| Pulmonary-renal syndromes: An update for respiratory physicians. | |
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MedLine Citation:
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PMID: 21684732 Owner: NLM Status: Publisher |
Abstract/OtherAbstract:
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Pulmonary-renal syndromes are a group of disorders characterised by necrotising glomerulonephritis and pulmonary haemorrhage. Small vessel systemic vasculitis is the most common cause of pulmonary-renal syndromes presenting to respiratory physicians. Rarer causes include systemic lupus erythematosus and connective tissue diseases though severe pneumonia or cardiac failure may mimic their presentation. Some forms of small vessel vasculitides have a predilection for the pulmonary and renal vascular beds and if left untreated can result in fulminant organ failure. Whilst the aetiology of these syndromes remains unclear, much is known about the disease mechanisms including the pathogenic role of autoantibodies, immune-complex mediated inflammation and microangiopathic in-situ thrombosis. Despite established treatments achieving successful remission induction, patient tolerability and side effect profiles have limited their use which has led to searches for more targeted treatments. Consequently newer biological therapies have gained wider acceptance despite little being known about their long term safety and efficacy. The European Vasculitis Study Group (EUVAS) have recently formulated guidelines to provide consensus on diagnosis and management in this area and work to define survival rates in these conditions with longer term follow-up studies is ongoing. This review summarises the current aetiopathogenesis thought to underlie these complex diseases, the diagnostic definitions and classification criteria currently in use and the evidence base for modern therapies. Though unusual for respiratory specialists to coordinate overall management of these patients, an update on their current management is regarded as important to their practice given the recently changing trends in treatments. |
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Authors:
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Colm McCabe; Quentin Jones; Aikaterini Nikolopoulou; Chris Wathen; Raashid Luqmani |
Publication Detail:
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Type: JOURNAL ARTICLE Date: 2011-6-17 |
Journal Detail:
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Title: Respiratory medicine Volume: - ISSN: 1532-3064 ISO Abbreviation: - Publication Date: 2011 Jun |
Date Detail:
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Created Date: 2011-6-20 Completed Date: - Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 8908438 Medline TA: Respir Med Country: - |
Other Details:
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Languages: ENG Pagination: - Citation Subset: - |
Copyright Information:
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Crown Copyright © 2011. Published by Elsevier Ltd. All rights reserved. |
Affiliation:
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Oxford Centre for Respiratory Medicine, Churchill Hospital, Oxford, OX 3 7LJ, United Kingdom. |
Export Citation:
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From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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