Rheumatic diseases clinics of North America
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Rheum. Dis. Clin. North Am. · May 2015
ReviewManagement of connective tissue disease-associated interstitial lung disease.
A thorough, often multidisciplinary assessment to determine extrathoracic versus intrathoracic disease activity and degrees of impairment is needed to optimize the management of connective tissue disease (CTD)-associated interstitial lung disease (ILD). Pharmacologic intervention with immunosuppression is the mainstay of therapy for all forms of CTD-ILD, but should be reserved for those that show clinically significant and/or progressive disease. The management of CTD-ILD is not yet evidence based and there is a need for controlled trials across the spectrum of CTD-ILD. Nonpharmacologic management strategies and addressing comorbidities or aggravating factors should be included in the comprehensive treatment plan for CTD-ILD.
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Pulmonary complications cause significant morbidity and mortality in the idiopathic inflammatory myopathies. Advances in biomarker discovery have facilitated clinical phenotyping, allowing investigators to better define at-risk patient subsets and to potentially gauge disease activity. ⋯ Pharmacologic management continues to rely on the use of corticosteroids, often in combination with additional immunosuppressive agents. The rarity of myositis-associated interstitial lung disease and lack of controlled trials have limited analyses of treatment efficacy, mandating the development of standardized outcome measures and improvement of data sharing between disciplines.
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Rheum. Dis. Clin. North Am. · May 2015
ReviewDetermining respiratory impairment in connective tissue disease-associated interstitial lung disease.
Connective tissue diseases (CTDs) can affect the lungs through diseases of the chest wall, pleura, vasculature, airways, and parenchyma. Interstitial lung disease (ILD) is a common complication of CTD associated with increased morbidity and mortality. ⋯ Patients with CTD with suspected ILD should undergo clinical, physiologic, and radiologic studies to evaluate for the presence of ILD, and these results should be integrated in a multidisciplinary setting to guide diagnosis and management. Screening for ILD may also be appropriate in asymptomatic patients with high-risk features.
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Rheum. Dis. Clin. North Am. · May 2015
ReviewImaging of pulmonary involvement in rheumatic disease.
Lung disease commonly occurs in connective tissue diseases (CTD) and is an important cause of morbidity and mortality. Imaging is central to the evaluation of CTD-associated pulmonary complications. ⋯ The contribution of imaging to monitoring disease, evaluating treatment response, and prognostication is reviewed. Finally, we address the role of imaging in the challenging multidisciplinary evaluation of interstitial lung disease where there is an underlying suspicion of an undiagnosed CTD.
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Pulmonary vasculitis encompasses inflammation in the pulmonary vasculature with involved vessels varying in caliber from large elastic arteries to capillaries. Small pulmonary capillaries are the vessels most commonly involved in vasculitis affecting the lung. The antineutrophil cytoplasmic antibody-associated vasculitides, which include granulomatosis with polyangiitis (formerly Wegener granulomatosis), microscopic polyangiitis, and eosinophilic granulomatosis with polyangiitis (formerly Churg-Strauss syndrome), are the small vessel vasculitides in which pulmonary vasculitis is most frequently observed and are the major focus of this review. Vasculitic involvement of the large pulmonary vessels as may occur in Behçet syndrome and Takayasu arteritis is also discussed.