scholarly journals Overall mortality in combined pulmonary fibrosis and emphysema related to systemic sclerosis

RMD Open ◽  
2019 ◽  
Vol 5 (1) ◽  
pp. e000820 ◽  
Author(s):  
Alarico Ariani ◽  
Mario Silva ◽  
Elena Bravi ◽  
Simone Parisi ◽  
Marta Saracco ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Pulmonary Fibrosis ◽  
Pulmonary Function ◽  
Pulmonary Function Tests ◽  
Rank Test ◽  
Semiquantitative Assessment ◽  
Kaplan Meier ◽  
Impaired Lung Function ◽  
Anticentromere Antibodies ◽  
Overall Mortality

ObjectivesThis multicentre study aimed to investigate the overall mortality of combined pulmonary fibrosis and emphysema (CPFE) in systemic sclerosis (SSc) and to compare CPFE-SSc characteristics with those of other SSc subtypes (with interstitial lung disease—ILD, emphysema or neither).MethodsChest CTs, anamnestic data, immunological profile and pulmonary function tests of patients with SSc were retrospectively collected. Each chest CT underwent a semiquantitative assessment blindly performed by three radiologists. Patients were clustered in four groups: SSc-CPFE, SSc-ILD, SSc-emphysema and other-SSc (without ILD nor emphysema). The overall mortality of these groups was calculated by Kaplan-Meier method and compared with the stratified log-rank test; Kruskal-Wallis test, t-Student test and χ² test assessed the differences between groups. P<0.05 was considered statistically significant.ResultsWe enrolled 470 patients (1959 patient-year); 15.5 % (73/470) died during the follow-up. Compared with the SSc-ILD and other-SSc, in SSc-CPFE there was a higher prevalence of males, lower anticentromere antibodies prevalence and a more reduced pulmonary function (p<0.05). The Kaplan-Meier survival analysis demonstrates a significantly worse survival in patients with SSc-CPFE (HR vs SSc-ILD, vs SSc-emphysema and vs other-SSc, respectively 1.6 (CI 0.5 to 5.2), 1.6 (CI 0.7 to 3.8) and 2.8 (CI 1.2 to 6.6).ConclusionsCPFE increases the mortality risk in SSc along with a highly impaired lung function. These findings strengthen the importance to take into account emphysema in patients with SSc with ILD.

scholarly journals Serum IGFBP-2 in systemic sclerosis as a prognostic factor of lung dysfunction

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Julien Guiot ◽  
Makon-Sébastien Njock ◽  
Béatrice André ◽  
Fanny Gester ◽  
Monique Henket ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Prognostic Factor ◽  
Pulmonary Function ◽  
Lung Disease ◽  
Pulmonary Function Tests ◽  
Serum Levels ◽  
Roc Curve Analysis ◽  
Significant Deterioration ◽  
Lung Dysfunction

AbstractSystemic sclerosis (SSc) is a rare connective tissue disease associated with rapid evolving interstitial lung disease (ILD), driving its mortality. Specific biomarkers associated with the progression of this lung disease are highly needed. We aimed to identify specific biomarkers of SSc-ILD to predict the evolution of the disease. For this, we compared prospectively serum levels of several biomarkers associated with lung fibrosis in SSc patients (n = 102), among which SSc-no ILD (n = 63) and SSc-ILD (n = 39), compared to healthy subjects (HS) (n = 39). We also performed a longitudinal study in a subgroup of 28 patients analyzing biomarkers variations and pulmonary function tests over a period of 2 years. Serum level of IGFBP-2 was significantly increased in SSc patients compared to HS, and negatively correlated with pulmonary function (assessed by carbon monoxide transfer coefficient (KCO)) (r = − 0.29, p < 0.01). Two-year longitudinal analysis in a subgroup of 28 SSc patients determined that IGFBP-2 variation was positively correlated with KCO at 2-year follow-up (r = 0.6, p < 0.001). SSc patients with a lower variation of IGFBP-2 (less than 22%) presented significant deterioration of pulmonary function at 2-year follow-up (p < 0.01). ROC curve analysis enabled us to identify that baseline IGFBP-2 > 105 ng/ml was associated with a poor outcome (KCO < 70% predicted) at 2-year follow-up (AUC = 0.75, p < 0.05). We showed for the first time that serum levels of IGFBP-2 might be a prognostic factor of the development of SSc-ILD.

Pulmonary Function Tests and Idiopathic Pulmonary Fibrosis

CHEST Journal ◽  
1997 ◽  
Vol 111 (1) ◽  
pp. 7-8 ◽  
Author(s):  
Steven H. Kirtland ◽  
Richard H. Winterbauer
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Pulmonary Function ◽  
Pulmonary Function Tests ◽  
Function Tests

scholarly journals Prognostic significance of pulmonary function tests in dyskeratosis congenita, a telomere biology disorder

2019 ◽  
Vol 5 (4) ◽  
pp. 00209-2019 ◽  
Author(s):  
Neelam Giri ◽  
Sandhiya Ravichandran ◽  
Youjin Wang ◽  
Shahinaz M. Gadalla ◽  
Blanche P. Alter ◽  
...  
Keyword(s):  
Pulmonary Fibrosis ◽  
Pulmonary Function ◽  
Pulmonary Disease ◽  
Cumulative Incidence ◽  
Pulmonary Function Tests ◽  
Dyskeratosis Congenita ◽  
Telomere Maintenance ◽  
Genetic Characteristics ◽  
Function Tests ◽  
Telomere Biology

Pulmonary fibrosis and pulmonary arteriovenous malformations are known manifestations of dyskeratosis congenita (DC), a telomere biology disorder (TBD) and inherited bone marrow failure syndrome caused by germline mutations in telomere maintenance genes resulting in very short telomeres. Baseline pulmonary function tests (PFTs) and long-term clinical outcomes have not been thoroughly studied in DC/TBDs.In this retrospective study, 43 patients with DC and 67 unaffected relatives underwent baseline PFTs and were followed for a median of 8 years (range 1–14). Logistic regression and competing risk models were used to compare PFT results in relation to clinical and genetic characteristics, and patient outcomes.Restrictive abnormalities on spirometry and moderate-to-severe reduction in diffusing capacity of the lung for carbon monoxide were significantly more frequent in patients with DC than relatives (42% versus 12%; p=0.008). The cumulative incidence of pulmonary disease by age 20 years was 55% in patients with DC with baseline PFT abnormalities compared with 17% in those with normal PFTs (p=0.02). None of the relatives developed pulmonary disease. X-linked recessive, autosomal recessive inheritance or heterozygous TINF2 variants were associated with early-onset pulmonary disease that mainly developed after haematopoietic cell transplantation (HCT). Overall, seven of 14 patients developed pulmonary disease post-HCT at a median of 4.7 years (range 0.7–12). The cumulative incidence of pulmonary fibrosis in patients with heterozygous non-TINF2 pathogenic variants was 70% by age 60 years.Baseline PFT abnormalities are common in patients with DC and associated with progression to significant pulmonary disease. Prospective studies are warranted to facilitate clinical trial development for patients with DC and related TBDs.

scholarly journals Serum biomarker CA 15-3 as predictor of response to antifibrotic treatment and survival in idiopathic pulmonary fibrosis

2020 ◽  
Vol 14 (11) ◽  
pp. 997-1007
Author(s):  
Sofia A Moll ◽  
Ivo A Wiertz ◽  
Adriane DM Vorselaars ◽  
Pieter Zanen ◽  
Henk JT Ruven ◽  
...  
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Pulmonary Function ◽  
Vital Capacity ◽  
Mixed Model ◽  
Pulmonary Function Tests ◽  
Hazard Ratio ◽  
Poor Survival ◽  
Function Test

Aim: Cancer antigen 15-3 (CA 15-3) is a baseline biomarker in idiopathic pulmonary fibrosis (IPF), but its value during follow-up is unknown. Materials and methods: Associations between serum CA 15-3 and pulmonary function tests during 1-year follow-up were evaluated by a mixed model in 132 IPF treated with pirfenidone or nintedanib. Results: Increased baseline (median: 56 kU/l) and follow-up CA 15-3 levels were inversely associated with forced vital capacity and diffusing capacity of the lung for carbon monoxide (estimates respectively: -5.21 and -4.69; p < 0.001). Baseline and 6-month CA 15-3 above 58.5 (hazard ratio: 1.67; p = 0.031) and 50.5 kU/l (hazard ratio: 2.99; p < 0.001), respectively, showed impaired survival compared with lower levels. Conclusion: CA 15-3 is associated with pulmonary function test during follow-up in IPF on antifibrotic treatment. Higher (follow-up) values are related with poor survival. Therefore, CA 15-3 is a promising follow-up biomarker in IPF.

scholarly journals P151 Forced vital capacity in patients with systemic sclerosis associated pulmonary fibrosis: predictors of meaningful decline

Rheumatology ◽  
2020 ◽  
Vol 59 (Supplement_2) ◽  
Author(s):  
Svetlana I Nihtyanova ◽  
Emma C Derrett-Smith ◽  
Carmen Fonseca ◽  
Voon H Ong ◽  
Christopher P Denton
Keyword(s):  
Systemic Sclerosis ◽  
Pulmonary Fibrosis ◽  
Forced Vital Capacity ◽  
Vital Capacity ◽  
Cox Regression ◽  
Pulmonary Function Tests ◽  
Age At Onset ◽  
Time Factors ◽  
Standard Management ◽  
Over Time

Abstract Background Pulmonary fibrosis (PF) is common in systemic sclerosis (SSc) and serial pulmonary function tests (PFTs) are used for routine PF monitoring. Forced vital capacity (FVC) decline reflects progression in PF and FVC is frequently used as an endpoint in clinical trials. We explore the changes in FVC over time in patients with SSc-PF, receiving standard management, including immunosuppression. Methods Only SSc patients with CT-confirmed PF were included. FVC changes over the first 10 years from onset and their associations were assessed using linear mixed effects models. Predictors of time from first PFT to development of threshold FVC (FVC&lt;70% and FVC&lt;50%) were analysed using Cox regression. Results We identified 505 SSc-PF subjects, 21.6% were male, average age at onset was 47 years and 49.3% had diffuse cutaneous subset (dcSSc). The most common autoantibody was anti-Scl70 in 40.4%, followed by anti-RNA polymerase (ARA) in 11.7% and anti-centromere (ACA) in 7.1%. In 16.4% of the patients, ANA was positive, but no ENAs were identified (ANA+ENA-). Average FVC at 12 months from onset was 80.1% (SD 19.3) and this declined by 0.32% per year (p = 0.007) at a group level. There was no significant correlation between baseline FVC and subsequent change (correlation coefficient -0.13; 95%CI -0.26, 0.01). For every year older age at onset, average FVC increased by 0.32%, p &lt; 0.001. Males had 3.3% lower FVC at 1 year from onset (p &lt; 0.001) and 0.6% faster decline per year (p = 0.034) compared to females. DcSSc subjects had 5.6% lower FVC compared to limited disease (p = 0.003). Average FVC at 1 year from onset in ARA+ subjects were higher than any other antibody (15.1% v. ANA+ENA-, p &lt; 0.001; 14.6% v. ATA, p &lt; 0.001 and 12.5% v. ACA, p = 0.010). Nevertheless, ARA+ subjects had FVC decline rates similar to ATA+ and ANA+ENA- subjects, while ACA+ patients had a small but significant increase in FVC over time. Factors that increased the risk for FVC drop below the thresholds were male sex, ATA and low baseline FVC (Table 1). Conclusion This study provides insight into long-term patterns of FVC change and develops a model that may help predict those most at risk of significant decline. Disclosures S.I. Nihtyanova None. E.C. Derrett-Smith None. C. Fonseca None. V.H. Ong None. C.P. Denton None.

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