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Semin Respir Crit Care Med. 2014 Apr;35(2):274-82. doi: 10.1055/s-0034-1371538. Epub 2014 Mar 25.

Supportive care for patients with pulmonary complications of connective tissue disease.

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Section of Pulmonary, Critical Care and Sleep Medicine, Department of Internal Medicine, Yale University School of Medicine, New Haven, Connecticut.


Patients with connective tissue disease often suffer from pulmonary complications, including interstitial lung disease and pulmonary hypertension. Supportive care for these patients aims to relieve symptoms and improve activity level and quality of life. A holistic approach to the management of patients with advanced connective tissue disease-associated pulmonary disorders includes a full assessment of patient symptoms as well as a careful search for side effects of treatment and treatable comorbidities. This article addresses supportive measures such as supplemental oxygen and pulmonary rehabilitation. Issues related to quality of life, sleep disturbances, and identification of mood disorders are discussed. In addition, we review significant comorbidities, including cardiovascular disease, glucocorticoid-induced osteoporosis, and gastroesophageal reflux disease. Essential facets of advanced lung disease, including mechanical ventilation, lung transplantation, end-of-life care, and hospice, are covered.

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