BTS Clinical Statement on pulmonary sarcoidosis

医学 结节病 肺功能测试 肺动脉高压 内科学 心脏病学
作者
Muhunthan Thillai,Christopher Atkins,Anjali Crawshaw,Simon P. Hart,Ling‐Pei Ho,Vasileios Kouranos,Karen Patterson,Nicholas Screaton,Joanna Whight,Athol U. Wells
出处
期刊:Thorax [BMJ]
卷期号:76 (1): 4-20 被引量:144
标识
DOI:10.1136/thoraxjnl-2019-214348
摘要

often occurs along lymphatic tracks: thus, peribronchial tissue and interlobular septa are the most common sites of sarcoid lesions.Pleural disease is uncommon 5 but should be considered in the setting of a lymphocytic effusion with other pulmonary features of the disease.Rarely, sarcoidosis affects the upper airways, including the trachea and larynx. 6Severe lower airway inflammation can result in fibrotic stenosis and distortion. 6Pneumothoraces are a recognised complication of fibrocystic sarcoidosis. 7Box 1 Clinical Practice Points -summary Clinical presentation1.The respiratory examination in pulmonary sarcoidosis is often normal, and is an unreliable measure of disease extent or morbidity.2. Pulmonary function tests are often normal in non-fibrotic sarcoidosis, and may not reflect disease activity or symptom burden.3. Screening for extra-thoracic disease is important.At baseline, patients should have a full blood count, biochemical tests (including urea and electrolytes, liver function tests and calcium), serum ACE levels (non-unanimous consensus) and a 12-lead ECG. 4. In patients with eye symptoms, a baseline ophthalmic review should be undertaken by either an optician or an ophthalmologist (depending on the severity of symptoms).5. Patients should be asked routinely about fatigue and mood disturbance.6.A comprehensive exposure and occupational history should be taken to exclude both berylliosis and silicosis which can present in a similar manner to sarcoidosis. Cardiac sarcoidosis and pulmonary hypertension1. Cardiac sarcoidosis and/or pulmonary hypertension should be considered in all patients with pulmonary sarcoidosis who have levels of breathlessness which are disproportionate to their lung function impairment.2. Baseline testing in all patients with suspected cardiac sarcoidosis (ie, those with ECG abnormalities, cardiac symptoms or breathlessness out of context with their pulmonary function) should include an ECG and an echocardiogram.Abnormalities in ECG or echocardiogram which suggest cardiac sarcoidosis should be confirmed with cardiac magnetic resonance imaging (CMR) or positron emission tomography (PET).3.All patients with palpitations should be offered a 24-hour Holter monitor.4. In patients with pulmonary sarcoidosis, the presence of cardiac involvement, based on advanced imaging (CMR or PET) findings, should be confirmed by a multidisciplinary team with experience in both sarcoidosis and other forms of cardiac disease.There is a current initiative to identify tertiary interstitial lung disease centres that have immediate access to specialist cardiac sarcoidosis expertise.
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