Short‐term and long‐term outcomes of interstitial lung disease in polymyositis and dermatomyositis: A series of 107 patients

Abstract

Objective. This study was undertaken to assess the characteristics and outcome of interstitial lung disease (ILD) in polymyositis/dermatomyositis (PM/ DM) and to determine variables predictive of ILD deterioration in PM/DM.Methods. Among 348 consecutive patients with PM/DM, 107 patients with ILD were identified by medical records search in 4 medical centers. All patients underwent pulmonary function tests (PFTs) and pulmonary high-resolution computed tomography (HRCT) scan.Results. ILD onset preceded PM/DM clinical manifestations in 20 patients, was identified concurrently with PM/DM in 69 patients, and occurred after PM/DM onset in 18 patients. Patients with ILD could be divided into 3 groups according to their presenting lung manifestations: patients with acute lung disease (n ‫؍‬ 20), patients with progressive-course lung signs (n ‫؍‬ 55), and asymptomatic patients with abnormalities consistent with ILD evident on PFTs and HRCT scan (n ‫؍‬ 32). We observed that 32.7% of the patients had resolution of pulmonary disorders, whereas 15.9% experienced ILD deterioration. Factors that predicted a poor ILD prognosis were older age, symptomatic ILD, lower values of vital capacity and diffusing capacity for carbon monoxide, a pattern of usual interstitial pneumonia on HRCT scan and lung biopsy, and steroid-refractory ILD. The mortality rate was higher in patients with ILD deterioration than in those without ILD deterioration (47.1% versus 3.3%).Conclusion. Our findings indicate that ILD results in high morbidity in PM/DM. Our findings also suggest that more aggressive therapy may be required in PM/DM patients presenting with factors predictive of poor ILD outcome. Polymyositis (PM) and dermatomyositis (DM)are systemic inflammatory disorders that affect skeletal muscles and other organs, especially the lungs, either primarily or through complications of muscle weakness, resulting in interstitial lung disease (ILD), ventilatory insufficiency, and aspiration pneumonia (1-4). The prevalence of pulmonary involvement has been reported to be as high as 46% in PM/DM, and pulmonary disorders are still considered to be a common cause of morbidity in PM/DM (1-4). ILD may lead to lifethreatening complications, i.e., ventilatory failure, secondary pulmonary arterial hypertension, or cor pulmonale (2,(5)(6)(7)(8)(9). Previous studies of small series have shown that once pulmonary involvement was recognized in PM/DM, the 5-year mortality rate ranged from 0 to 55% (5,7,10). The early detection of ILD is, therefore, a high priority in PM/DM patients. The aims of this retrospective study were to assess the characteristics and outcome of ILD in patients with PM/DM and to determine factors that are predictive of ILD deterioration in PM/ DM. PATIENTS AND METHODSIdentification of the patients. This retrospective study began with a search of the institutional centers' medical record 1 I. Marie, MD, PhD: Centre Hospitalier Universitaire Rouen, INSERM U 905,

Nuclear actin aggregation is a hallmark of anti-synthetase syndrome–induced dysimmune myopathy

“…5 In antisynthetase syndrome, myositis may present as either dermatomyositis (DM) or polymyositis (PM) [8][9][10] ; however, this association seems to be arbitrary, and is not clinically helpful.…”

“…6,7 Although it is unclear why AARS are recognized as autoantigens, theories that link ASA to autoimmunity have been proposed. 5 In antisynthetase syndrome, myositis may present as either dermatomyositis (DM) or polymyositis (PM) [8][9][10] ; however, this association seems to be arbitrary, and is not clinically helpful.…”

Pulmonary hypertension in antisynthetase syndrome: prevalence, aetiology and survival

“…Moreover, certain causes require specific treatments. These treatments could be essential, as the long-term prognosis and the survival of patients with ASS, based on retrospective studies, showed a clear correlation with lung involvement [2,[18][19][20]. However, to date, the influence of cofactors associated with ILD, such as PH, has rarely been evaluated in large series.…”

Idiopathic Inflammatory Myopathies: A Case of a Woman with Antisynthetase Syndrome

“…The adverse clinical outcome, with relatively high morbidity and mortality rates compared with those of other forms of inflammatory myosites, is primarily due to irreversible Some of the most common clinical entities and the corresponding HRCT patterns and histological features observed in patients with ASS are: 1) Cryptogenic Organizing Pneumonia (COP), characterized by patchy consolidations and/or nodules [56][57][58][59][60][61] ; the majority of histopatological changes center on small airways. There is a mild associated interstitial inflammatory infiltrate, type II cell metaplasia, and an increase in alveolar macrophages, some of which may be foamy.…”