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.

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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,

The MITAX, MYOACT and MDI tools, which are now undergoing validity testing, should enhance the consistency, comprehensiveness and reliability of disease activity and damage assessment in patients with myositis.

Objective. To assess prevalence and characteristics of opportunistic infections in patients with polymyositis/dermatomyositis (PM/DM). To determine the predictive values for opportunistic infections on clinical presentation, biochemical findings, and paraclinical features of PM/DM to detect patients at risk of opportunistic infections. Methods. The medical records of 156 consecutive PM/DM patients in 3 medical centers were reviewed. Results. Eighteen PM/DM patients (11.5%) developed opportunistic infections. The majority of patients exhibited an opportunistic infection after the onset of PM/DM (89% of cases). Opportunistic infections occurred most frequently during the first year following PM/DM diagnosis (62.5%). The pathogen microorganisms responsible for opportunistic infections were various, i.e., Candida albicans, Pneumocystis carinii, Aspergillus fumigatus, Geotrichum capitatum, Mycobacterium avium-intracellulare complex, M. xenopi, M. marinum, M. tuberculosis, Helicobacter heilmanii, cytomegalovirus, and herpes simplex virus. Mortality rates were as high as 27.7% in these PM/DM patients. Higher mean daily doses of steroids, lymphopenia, and lower serum total protein levels were significantly more frequent in the group of PM/DM patients with opportunistic infections. Conclusion. Our study underscores the high frequency of opportunistic infections in PM/DM, resulting in an increased mortality rate. It also indicates that a great variety of microorganisms are responsible for opportunistic infections, although they were more often due to fungi (>50% of cases). Our series highlights a predominance of both lung and digestive opportunistic infections (89% of cases). In addition, our results suggest that PM/DM patients presenting with factors predictive of opportunistic infection may require closer monitoring.

Demographic, clinical, and laboratory features that predict underlying malignancy in patients with dermatomyositis (DM) are poorly known. We conducted a retrospective study in all adult patients with a definite (n = 75) or probable (n = 32) diagnosis of DM according to Bohan and Peter criteria or with amyopathic DM (n = 14) who were referred to 2 departments during a 13-year period. The diagnosis of malignancy-associated DM was retained if DM occurred in a context of recently diagnosed malignancy or if a malignancy was diagnosed during the 5 years following the diagnosis of DM. The Kaplan-Meier method was used to assess the cumulative incidence rates of underlying malignancy during the first 5 years of DM. Factors associated with malignancy in patients with DM were identified by Cox proportional hazards models. During the study period, 121 patients fulfilled the inclusion criteria (median age, 52 yr; range, 19-77 yr; women: 70%). For 29 of them, the diagnosis of malignancy-associated DM was retained. The cumulative incidence rate of malignancy was 21 +/- 4% and 28 +/- 5%, 1 year and 5 years after the diagnosis of DM, respectively. The median duration of follow-up of the 92 patients with no malignancy diagnosed was 36 months (range, 1-140 mo). In multivariate analysis, independent factors associated with an underlying malignancy in patients with DM were an age at diagnosis >52 years (hazard ratio [HR], 7.24; 95% confidence interval [CI], 2.35-22.31), a rapid onset of skin and/or muscular symptoms (HR, 3.11; 95% CI, 1.07-9.02), the presence of skin necrosis (HR, 3.84; 95% CI, 1.00-14.85) or periungual erythema (HR, 3.93; 95% CI, 1.16-13.24), and a low baseline level of complement factor C4 (HR, 2.74; 95% CI, 1.11-6.75). Lastly, low baseline lymphocyte count (<1500/mm(3)) was a protective factor of malignancy (HR, 0.33; 95% CI, 0.14-0.80). Taken together, these data may help physicians focus on a group of patients who might benefit from extensive evaluation for malignancy.

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Objective. To assess the long-term outcome of esophageal complications in the group of patients receiving intravenous immunoglobulins (IVIG) for the treatment of severe steroid-refractory esophageal involvement related to polymyositis/ dermatomyositis (PM/DM). Methods. We retrospectively reviewed the medical records of 73 patients (39 with PM, 34 with DM) with steroid-resistant esophageal involvement. Esophageal involvement was evaluated by clinical and manometric investigations. Results. Seventy-three patients with steroid-refractory esophageal involvement related to PM/DM received IVIG therapy (2 gm/kg monthly). The median interval between PM/DM diagnosis and the onset of esophageal complications was 6 months. The most common clinical manifestations revealing esophageal dysfunction were dysphagia (69.9%), coughing while eating (61.6%), and gastroesophageal reflux into the pharynx and/or mouth (34.2%). Twenty-five patients exhibited life-threatening esophageal complications requiring exclusive enteral feeding; 33 patients (45.2%) with esophageal impairment developed aspiration pneumonia. Sixty patients (82.2%) exhibited resolution of esophageal clinical manifestations, leading to a return to normal oral feeding and ablation of feeding enteral tubes. Four other patients (5.5%) improved, although they still experienced mild dysphagia intermittently. Because of impaired cricopharyngeal muscle relaxation, another patient successfully underwent cricopharyngeal myotomy. Eight patients died from aspiration pneumonia (n ‫؍‬ 6) and cancer (n ‫؍‬ 2). Muscle weakness, thoracic myopathy, and aspiration pneumonia were independent predictive factors of IVIG-treated esophageal complications in PM/DM patients. Conclusion. Our findings indicate that IVIG should be considered in life-threatening esophageal impairment complicating steroid-resistant PM/DM. We also suggest that combined therapy of IVIG and high-dose steroids may be the first-line therapy in PM/DM patients with life-threatening esophageal manifestations.