The most common causes of disability and death from sarcoidosis are directly or indirectly
related to complications of fibrotic lung disease [
[1]
]. Fibrosis develops in up to 20% of pulmonary sarcoidosis patients [
[2]
,
[3]
] and may result in end-stage parenchymal lung disease [
[4]
]. Fibrotic pulmonary sarcoidosis is associated with distortion of airways leading
to airflow obstruction, bronchiectasis, and increased episodes of clinical worsening
that are often related to pulmonary infections [
4
,
5
,
6
,
7
]. Sarcoidosis associated pulmonary hypertension is caused by a variety of mechanisms,
but the most common of these is distortion of the pulmonary vasculature from fibrosis
[
[8]
,
[9]
]. Mycetoma that are associated with pulmonary sarcoidosis occur almost exclusively
in those with fibrocystic disease [
[10]
] where they may cause life-threatening hemoptysis [
[11]
]. All the aforementioned manifestations of fibrotic pulmonary sarcoidosis commonly
result in severe quality of life impairment and are potentially life-threatening [
10
,
11
,
12
,
13
].Keywords
To read this article in full you will need to make a payment
Purchase one-time access:
Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online accessOne-time access price info
- For academic or personal research use, select 'Academic and Personal'
- For corporate R&D use, select 'Corporate R&D Professionals'
Subscribe:
Subscribe to Respiratory MedicineAlready a print subscriber? Claim online access
Already an online subscriber? Sign in
Register: Create an account
Institutional Access: Sign in to ScienceDirect
References
- Strategies for identifying pulmonary sarcoidosis patients at risk for severe or chronic disease.Expet Rev. Respir. Med. 2017; 11: 111-118
- Pulmonary fibrosis of sarcoidosis. New approaches, old ideas.Am. J. Respir. Cell Mol. Biol. 2003; 29: S37-S41
- The clinical course of sarcoidosis: presentation, diagnosis, and treatment in a large white and black cohort in the United States.Sarcoidosis Vasc. Diffuse Lung Dis. 2012; 29: 119-127
- Frequency of acute worsening events in fibrotic pulmonary sarcoidosis patients.Respir. Med. 2013; 107: 2009-2013
- CT findings in severe thoracic sarcoidosis.Eur. Radiol. 2005; 15: 23-30
- Clinical bronchiectasis complicating pulmonary sarcoidosis: case series of seven patients.Sarcoidosis Vasc. Diffuse Lung Dis. 2002; 19: 154-159
- Airway involvement in sarcoidosis.Chest. 2009; 136: 1371-1380
- Incidence of pulmonary hypertension and its clinical relevance in patients with sarcoidosis.Chest. 2006; 129: 1246-1252
- Clinical features of sarcoidosis associated pulmonary hypertension: results of a multi-national registry.Respir. Med. 2018; 139: 72-78
- Chronic pulmonary aspergillosis complicating sarcoidosis.Eur. Respir. J. 2017; 49
- A modern series of percutaneous intracavitary instillation of amphotericin B for the treatment of severe hemoptysis from pulmonary aspergilloma.Chest. 2013; 143: 1414-1421
- Predictors of mortality in pulmonary sarcoidosis.Chest. 2018; 153: 105-113
- An integrated clinicoradiological staging system for pulmonary sarcoidosis: a case-cohort study.Lancet Respir Med. 2014; 2: 123-130
- End-stage sarcoid lung disease is distinct from usual interstitial pneumonia.Am. J. Surg. Pathol. 2013; 37: 593-600
- Histopathology of explanted lungs from patients with a diagnosis of pulmonary sarcoidosis.Chest. 2016; 149: 499-507
- Pulmonary sarcoidosis: typical and atypical manifestations at high-resolution CT with pathologic correlation.Radiographics. 2010; 30: 1567-1586
- Severity of pulmonary involvement and (18)F-FDG PET activity in sarcoidosis.Respir. Med. 2013; 107: 439-447
- Chronic interstitial pneumonitis in end-stage sarcoidosis.Eur. Respir. J. 2010; 35: 695-697
- Sarcoidosis and idiopathic pulmonary fibrosis: the same tale or a tale of two diseases in one.Respir. Med. 2019; 160: 105668
- A phase 3 trial of pirfenidone in patients with idiopathic pulmonary fibrosis.N. Engl. J. Med. 2014; 370: 2083-2092
- Efficacy and safety of nintedanib in idiopathic pulmonary fibrosis.N. Engl. J. Med. 2014; 370: 2071-2082
- Nintedanib in progressive fibrosing interstitial lung diseases.N. Engl. J. Med. 2019; 381: 1718-1727
- Multidisciplinary approach and long-term follow-up in a series of 640 consecutive patients with sarcoidosis: cohort study of a 40-year clinical experience at a tertiary referral center in Barcelona, Spain.Medicine (Baltim.). 2017; 96e7595
- Prediction of relapse after discontinuation of infliximab therapy in severe sarcoidosis.Eur. Respir. J. 2014; 43: 602-609
- Evaluation of CD103 as a cellular marker for the diagnosis of pulmonary sarcoidosis.Clin. Immunol. 2008; 126: 338-344
- Blau syndrome of granulomatous arthritis, iritis, and skin rash: a new family and review of the literature.Am. J. Med. Genet. 1998; 76: 217-221
- Transforming growth factor-beta gene polymorphisms in sarcoidosis patients with and without fibrosis.Chest. 2006; 129: 1584-1591
Article info
Publication history
Published online: March 22, 2021
Accepted:
March 12,
2021
Received in revised form:
March 11,
2021
Received:
November 23,
2020
Identification
Copyright
© 2021 Elsevier Ltd. All rights reserved.