Seminars in respiratory and critical care medicine
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Semin Respir Crit Care Med · Apr 2019
ReviewPulmonary Complications of Systemic Lupus Erythematosus.
Systemic lupus erythematosus (SLE) is an autoimmune rheumatic disease characterized by the production of pathogenic autoantibodies and immune complexes and is responsible for significant morbidity and mortality through a wide range of clinical manifestations which can affect almost any organ. Pulmonary involvement is prevalent and seen in 50 to 70% of SLE patients and may even be the presenting feature in 4 to 5% of patients. By 10 years postdiagnosis, 12% will have accumulated an element of permanent lung damage. ⋯ ILD and pulmonary hypertension are both frequently seen in other autoimmune rheumatic diseases such as systemic sclerosis; however, in SLE, they tend to be milder and have a comparatively favorable prognosis. Although collectively pulmonary involvement in SLE is common, the heterogeneity of SLE and rareness of individual complications make clinical trials difficult and treatment is usually based on case series reports and anecdotal experience with various immunosuppressive agents. Some of these immunosuppressive agents such as azathioprine, methotrexate, and cyclophosphamide have also been linked with drug-induced lung injury.
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Semin Respir Crit Care Med · Apr 2019
ReviewPulmonary and Bronchiolar Involvement in Sjogren's Syndrome.
Sjogren's syndrome (SS) is a chronic autoimmune disease characterized by mononuclear cells (principally lymphocytes) infiltrating exocrine glands (e.g., salivary and lacrimal glands), leading to destruction of exocrine epithelial cells and dryness of mucosal surfaces. Cardinal symptoms are dry eyes (xerophthalmia) and dry mouth (xerostomia). Extraglandular sites are affected in 30 to 40% of cases of SS (particularly neurological, kidneys, skin, and lungs). ⋯ Primary SS is not associated with other autoimmune diseases. However, SS can complicate diverse autoimmune disorders (particularly systemic lupus erythematosus, rheumatoid arthritis, and scleroderma); this form is termed "secondary SS." Pulmonary involvement is usually not a dominant feature of SS, but may be severe in some cases. In this review, we discuss specific tracheal, bronchiolar, and pulmonary complications of SS including xerotrachea, bronchiolitis, bronchiectasis, interstitial lung disease, nonspecific interstitial pneumonia, usual interstitial pneumonia, lymphoid interstitial pneumonia, organizing pneumonia, acute fibrinous and organizing pneumonia, pulmonary cysts, pleural effusions, pulmonary amyloidosis, and bronchus- or lung-associated lymphomas.
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Semin Respir Crit Care Med · Apr 2019
ReviewSystemic Sclerosis Associated Interstitial Lung Disease: A Comprehensive Overview.
Interstitial lung disease (ILD) is a common complication of systemic sclerosis (SSc). SSc-ILD adversely impacts quality of life and is currently the leading cause of death in this multisystem disease. Identifying clinically significant SSc-ILD is critically important. ⋯ For those with refractory or very advanced disease, studies into disease-specific palliative approaches are in their infancy. Lung transplantation as an option for SSc-ILD remains patient- and center-specific, with data to suggest equivalent outcomes to other fibrotic lung diseases, in carefully selected cases. This review aims to provide a comprehensive overview of all key aspects of SSc-ILD.
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Semin Respir Crit Care Med · Apr 2019
ReviewMultidisciplinary Evaluation in Patients with Lung Disease Associated with Connective Tissue Disease.
Multidisciplinary diagnosis is now viewed as the diagnostic reference standard in interstitial lung disease (ILD). This process consists of the integration of the evidence base with clinical reasoning in the formulation of a diagnosis and requires input from clinicians, radiologists, and, in selected cases, histopathologists. In ILD associated with connective tissue disease (CTD-ILD), multidisciplinary evaluation is especially helpful when CTD is suspected but cannot be diagnosed using strict criteria. ⋯ MDD also provides a means to combine the views of respiratory physicians and rheumatologists in formulating a treatment strategy. It is often possible to reach a robust view as to whether management should be driven by systemic disease, pulmonary disease, or both. When treatment needs to be introduced or modified for both systemic and pulmonary reasons, face-to-face discussion facilities the selection of therapeutic agents that are likely to be efficacious for both systemic and pulmonary diseases.
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Semin Respir Crit Care Med · Apr 2019
ReviewInterstitial Pneumonia with Autoimmune Features and Undifferentiated Connective Tissue Disease.
In 2015, a multidisciplinary task force comprising pulmonologists, rheumatologists, pathologists, and radiologists representing the European Respiratory Society and American Thoracic Society published a diagnostic classification schema for individuals with interstitial lung disease and autoimmune features who did not meet criteria for a defined connective tissue disease. The term interstitial pneumonia with autoimmune features (IPAF) was applied. ⋯ The natural history and appropriate management of IPAF have not been clarified, as data are largely limited to retrospective studies. In this review, we discuss the salient clinical, serologic, histologic, and radiographic features of IPAF and discuss an approach to management.