THE LUNG IN POLYMYOSITIS - 09/09/11
Résumé |
Polymyositis (PM) is an idiopathic inflammatory myopathy with systemic manifestations and mediated by autoimmune and cellular mechanisms. It results in proximal muscle weakness and often chronic disability. Systemic complications, in particular, lung involvement, are responsible for increased morbidity and mortality. In the United States 5 to 10 new cases of PM or its related disorder dermatomyositis (DM) per million population is discovered yearly.58 Women are more apt to be affected than men, and there is a predilection for African-American women.52 There are two disease peaks: one in children and the other in young to middle-aged adults.28 A classification of PM follows54:
• | Adult polymyositis |
• | Adult dermatomyositis |
• | Childhood polymyositis/dermatomyositis |
• | Association with an underlying malignancy |
• | Complicating an established collagen vascular disease |
Polymyositis and DM are similar except for the prominent dermatologic rash and less muscle involvement in DM. The onset of PM or DM in an elderly patient may be the result of an underlying malignancy. Although sometimes disputed, Manchel et al, using strict criteria, found malignancy in 15 of 71 patients with PM or DM.45 These subjects were over 50 years with primaries in either the colon, breast, prostate, lung, or uterus. The neoplasm either predated, appeared concomitantly with, or became apparent at some point following the diagnosis of PM or DM. Neoplasms that preceded the onset were present for up to 20 years before the episode of PM or DM. In malignancies that appeared after the diagnosis of PM or DM, there was a 1- to 4-year delay. Two other studies indicated that DM but not PM is associated with an increased incidence of malignancy.1, 88
Polymyositis can also complicate the course of another collagen vascular disease and myositis can be a prominent manifestation of systemic lupus erythematosus, scleroderma, mixed connective tissue disease, and less frequently rheumatoid arthritis. This form of the inflammatory myopathy invariably occurs in women.52
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| Address reprint requests to Marvin I. Schwarz, MD, Division of Pulmonary Sciences and Critical Care Medicine, Box C272, University of Colorado Health Sciences Center, 4200 E. 9th Avenue, Denver, CO 80262 Supported by SCOR Grant #HL27353-04 from the National Heart, Lung and Blood Institute, National Institutes of Health. |
Vol 19 - N° 4
P. 701-712 - décembre 1998 Retour au numéroBienvenue sur EM-consulte, la référence des professionnels de santé.
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