Prognostic factors in polymyositis/dermatomyositis. A computer‐assisted analysis of ninety‐two cases

Abstract

An effort was made to identify all patients with polymyositis/dermatomyositis (PM/DM) admitted to hospitals in Israel from 1956-1976. The diagnosis of PM/DM was retrospectively reviewed in 92 (46 definite, 26 probable, and 20 possible) cases. The most common complaints and physical findings in the course of the disease were muscle weakness (86 patients), rash (53 patients), arthritis or arthralgia (39 patients), and dysphagia (35 patients). Elevated serum aldolase levels were found in 64% of the patients for whom data were available; 92% had abnormal electromyogram results, and 60.9% had muscle histopathology consistent with PM/DM. Malignancy was diagnosed in 13 patients. Malignancy, ischemic heart disease, and pulmonary complications were the most common causes of death. The actuarial survival curve was heterogeneous, with an accelerated mortality during the first year after diagnosis and a slower mortality during the following 7 years. Independent unfavorable prognostic signs were: failure to induce remission, leukocytosis, fever, older age, a shorter disease history, and dysphagia.

Dermatomyositis a Diagnositic Delimma: An Interesting Case Series and Review of Literature

“…The disease may unexpectedly remit in as many as 20% of the cases and Pulmonary and cardiac manifestations are the main cause of mortality in dermatomyositis. The prognostic criteria contains persistent skin rash and cardiac or pulmonary involvement [9]. Long term consequences are seen in 30% of patients.…”

A Fatal Presentation of Dermatomyositis

“…22 Esophageal motor activity disorders related to PM/DM may be responsible for swallowing dis-orders, including difficulty with solids and liquids, pain and pre-prandial discomfort in the sternal area, gastro esophageal reflux into the pharynx and/or mouth, coughing while eating, as well as life-threatening complications (e.g., aphagia to solids/liquids requiring total enteral feeding and aspiration pneumonia). 12,[21][22][23][24] Cardiac impairment may occur in up to 50% of patients, although it is more often asymptomatic in DM. 1,25 Various abnormalities have been described, including mainly conduction defects and primarily endrhythm disturbances; congestive heart failure, pericarditis, and valvular disease are severe, less frequent cardiac complications in PM/DM patients.…”

“…1,25 Evidence for an association between PM/DM and solid tumors came first from case reports, with the reported rate of cancer varying from 6% to 60%. 1,12,23,[26][27][28][29][30] Lab investigation shows raised muscle enzymecreatine kinase, lactate dehydrogenase, aldolase, and aminotransferases; and serological test can be ANA positive, Anti Jo positive. Autoantibodies are found in a majority of patients.…”

Dermatomiosite: estudo de 109 pacientes avaliados no HCFMUSP

“…Na Austrália, estudando-se a incidência de malignidade em miopatias inflamatórias, a DM foi delas a que apresentou a maior associação (Buchbinder et al, 2001). A relação entre DM e neoplasia encontrada em nosso trabalho foi pequena comparada a outros estudos publicados (Benbassat et al, 1985;Pollak, 1986;Gallais et al, 1996;Maoz et al, 1998;Dourmishev, 1999;Chen et al, 2001;Stockton et al, 2001;Sparsa et al, 2002;Pinto et al, 2003;Mebazâa et al, 2003).…”

“…Em trabalhos como os de Bohan et al (1977), Benbassat et al (1985) e Maugars et al (1996), o FAN foi positivo respectivamente em 2%, 23,6% e 18,9% dos casos, porém índices maiores ocorreram com Tymms e Webb (1985), Koh et al (1993) e Weitoft (1997, onde se encontraram, respectivamente, 52%, 47,3% e 52,6% de positividade. Publicações mais recentes mostram a positividade do FAN na maioria dos pacientes (Drake et al, 1996;Kovacs, Kovacs, 1998) e nosso trabalho ratifica isto.…”