Predictors of Mortality in Pulmonary Sarcoidosis

Kırkıl, Gamze; Lower, Elyse E.; Baughman, Robert P.

doi:10.1016/j.chest.2017.07.008

Cited by 159 publications

(125 citation statements)

References 26 publications

Supporting

Mentioning

117

Contrasting

Unclassified

Order By: Relevance

“…In this report, the 3-and 5-year survival rates were 74% and 55% respectively, similar to previously published papers [10,18]. In a recent report, a Cox proportional hazards regression model found the presence of pulmonary hypertension to be an independent predictor of mortality from sarcoidosis [23]. However, it remains unclear whether or not S-APH is an adaptive phenomenon and a surrogate for other deleterious aspects of the disease.…”

supporting

confidence: 76%

Treatment of sarcoidosis-associated pulmonary hypertension: so close, and yet so far

Baughman

Shlobin

2017

Eur Respir J

Self Cite

View full text Add to dashboard Cite

supporting

confidence: 76%

Treatment of sarcoidosis-associated pulmonary hypertension: so close, and yet so far

Baughman

Shlobin

2017

Eur Respir J

Self Cite

View full text Add to dashboard Cite

“…Previous studies have reported a wide range of figures for the prevalence of progressive disease in patients with ILDs, reflecting the different cohorts studied and the variety of criteria used to define progression. However, it is clear that a sizeable minority of patients with CTD-ILDs, chronic HP, iNSIP, sarcoidosis-ILD and unclassifiable IIP develop a progressive fibrosing phenotype characterized by decline in lung function, worsening symptoms and premature death 4,5,7,25,27,28,[32][33][34][35] . The physicians who participated in this survey estimated that patients with non-IPF progressive fibrosing ILDs die approximately 4-5 years after initial diagnosis of ILD.…”

Section: Discussionmentioning

confidence: 99%

Progressive fibrosing interstitial lung diseases: current practice in diagnosis and management

Wijsenbeek

Kreuter

Olson

et al. 2019

Current Medical Research and Opinion

155

View full text Add to dashboard Cite

Objective: Some patients with interstitial lung diseases (ILDs) other than idiopathic pulmonary fibrosis (IPF) develop a progressive fibrosing phenotype. We investigated the diagnosis and management of non-IPF ILDs using data from a survey of physicians and from US insurance claims. Methods: Pulmonologists, rheumatologists and internists in France, Germany, Italy, Japan, Spain, UK and US who had managed 10 patients with non-IPF ILDs in the past year, including those with progressive fibrosing ILDs, completed an online survey. Data on US insurance and prescription claims were obtained from a repository that aggregates data on claims routed from providers or pharmacies to payers. Results: In May-June 2017, 243 pulmonologists, 203 rheumatologists and 40 internists completed an online survey. Respondents estimated that 18-32% of patients diagnosed with non-IPF ILDs develop progressive fibrosis and that time from symptom onset to death in these patients was 61-80 months. Drug treatment was given to 50-75% of patients with non-IPF progressive fibrosing ILDs. Reasons for patients not being treated included that physicians considered patients to have mild or slowly progressing disease, or did not believe that available treatments are effective or well tolerated. Corticosteroids were the preferred first-line treatment for all types of non-IPF ILD. There was considerable heterogeneity in preferences for second-and third-line treatments. US insurance claims data from 3823 patients indicated that, in 2016, 50-75% of patients with ILDs received drug treatment (mostly corticosteroids) for their ILD. Conclusions: Physicians estimate that 18-32% of patients diagnosed with non-IPF ILDs develop a progressive fibrosing phenotype and that these patients experience significant delays in the diagnosis of ILD and the detection of progressive fibrosis. Between 25% and 50% of patients with progressive fibrosing ILDs do not receive drug therapy. There is an unmet need for effective and well tolerated treatments for progressive fibrosing ILDs.

show abstract

“…5 Another study confirmed the Walsh model but found that pulmonary hypertension and the presence of more than 20% fibrosis on HRCT were independent predictors of mortality. 4…”

Section: Monitoring and Prognosismentioning

confidence: 99%

“…95 As noted earlier, pulmonary hypertension is an independent risk factor for mortality in sarcoidosis. 4 It has been shown that precapillary pulmonary hypertension has significantly higher mortality than pulmonary hypertension associated with left ventricular disease. 89 Therefore, sarcoidosis patients with advanced pulmonary disease should be evaluated for pulmonary hypertension.…”

Section: Sarcoidosis Associated Pulmonary Hypertensionmentioning

confidence: 99%

“…6 As previously noted, increased CPI and SAPH are factors predictive of increased mortality in advanced pulmonary sarcoidosis. 4,5 Diagnosis is made on the basis of imaging and is confirmed by direct evidence of Aspergillus or a positive galactomannan test on sputum and/or BAL samples, and a serological response to Aspergillus. 117,120 Precipitins against Aspergillus are positive in more than 90% of cases.…”

Section: Chronic Pulmonary Aspergillosismentioning

confidence: 99%

See 1 more Smart Citation

Advanced Pulmonary Sarcoidosis

Gupta

Baughman

2020

Semin Respir Crit Care Med

Self Cite

View full text Add to dashboard Cite

At least 5% of sarcoidosis patients die from their disease, usually from advanced pulmonary sarcoidosis. The three major problems encountered in advanced pulmonary sarcoidosis are pulmonary fibrosis, pulmonary hypertension, and respiratory infections. Pulmonary fibrosis is the result of chronic inflammation, but other factors including abnormal wound healing may be important. Sarcoidosis-associated pulmonary hypertension (SAPH) is multifactorial including parenchymal fibrosis, vascular granulomas, and hypoxia. Respiratory infections can be cause by structural changes in the lung and impaired immunity due to sarcoidosis or therapy. Anti-inflammatory therapy alone is not effective in most forms of advanced pulmonary sarcoidosis. New techniques, including high-resolution computer tomography and 18F-fluorodeoxyglucose positron emission tomography (PET) have proved helpful in identifying the cause of advanced disease and directing specific therapy.

show abstract

Predictors of Mortality in Pulmonary Sarcoidosis

Cited by 159 publications

References 26 publications

Treatment of sarcoidosis-associated pulmonary hypertension: so close, and yet so far

Treatment of sarcoidosis-associated pulmonary hypertension: so close, and yet so far

Progressive fibrosing interstitial lung diseases: current practice in diagnosis and management

Advanced Pulmonary Sarcoidosis

Contact Info

Product

Resources

About