Seminars in respiratory and critical care medicine
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Semin Respir Crit Care Med · Apr 2014
ReviewHigh-resolution computed tomography of the pulmonary manifestations of connective tissue diseases.
Pulmonary disease is common in patients with connective tissue disease (CTD) and confers significant morbidity and mortality. High-resolution computed tomography (HRCT) provides considerable information regarding the distribution and severity of thoracic disease in CTD. ⋯ Finally, the risk of developing cancer is increased in patients with certain CTDs. In this review, the HRCT manifestations of CTD are initially discussed with respect to the different compartments involved, followed by a description of the spectrum of pulmonary disease commonly occurring in specific CTDs.
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Sjögren syndrome (SS) is a chronic autoimmune disease characterized by lymphocytic inflammation of exocrine glands and a variety of extraglandular sites. Lung involvement as defined by symptoms and either pulmonary function testing or radiographic abnormalities occurs in approximately 10 to 20% of patients. Subclinical lung disease is even more frequent and often includes evidence of small airways disease and airway inflammation. ⋯ Bronchiolitis and bronchiectasis are the most common airway manifestations while the interstitial pathologies associated with SS include nonspecific interstitial pneumonitis, usual interstitial pneumonitis, and lymphocytic interstitial pneumonitis. Patients with SS are also at an increased risk of lymphoma. A protean of other lung abnormalities including amyloidosis, granulomatous lung disease, pseudolymphoma, pulmonary hypertension, and pleural disease have been described.
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Semin Respir Crit Care Med · Apr 2014
Cross-disciplinary collaboration in connective tissue disease-related lung disease.
Lung disease is a common manifestation of connective tissue disease (CTD) and is associated with significant morbidity and mortality. The evaluation of lung disease, and interstitial lung disease (ILD) in particular, in patients with CTD is complex because of the heterogeneity of the CTDs, the varied types and degrees of severity of ILD encountered, and because ILD can be identified at any point in time in these patients. ⋯ The detection of occult CTD in patients with "idiopathic" ILD requires careful attention to the demographic profile, historical clues, subtle physical examination findings, specific autoantibody positivity, radiological and histopathological features, and can be optimized by a multidisciplinary approach that includes rheumatological collaboration. Not all patients with CTD-associated ILD require pharmacological therapy, and management decisions should consider pace and severity of the disease, intra- and extrathoracic features of activity and can be optimized by cross-disciplinary collaboration.
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Semin Respir Crit Care Med · Apr 2014
ReviewSupportive care for patients with pulmonary complications of connective tissue disease.
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. ⋯ 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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Semin Respir Crit Care Med · Apr 2014
ReviewImmunosuppression for connective tissue disease-related pulmonary disease.
With improvements in the systemic treatment of the various connective tissue diseases (CTDs) the pulmonary complications of these conditions are now, for many patients, the major cause of morbidity and impaired quality of life. Furthermore, at least in scleroderma, pulmonary disease has become the leading cause of death for this patient group. Although, the pathogenesis of CTD-related pulmonary disease is poorly understood there is an assumption that it arises as a sequelae of immune-mediated injury to the lung. ⋯ Therefore, treatment decisions are informed by registry data, case series, and individual case reports. With this in mind, corticosteroids together with azathioprine and mycophenolate mofetil are widely used for the management of mild disease or as a maintenance therapy, while cyclophosphamide and rituximab have emerged as treatments for refractory or rapidly progressive disease. This article examines the data underpinning the use of different immunosuppressants in CTD-associated pulmonary disease while highlighting limitations in the existing knowledge base and identifying questions for future clinical study.