Journal
RESPIRATION
Volume 91, Issue 3, Pages 181-201Publisher
KARGER
DOI: 10.1159/000444535
Keywords
Acute 'exacerbations' in fibrotic interstitial pneumonias; Diffuse alveolar hemorrhage syndromes; Acute pulmonary thromboembolism; Catastrophic antiphospholipid syndrome; Organizing pneumonia; Acute fibrinous organizing pneumonia; Acute hypercapnic respiratory failure; Myositis; ventilatory muscles; Pneumothorax; Pneumomediastinum; Aspiration pneumonitis; Pericarditis; Pleuropericarditis; Drug toxicity; Lung infections; Rheumatoid arthritis; Systemic lupus erythematosus; Systemic sclerosis; Dermatomyositis-polymyositis; Primary Sjogren's syndrome; Mixed connective tissue disease; Ankylosing spondylitis
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Subacute-acute, hyperacute, or even catastrophic and fulminant respiratory events occur in almost all classic connective tissue disorders (CTDs); they may share systemic life-threatening manifestations, may precipitously lead to respiratory failure requiring ventilatory support as well as a combination of specific therapeutic measures, and in most affected patients constitute the devastating end-of-life event. In CTDs, acute respiratory events may be related to any respiratory compartment including the airways, lung parenchyma, alveolar capillaries, lung vessels, pleura, and ventilatory muscles. Acute respiratory events may also precipitate disease-specific extrapulmonary organ involvement such as aspiration pneumonia and lead to digestive tract involvement and heart-related respiratory events. Finally, antirheumatic drug-related acute respiratory toxicity as well as lung infections related to the rheumatic disease and/or to immunosuppression complete the spectrum of acute respiratory events. Overall, in CTDs the lungs significantly contribute to morbidity and mortality, since they constitute a common site of disease involvement; a major site of infections related to the 'mater' disease; a major site of drug-related toxicity, and a common site of treatment-related infectious complications. The extreme spectrum of the abovementioned events, as well as the 'vicious' coexistence of most of the aforementioned manifestations, requires skills, specific diagnostic and therapeutic means, and most of all a multidisciplinary approach of adequately prepared and expert scientists. Avoiding lung disease might represent a major concern for future advancements in the treatment of autoimmune disorders. (C) 2016 S. Karger AG, Basel
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