Collagen lung disease polymyositis

by Nathan Wei, MD, FACP, FACR

Nathan Wei is a nationally known board-certified rheumatologist and author of the Second Opinion Arthritis Treatment Kit. It's available exclusively at this website... not available in stores.

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The collagen vascular diseases (CVD) are a group of autoimmune disorders that share clinical characteristics, including inflammation of multiple organ systems including the musculoskeletal system, the cardiopulmonary system, and vascular system.

The frequency of cardiopulmonary involvement is impressive: Two thirds of 109 patients in one study showed x-ray changes in lungs, pleura or heart; pathologic lesions were found at autopsy in 28 of 34 cases in another study.

While these disorders can often be categorized into a specific "niche", often, there is overlap.

Weakness of the muscles that permit speech and swallowing may lead to aspiration pneumonia. Patients may also experience shortness of breath secondary to weakness involving the muscles of the diaphragm and chest wall.

Lung disease affecting the connective tissue that makes up lung tissue is called interstitial lung disease. This problem occurs in 5-30% of patients with idiopathic inflammatory muscle disease (associated with anti-synthetase antibodies, especially anti-Jo-1).

Patients may present with shortness of breath, cough, and fever.

Treatment also may have deleterious effects. Patients receiving immunosuppressive drugs have an increased risk of infections.

Lung involvement may be severe enough to produce acute respiratory failure. While diseases such as polymyositis and dermatomyositis have been associated with a concomitant malignancy, the incidence of coexistent carcinoma is probably not as great as has been claimed. Only 16% in one series and 6% in another series had carcinoma. The institution of an exhaustive search for malignancy is debatable.

For more in-depth scientific information, here are some sources...

Polymyositis and diffuse interstitial lung disease. A review of the pulmonary histopathologic findings

G. Salmeron, S. D. Greenberg and M. D. Lidsky

A retrospective analysis was performed of 105 patients with polymyositis for eight years. Roentgenographic evidence of pulmonary interstitial disease was present in ten adult patients (9%) with polymyositis unassociated with other connective-tissue disorders. Review of the pulmonary histopathologic findings indicated a spectrum of pulmonary diffuse interstitial infiltrates and fibroplasia of the alveolar septae. Response to glucocorticoids with regard to pulmonary symptoms was variable in the patients studied. Therapeutic response seemed to be influenced by both the cellularity of the chronic interstitial infiltrates and the degree of fibroplasia of the alveolar septae. Electron microscopic studies of the lung tissue from two patients with polymyositis and diffuse interstitial lung disease failed to demonstrate either immune complexes or viral particles.

Interstitial lung diseases associated with collagen vascular diseases: radiologic and histopathologic findings.

Kim EA, Lee KS, Johkoh T, Kim TS, Suh GY, Kwon OJ, Han J.

Department of Radiology, Samsung Medical Center, Sungkyunkwan University School of Medicine, 50 Ilwon-Dong, Kangnam-Ku, Seoul 135-710, Korea.

Collagen vascular diseases that demonstrate features of interstitial lung disease include systemic lupus erythematosus, rheumatoid arthritis, progressive systemic sclerosis, dermatomyositis and polymyositis, ankylosing spondylitis, SjÃgren syndrome, and mixed connective tissue disease. At histopathologic analysis, interstitial lung diseases associated with collagen vascular diseases are diverse and include nonspecific interstitial pneumonia, usual interstitial pneumonia, bronchiolitis obliterans organizing pneumonia (BOOP), apical fibrosis, diffuse alveolar damage, and lymphocytic interstitial pneumonia. Although proportions of interstitial pneumonias vary, nonspecific interstitial pneumonia accounts for a large proportion, especially in progressive systemic sclerosis, dermatomyositis and polymyositis, and mixed connective tissue disease. The more favorable prognosis in interstitial pneumonia associated with collagen vascular diseases than in idiopathic interstitial pneumonias may be explained by the larger proportion of nonspecific interstitial pneumonia than of usual interstitial pneumonia. High-resolution computed tomography seems to help characterize and determine the extent of interstitial lung disease in collagen vascular diseases.

Importance of interstitial lung disease in collagen vascular disease: analysis of outcome]

[Article in Japanese]

Takizawa H, Suzuki N, Yanagawa T, Okazaki H, Satoh M, Akiyama N, Kohyama T, Ito K, Oka T.

Department of Internal Medicine and Physical Therapy, Faculty of Medicine, University of Tokyo, Japan.

We studied length of survival and related clinical findings in 715 inpatients with collagen-vascular diseases (1984 through 1994), the diagnostic Kaplan-Meier analysis showed that patients with polymyositis/dermatomyositis and those with systemic sclerosis did not survive as long as those with other types of collagen-vascular disease. Of the patients who died 37% died of respiratory failure due to interstitial lung disease. Patients with interstitial lung disease had better outcomes than did those with idiopathic interstitial pneumonia: they were younger, had higher initial vital capacities, and fewer episodes of acute exacerbation of lung disease than did those with idiopathic interstitial pneumonia. Among patients with interstitial lung disease, those who died of polymyositis/dermatomyositis did so within 1 year, but those who died of systemic sclerosis lived longer. Interstitial lung disease is an important prognostic factor in collagen-vascular disease, and needs further evaluation.

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