Elsevier

Clinics in Chest Medicine

Volume 19, Issue 4, 1 December 1998, Pages 701-712
Clinics in Chest Medicine

THE LUNG IN POLYMYOSITIS

https://doi.org/10.1016/S0272-5231(05)70111-8Get rights and content

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:

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

Section snippets

CLINICAL PRESENTATION AND HISTOLOGY

The most obvious clinical feature of PM is a progressive proximal muscle weakness evolving over several weeks to several months. Typically, walking upstairs, brushing one's hair, and getting up from a seated position are acts that are difficult to perform. Mastication or other muscular acts involving facial muscles are usually unaffected.58 Muscle tenderness and myalgias are expected, but severe pain is unusual. Progressive and recurrent disease results in atrophy of the affected muscles and

PULMONARY COMPLICATIONS

The possible causes for pulmonary disease in PM are outlined in the list above. Recurrent aspiration pneumonia is possibly the most frequently encountered pulmonary complication of PM and adds significantly to the morbidity and mortality of this disease because of the development of bacterial pneumonia, lung abscess, and sometimes the acute respiratory distress syndrome.4, 25, 48 Aspiration pneumonia has been reported to occur in 15% to 20% of patients but 40% to 45% report some degree of

TREATMENT OF INTERSTITIAL LUNG DISEASE

In general, response to treatment of ILD depends largely on the underlying histology. In patients with the more cellular forms of ILD (i.e., bronchiolitis obliterans organizing pneumonia, cellular interstitial pneumonia, and pulmonary capillaritis) opposed to those with advanced usual interstitial pneumonitis honeycomb lung and diffuse alveolar damage, there is a response to treatment with corticosteroids alone or in combination with another immunosuppressive agent, such as cyclophosphamide or

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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.

    *

    Department of Medicine, Division of Pulmonary Sciences and Critical Care Medicine, University of Colorado Health Sciences Center, Denver, Colorado

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