Infections in Systemic Connective Tissue Diseases: Systemic Lupus Erythematosus, Scleroderma, and Polymyositis/Dermatomyositis

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Infections in systemic lupus erythematosus

Infections are responsible for 30% to 50% of the morbidity and mortality in children and adults who have SLE [2], [3], [5], [11], [12], [13], [17], [18], [19], [20], [21], [22], [23], [24]. These infectious processes usually result from common microorganisms [2], [11], [14], [16], [17], [20], [25], [26], [27], [28], but opportunistic infections also may occur and are important causes of death in patients who receive corticosteroid and immunosuppressive therapy [9], [18], [19], [20], [25], [29],

Predisposing factors

Risk factors associated with infections in patients who have scleroderma include esophageal and pulmonary involvement, severe Raynaud's phenomenon or calcinosis, and the use of specific treatments for the management of the disease. Scleroderma patients who have smooth muscle involvement of the esophagus are at increased risk for aspiration pneumonia, because these patients usually have lower esophageal sphincter dysfunction and severe gastroesophageal reflux [14], [118], [119], [120]. To avoid

Predisposing factors

Patients PM/DM have a host of predisposing factors placing them at risk for developing infections. These factors include upper esophageal involvement, thoracic muscle myopathy, calcinosis cutis, and the use of immunosuppressive drugs.

Some patients who have PM/DM have involvement of the striated muscle of the hypopharynx and the upper third of the esophagus resulting in altered swallowing, gastroesophageal reflux, and aspiration, and a greater risk for developing aspiration pneumonia [7], [8],

Summary

In SLE, scleroderma, and PM/DM, infections are important causes of morbidity and mortality. This increased risk for developing infections is the result of immune abnormalities and of organ system manifestations associated with these diseases and their treatments. Common bacteria are responsible for most mild and lethal infections; however, opportunistic microorganisms cause death in some patients, particularly in those receiving high doses of corticosteroid and immunosuppressive therapy.

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      2020, Handbook of Systemic Autoimmune Diseases
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      Sjogren syndrome may cause both a reduced salivary production (with loss of its antibacterial effect and dysphagia) and a defect in the mucociliary clearance of the lower airways secondary to the involvement by the disease of the exocrine glands of the tracheobronchial mucosa, increasing the incidence of bacterial pneumonia [8,14]. Finally, secondary pulmonary fibrosis and/or bronchiectasis (which are potential complications of many systemic autoimmune diseases, but particularly of SS, RA, and DM/PM) can predispose to bacterial pneumonias [7,8,23]. Many immunomodulatory/immunosuppressive drugs used for the treatment of systemic autoimmune diseases may increase the risk of bacterial infections of the lungs (see Table 1.1).

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    Portions of this article originally appeared in Juárez M, Misischia R, Alarcón GS. Infections in systemic connective tissue diseases: systemic lupus erythematosus, scleroderma, and polymyositis/dermatomyositis. Rheumatic Disease Clinics of North America 2003;29(1):163–84.

    Supported by MCRC grant M01-RR00032 from the National Institute of Arthritis and Musculoskeletal and Skin Disorders.

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