antibody compared to ϳ30% of patients with IIM in the absence of antisynthetase antibodies (6)(7)(8).…”