Sarcoid-Associated Pulmonary Hypertension and Lung TransplantSarcoid-Associated Pulmonary Hypertension and Lung Transplant

What's the role for lung transplantation in patients with sarcoidosis? Seminars in Respiratory and Critical Care Medicine
Source: Medscape Today Headlines - Category: Consumer Health News Tags: Pulmonary Medicine Journal Article Source Type: news

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ConclusionsPatients with CS have similar post-transplant hemodynamics as patients without CS, without evidence of right ventricular dysfunction or pulmonary hypertension. Neither significant rejection nor recurrence of sarcoid in the allograft was observed in this cohort of patients with CS. Survival is similar between patients with CS and those without CS. Heart transplant is a viable strategy in selected patients with CS with excellent outcomes.RésuméIntroductionLa transplantation cardiaque orthotopique (TCO) est de plus en plus utilisée lors d’insuffisance cardiaque terminale liée &agra...
Source: Canadian Journal of Cardiology - Category: Cardiology Source Type: research
Clinicians in pulmonary medicine frequently confront the challenge of screening, diagnosis, and management of pulmonary hypertension (PH) in sarcoidosis patients who present with unexplained dyspnea. Sarcoidosis-associated pulmonary hypertension (SAPH) is most prevalent in patients with pulmonary fibrosis, although it can be independent of airflow obstruction or restriction. SAPH is independently associated with significantly increased mortality and decreased functional capacity, outcomes that can be mitigated by early detection and focused treatment. In this review, we discuss the pathophysiology of SAPH, which may resemb...
Source: Clinical Pulmonary Medicine - Category: Respiratory Medicine Tags: Interstitial, Inflammatory and Occupational Lung Disease Source Type: research
Studies reporting the effects of modern strategies with pulmonary arterial hypertension (PAH)-targeted therapies in sarcoidosis-associated pulmonary hypertension (S-APH) are limited. Clinical and haemodynamic data from newly diagnosed patients with severe S-APH (mean pulmonary artery pressure (mPAP)>35 mmHg or mPAP 25–35 mmHg with cardiac index
Source: European Respiratory Journal - Category: Respiratory Medicine Authors: Tags: Interstitial and orphan lung disease, Pulmonary vascular disease Original Articles: Pulmonary vascular diseases and sarcoidosis Source Type: research
Chronic pulmonary aspergillosis (CPA) complicating sarcoidosis (SA) is associated with high mortality, and there is a lack of clarity regarding the relative contributions of SA or CPA. This was a retrospective single-centre study on CPA-SA. In total, 65 patients (44 men), aged 41.4±13.5 and 48.3±11.9 years at the time of SA and CPA diagnoses, respectively, were included between 1980 and 2015. Of these, 64 had fibrocystic SA, most often advanced, with composite physiological index (CPI)>40 (65% of patients) and pulmonary hypertension (PH) (31%), and 41 patients (63%) were treated for SA (corticosteroid...
Source: European Respiratory Journal - Category: Respiratory Medicine Authors: Tags: Interstitial and orphan lung disease, Respiratory infections and tuberculosis Original Articles: Sarcoidosis and aspergillosis Source Type: research
Authors: Mirsaeidi M, Omar HR, Baughman R, Machado R, Sweiss N Abstract INTRODUCTION: Pulmonary hypertension (PH) is a feared complication in patients with sarcoidosis.It is an important negative prognostic factor which is reflected as a priority given to these subjects for orthotopic lung transplantation. We evaluated the relationship between BNP, 6MWD test, DLCO% values and the severity of sarcoidosis-associated pulmonary hypertension. METHODS: A retrospective chart review was conducted between January 2010 and January 2015 on consecutive confirmed adult subjects diagnosed with sarcoidosis. Demographics, medi...
Source: Sarcoidosis Vasculitis and Diffuse Lung Diseases - Category: Respiratory Medicine Tags: Sarcoidosis Vasc Diffuse Lung Dis Source Type: research
Fibrosing mediastinitis (FM) is caused by proliferation of fibrous tissue in the mediastinum with encasement of mediastinal viscera and compression of mediastinal structures. Pulmonary hypertension (PH) is caused by extrinsic compression of the pulmonary arteries and/or veins.We reviewed clinical, functional, hemodynamic, radiological characteristics and outcome of 27 consecutive cases of PH associated with FM diagnosed at the French Referral Centre for PH (2003-2014).Right heart catheterization (RHC) confirmed PH in 14 men and 13 women. The causes of FM were sarcoidosis (n=13), tuberculosis-infection (n=9), mediastinal ir...
Source: European Respiratory Journal - Category: Respiratory Medicine Authors: Tags: 4.3 Pulmonary Circulation and Pulmonary Vascular Diseases Source Type: research
We describe a case of a 35-year-old woman with idiopathic pulmonary arterial hypertension (IPAH) and a novel BMPR2 mutation, who underwent a successful lung transplantation.  Extensive granulomatous inflammation was seen in the resected lungs. The granulomatous inflammation found in the histology supports  a sarcoid-like reaction due to pulmonary hypertension in the context of the BMPR2 mutation. PMID: 27537724 [PubMed - as supplied by publisher]
Source: Sarcoidosis Vasculitis and Diffuse Lung Diseases - Category: Respiratory Medicine Tags: Sarcoidosis Vasc Diffuse Lung Dis Source Type: research
Patients with sarcoidosis undergo spontaneous remission or may be effectively controlled with glucocorticoids alone in many cases. Progressive and refractory pulmonary sarcoidoisis constitute>10% of the patients seen at specialized centers. Pulmonary fibrosis and associated complications, such as infections and pulmonary hypertension, are leading causes of mortality. No universal definition of refractoriness exists; we therefore propose classifying patients as having refractory disease when the following criteria are fulfilled: (1) progressive disease despite at least 10 mg of prednisolone or equivalent for at least 3 m...
Source: Clinical Pulmonary Medicine - Category: Respiratory Medicine Tags: Interstitial, Inflammatory, & Occupational Lung Disease Source Type: research
Abstract: Fibrosing mediastinitis is caused by a proliferation of fibrous tissue in the mediastinum with encasement of mediastinal viscera and compression of mediastinal bronchovascular structures. Pulmonary hypertension (PH) is a severe complication of fibrosing mediastinitis caused by extrinsic compression of the pulmonary arteries and/or veins. We have conducted a retrospective observational study reviewing clinical, functional, hemodynamic, radiological characteristics, and outcome of 27 consecutive cases of PH associated with fibrosing mediastinitis diagnosed between 2003 and 2014 at the French Referral Centre for PH....
Source: Medicine - Category: Internal Medicine Tags: Research Article: Observational Study Source Type: research
Background: Pulmonary hypertension (PH) is a frequent complication of sarcoidosis. Its mechanisms are multiple and unclear. Few studies reported the effects of pulmonary arterial hypertension-targeted therapies (PAH-TT) in sarcoidosis-associated PH (S-APH).Methods: Clinical and hemodynamic data from newly diagnosed patients with S-APH were collected from the French prospective PH registry (2004-2014).Results: All 121 consecutive patients with S-APH were analyzed (57±11 years; 67 females). Mean FVC was 64%±20 and 78% had radiologic stage IV at time of PH diagnosis. Sixty two percent of patients had out of prop...
Source: European Respiratory Journal - Category: Respiratory Medicine Authors: Tags: 4.3 Pulmonary Circulation and Pulmonary Vascular Disease Source Type: research
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