An Analysis of Connective Tissue Disease-associated Interstitial Lung Disease at a US Tertiary Care Center: Better Survival in Patients with Systemic Sclerosis

2011 ◽  
Vol 38 (4) ◽  
pp. 693-701 ◽  
Author(s):  
ROBERT SU ◽  
MIHOKO BENNETT ◽  
SUSAN JACOBS ◽  
TESSA HUNTER ◽  
CARISSA BAILEY ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Interstitial Lung Disease ◽  
Connective Tissue ◽  
Lung Disease ◽  
Connective Tissue Disease ◽  
Care Center ◽  
Tertiary Care ◽  
Survival Rates ◽  
Tertiary Care Center ◽  
Risk Of Death

Objective.To compare survival of patients with connective tissue disease-associated interstitial lung disease (CTD-ILD) versus idiopathic pulmonary fibrosis (IPF) and patients with systemic sclerosis-associated ILD (SSc-ILD) versus other CTD-ILD followed at our center.Methods.We used the Stanford ILD database, which contains prospectively collected information on patients with ILD evaluated at our tertiary care center from 2002 to 2009. Survival at last followup from time of ILD diagnosis was calculated using the Kaplan-Meier estimator. Prognostic factors for survival in the overall cohort (IPF and CTD-ILD) and in the CTD-ILD group were identified with univariate and multivariate Cox regression models.Results.Of 427 patients with ILD, 148 (35%) had IPF and 76 (18%) had CTD-ILD at the baseline visit. The cumulative incidence of CTD was 4%. After a median followup of 4 years, 67 patients (36.4%) had died and 4 (2.2%) were lost to followup. Patients with IPF (n = 122) and CTD-ILD (n = 62) experienced similar survival rates (5-year survival about 50%). Patients with SSc-ILD (n = 24) experienced better survival than those with other CTD-ILD (n = 38), with 1-year, 3-year, and 5-year survival rates of 100%, 90%, and 77%, respectively, versus 78%, 42%, and 38% (p = 0.01). The presence of SSc in patients with CTD-ILD decreased the risk of death by > 80% even after correcting for age at ILD diagnosis, sex, and ethnicity (HR = 0.17, 95% CI 0.04–0.83).Conclusion.Survival in patients with SSc-ILD was better than in patients with other CTD-ILD, potentially related to routine screening for and early detection of ILD in patients with SSc at our center.

scholarly journals A study of respiratory system involvement in autoimmune connective tissue diseases: a marker of morbidity

2020 ◽  
Vol 6 (4) ◽  
pp. 532
Author(s):  
Neha T. Solanki ◽  
Sahana P. Raju ◽  
Deepmala Budhrani ◽  
Bharti K. Patel
Keyword(s):  
Systemic Sclerosis ◽  
Interstitial Lung Disease ◽  
Connective Tissue ◽  
Pulmonary Function ◽  
Lung Disease ◽  
Pulmonary Function Tests ◽  
Tertiary Care ◽  
Connective Tissue Diseases ◽  
Cutaneous Manifestations ◽  
Respiratory Involvement

<p class="abstract"><strong>Background:</strong> The auto-immune connective tissue diseases (AICTD) are polygenic clinical disorders having heterogeneous overlapping clinical features. Certain features like autoimmunity, vascular abnormalities, arthritis/arthralgia and cutaneous manifestations are common to them. Lung involvement can present in AICTDs in form of: pleurisy, acute/ chronic pneumonitis, pulmonary artery hypertension (PAH), shrinking lung syndrome, diffuse alveolar damage, pulmonary embolism (PE), bronchiolitis obliterans organizing pneumonia, pulmonary infections, cardiogenic pulmonary edema, etc. High-resolution computed tomography (HRCT) plays an important role in identifying patients with respiratory involvement. Pulmonary function tests are a sensitive tool detecting interstitial lung disease.</p><p class="abstract"><strong>Methods:</strong> The present study is an observational study carried out on 170 patients of AICTD in department of Dermatology, Venereology and Leprosy at a tertiary care centre during a period of 2 years from October 2017 to August 2019. Detailed history, examination and relevant investigations like chest X-ray, pulmonary function test (PFT), HRCT thorax were done as indicated.<strong></strong></p><p class="abstract"><strong>Results:</strong> The overall incidence of respiratory involvement was 56.7% with maximum involvement in systemic sclerosis cases (82.8% of cases). 45.7% of patients of systemic lupus erythematosus had respiratory involvement, most common being pleural effusion in 11.5%. Impaired PFT’s were seen in 82.8% cases of systemic sclerosis (SSc)  and all cases of UCTD. Interstitial lung disease was seen in 34.7% and 25% cases of SSc and DM respectively. PAH was found in 15.2% cases of SSc and 9.8% cases of mixed connective tissue diseases.</p><p class="abstract"><strong>Conclusions:</strong> AICTD are multisystem disorders in which pulmonary involvement can be an important cause of morbidity to the patient and early detection is necessary for prevention of long-term respiratory complications.</p>

scholarly journals SAT0582 RISK OF INTERSTITIAL LUNG DISEASE IN SYSTEMIC AUTOIMMUNE RHEUMATIC DISEASES: A NATIONWIDE, POPULATION-BASED COHORT STUDY

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1250.1-1250
Author(s):  
H. H. Chen ◽  
K. H. Ng ◽  
W. C. Chao ◽  
C. H. Lin
Keyword(s):  
Cohort Study ◽  
Systemic Sclerosis ◽  
Interstitial Lung Disease ◽  
Connective Tissue ◽  
Lung Disease ◽  
Connective Tissue Disease ◽  
Rheumatic Diseases ◽  
Population Based ◽  
Autoimmune Rheumatic Diseases ◽  
Comparison Groups

Background:To date, very few studies had investigated the epidemiology of interstitial lung disease (ILD) among patients with systemic autoimmune rheumatic disease (SARD).Objectives:To study the risk of interstitial lung disease (ILD) among patients with various systemic autoimmune rheumatic diseases (SARDs) including rheumatoid arthritis (RA), dermatomyositis (DMtis), polymyositis (PM), systemic sclerosis (SSc), systemic lupus erythematosus (SLE), and primary Sjögren’s syndrome (pSS).Methods:Using 1997–2013 claims data from the Taiwanese National Health Insurance Research Database, we identified 63,277 newly diagnosed patients with various SARDs after excluding those with overlapping SARD diagnoses from 2001-2013 and randomly selected 253,108 non-SARD subjects matching (1:4) SARD patients for SARD diagnosis, age, sex and the year of the index date. We calculated the incidence rates (IRs) of ILD (ICD-9 code 515) in various SARD groups and the corresponding non-SARD comparison groups and estimated the IR ratios (IRRs) with 95% confidence intervals (CI) of ILD development. Using multivariable cox regression analyses, we estimated hazard ratios (HRs) with 95% CIs of ILD in various SARD groups compared with their comparison groups after adjusting for age, sex, Charlson comorbidity index, amiodarone use and methotrexate use. Sensitivity analyses were conducted by using a narrow definition of ILD.Results:As shown in Table 1, the IRs of ILD were greatest in SSc patients (2,523 per 105years), followed by patients with DMtis (2,463 per 105years), PM (1,956 per 105years), SS (601 per 105years), RA (279 per 105years), and SLE (276 per 105years). Multivariable analyses showed that the risks of ILD were significantly increased in patients with SSc (HR, 66.01; 95% CI, 32.73—133.13), DMtis (128.74, 95% CI, 40.19—412.47), PM (HR, 30.39; 95% CI, 11.24—82.15), pSS (HR, 8.76; 95% CI, 7.03—10.90), RA (HR, 4.22; 95% CI, 3.51—5.08), and SLE (HR, 13.98; 95% CI, 9.25—21.14).Conclusion:This nationwide, population-based, matched cohort study demonstrated that the risks of ILD were significantly increased in patients with SARDs.References:[1]Su R, Bennett M, Jacobs S, Hunter T, Bailey C, Krishnan E, et al. An analysis of connective tissue disease-associated interstitial lung disease at a US Tertiary Care Center: better survival in patients with systemic sclerosis. The Journal of rheumatology. 2011;38(4):693-701.[2]Hu Y, Wang LS, Wei YR, Du SS, Du YK, He X, et al. Clinical Characteristics of Connective Tissue Disease-Associated Interstitial Lung Disease in 1,044 Chinese Patients. Chest. 2016;149(1):201-8.Araki T, Putman RK, Hatabu H, Gao W, Dupuis J, Latourelle JC, et al. Development and Progression of Interstitial Lung Abnormalities in the Framingham Heart Study. Am J Respir Crit Care Med 2016;194:1514-1522.Disclosure of Interests:None declared

Comparative analysis of connective tissue disease-associated interstitial lung disease and idiopathic pulmonary fibrosis from a tertiary care centre in Pakistan

2021 ◽  
Vol 71 (10) ◽  
pp. 2330-2334
Author(s):  
Ali Bin Sarwar Zubairi ◽  
Huzaifa Ahmad ◽  
Maryam Hassan ◽  
Faraz Siddiqui ◽  
Nousheen Iqbal ◽  
...  
Keyword(s):  
Comparative Analysis ◽  
Interstitial Lung Disease ◽  
Idiopathic Pulmonary Fibrosis ◽  
Connective Tissue ◽  
Pulmonary Fibrosis ◽  
Lung Disease ◽  
Connective Tissue Disease ◽  
Interstitial Pneumonitis ◽  
Tertiary Care ◽  
Data Registry

Objectives: The burden of interstitial lung disease (ILD) is rising globally. This study aimed to describe and compare characteristics of Connective Tissue Disease-associated ILD with Idiopathic Pulmonary Fibrosis, the two most commonly observed ILDs among outpatients at a tertiary care hospital in Karachi, Pakistan. Methods: A retrospective research study was conducted. Patients with ILD were identified through the outpatient data registry at the Aga Khan University Hospital (AKUH), Karachi from October 2016 to October 2017. We obtained data pertaining to demographics, clinical and radiologic features. A comparative analysis was done to compare the patient characteristics and key features between CTD-ILD and IPF patients. The analysis was done using STATA version 12.0. Results: We identified 184 patients with ILD, which included 52 (29.3%) with CTD-ILD and 62 (35%) with IPF. The most prevalent conditions among CTD-ILD patients included rheumatoid arthritis (42.3%) and scleroderma (25%). Usual interstitial pneumonitis was the common radiologic pattern in RA-ILD (63.6%) and scleroderma (61.5%) while non-specific interstitial pneumonitis was more common in MCTD (85.7%) and SLE (80%). Compared to patients with IPF, those with CTD-ILD were predominantly younger (p<0.001) and female (88.5 % v 45.2%, p<0.001). History of GERD was also significantly lower in CTD-ILD (p=0.05). Conclusion: CTD-ILD patients in our registry were younger and predominantly female compared to IPF. Further studies and ongoing data registry are needed to understand the full spectrum of this disease and long term clinical outcomes. Keywords: Connective tissue disease, Interstitial lung disease, Idiopathic pulmonary fibrosis. Continuous...

scholarly journals Demographic and clinical predictors of progression and mortality in connective tissue disease-associated interstitial lung disease: a retrospective cohort study

2019 ◽  
Vol 19 (1) ◽  
Author(s):  
Chrystal Chan ◽  
Christopher J. Ryerson ◽  
James V. Dunne ◽  
Pearce G. Wilcox
Keyword(s):  
Systemic Sclerosis ◽  
Interstitial Lung Disease ◽  
Connective Tissue ◽  
Lung Disease ◽  
First Nations ◽  
Connective Tissue Disease ◽  
South Asian ◽  
Multivariable Analysis ◽  
Older Age ◽  
Male Sex

Abstract Background Connective tissue disease-associated interstitial lung disease (CTD-ILD) is associated with reduced quality of life and poor prognosis. Prior studies have not identified a consistent combination of variables that accurately predict prognosis in CTD-ILD. The objective of this study was to identify baseline demographic and clinical characteristics that are associated with progression and mortality in CTD-ILD. Methods Patients were retrospectively identified from an adult CTD-ILD clinic. The predictive significance of baseline variables on serial forced vital capacity (FVC), diffusion capacity (DLCO), and six-minute walk distance (6MWD) was assessed using linear mixed effects models, and Cox regression analysis was performed to assess impact on mortality. Results 359 patients were included in the study. Median follow-up time was 4.0 (IQR 1.5–7.6) years. On both unadjusted and multivariable analysis, male sex and South Asian ethnicity were associated with decline in FVC. Male sex, positive smoking history, and diagnosis of systemic sclerosis (SSc) vs. other CTD were associated with decline in DLCO. Male sex and usual interstitial pneumonia (UIP) pattern predicted decline in 6MWD. There were 85 (23.7%) deaths. Male sex, older age, First Nations ethnicity, and a diagnosis of systemic sclerosis vs. rheumatoid arthritis were predictors of mortality on unadjusted and multivariable analysis. Conclusion Male sex, older age, smoking, South Asian or First Nations ethnicity, and UIP pattern predicted decline in lung function and/or mortality in CTD-ILD. Further longitudinal studies may add to current clinical prediction models for prognostication in CTD-ILD.

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