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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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.
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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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A significant proportion of patients with autoimmune connective tissue disease (CTD) show lung involvement that results in clinical interstitial lung disease (ILD). Surgical lung biopsy is helpful for diagnosis of CTD-ILD in many cases. In this review, we discuss the histologic manifestations of different types of CTD-ILD, focusing on patterns of disease and their differential diagnoses. Acquired autoimmune connective tissue diseases will be covered in this review, while lung involvement in vasculitides, heritable connective tissue disorders, and drug-induced CTD-like conditions will not be discussed.
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Semin Respir Crit Care Med · Apr 2014
ReviewRheumatoid arthritis and lung disease: from mechanisms to a practical approach.
Rheumatoid arthritis (RA) is a common chronic systemic autoimmune disease characterized by joint inflammation and, in a proportion of patients, extra-articular manifestations (EAM). Lung disease, either as an EAM of the disease, related to the drug therapy for RA, or related to comorbid conditions, is the second commonest cause of mortality. All areas of the lung including the pleura, airways, parenchyma, and vasculature may be involved, with interstitial and pleural disease and infection being the most common problems. ⋯ Citrullination of proteins in the lung, frequently thought to be incited by smoking, and the subsequent development of ACPA appear to play an important role in the development of lung and possibly joint disease. The biologic and nonbiological disease modifying antirheumatic drugs (DMARDs) have had a substantial impact on morbidity and mortality from RA, and although there multiple reports of drug-related lung toxicity and possible exacerbation of underlying ILD, overall these reactions are rare and should only preclude the use of DMARDs in a minority of patients. Common scenarios facing pulmonologists and rheumatologists are addressed using the current best evidence; these include screening the new patient; monitoring and choosing RA treatment in the presence of subclinical disease; treating deteriorating ILD; and establishing a diagnosis in a patient with an acute respiratory presentation.