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The collagen vascular diseases (Table 11–1) are a heterogeneous group of idiopathic, inflammatory, systemic diseases. Common to these diseases is an aberrant immunological response resulting in end-organ damage. Pulmonary involvement results in significantly increased morbidity and mortality independent of that associated with the underlying connective tissue disease. Therefore, physicians must be aware of the pulmonary manifestations of the connective tissue diseases. In doing so, appropriate therapy and surveillance may be instituted to limit or ameliorate progressive dysfunction.

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Table 11–1. Collagen Vascular Diseases.
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Pulmonary abnormalities occur in the airways, lung parenchyma, pulmonary vasculature, or pleura. Dysfunction may be either a direct effect of the underlying disease process or a secondary complication due to treatment toxicities and opportunistic infection. Although infectious complications are numerous, this chapter will focus solely on the noninfectious aspects of lung involvement of the connective tissue diseases. Specifically, the focus will be on the common pulmonary manifestations of rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), systemic sclerosis (SSc), and polymyositis/dermatomyositis (PM/DM). Pulmonary manifestations of less common diseases such as Sjögren’s syndrome and mixed connective tissue disease will be highlighted briefly. The pulmonary vasculitides are discussed in Chapter 20: Vasculitis & the Diffuse Alveolar Hemorrhage Syndromes.

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Essentials of Diagnosis

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  • • Elevated rheumatoid factor.
  • • Symmetric, small joint arthralgias with stiffness that may progress to joint deformities.
  • • Dyspnea with or without a productive cough.

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General Considerations

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Rheumatoid arthritis is a systemic inflammatory disease predominantly affecting the synovial membranes of the diarthrodial joints. The disease affects all ethnicities, with an increasing prevalence between the fourth and sixth decades. In North America, the annual incidence is estimated to be 0.3–1.5%. The prevalence in women is 2.5 times higher than in men. However, pulmonary manifestations (Table 11–2) are more common in men, with a 3:1 ratio, and in those who develop disease later in life.

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Table 11–2. Pulmonary Manifestations in Rheumatoid Arthritis.
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Pathogenesis

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Rheumatoid arthritis appears to be a disease in which there is an aberrant immunological ...

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