FRI0485 RISK OF PROGRESSION OF IDIOPATHIC PULMONARY FIBROSIS TO CONNECTIVE TISSUE DISEASE

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 840-841
Author(s):  
B. Ghang ◽  
S. H. Nam ◽  
Y. G. Kim ◽  
B. Yoo ◽  
C. K. Lee
Keyword(s):  
Risk Factors ◽  
Idiopathic Pulmonary Fibrosis ◽  
Connective Tissue ◽  
Pulmonary Fibrosis ◽  
Rheumatoid Factor ◽  
Connective Tissue Disease ◽  
Proportional Hazards ◽  
Cox Proportional Hazards ◽  
Survival Duration ◽  
Citrullinated Protein

Background:Connective tissue disease (CTD) may be observed during the course of idiopathic pulmonary fibrosis (IPF). However, clinical factors associated with the development of CTD in patients with IPF have not yet been identified. These factors might be valuable clues for determining the pathogenesis of pulmonary fibrosis in patients with CTD. We hypothesize that some IPF patients have a clinically significant association with autoimmunity, and that autoantibodies are important biomarkers for identifying these patients.Objectives:Based on this hypothesis, we investigated whether the serology criteria (anti-neutrophil cytoplasmic antibody (ANCA) or autoantibodies that met the serology criteria for interstitial pneumonitis with autoimmune features (IPAF)) were associated with the development of CTD during the clinical course of IPF in the patients from our previous study(1), with a particular focus on which antibodies have a significant association with the development of CTD.Methods:We retrospectively reviewed the records of 527 patients with a first diagnosis of IPF between January 2007 and March 2014, and investigated the length of time from first visit to the clinic for IPF diagnosis (baseline) to CTD diagnosis by an expert rheumatologist in patients with IPF. Multivariable Cox proportional-hazards models with backward elimination were used to investigate the risk factors for the development of CTD.Results:CTD developed in 15 patients at a median of 2.1 years (range 1.2 to 4.8) after IPF diagnosis. All these patients had ANCA or autoantibodies that met the serology criteria for IPAF. A significant number of IPF patients with high titers of RF, ACPA or MPO-ANCA tested at first visit to the clinic progressed to CTD(Figure 1). Survival duration for IPF patients with progression to CTD was 5.3 [3.8; 6.7] years, which was significantly longer than for the IPF patients without progression to CTD (2.9 [1.7; 4.8], p = 0.001). Independent risk factors for development of CTD in IPF patients included female gender (adjusted hazard ratio (HR) 5.319, p = 0.0082), titer of rheumatoid factor (RF) (adjusted HR 1.006, p = 0.022), titer of anti-citrullinated protein antibody (ACPA) (adjusted HR 1.009, p = 0.0011), and titer of myeloperoxidase (MPO) ANCA (adjusted HR 1.02, p < 0.0001).Figure 1.Connective tissue disease development in each autoantibody positive IPF patient. ACPA = anti–citrullinated protein antibody; ANA = antinuclear antibody; CTD = connective tissue disease; MPA = microscopic polyangiitis; PAN = polyarteritis nodosa; RA = rheumatoid arthritis; RF = rheumatoid factor; UCTD = Undifferentiated connective tissue disease; SjS = Sjögren’s syndrome.Conclusion:We observed development of CTD in IPF patients with ANCA or autoantibodies that met the IPAF serology criteria. Among these autoantibodies, RF, ACPA, and MPO-ANCA were significantly associated with the development of CTD in IPF patients. Progression to CTD is uncommon in IPF patients, but a significant number of IPF patients with high titers of RF, ACPA or MPO-ANCA progressed to connective tissue disease. IPF with high titers of RF, ACPA or MPO-ANCA might be the initial clinical manifestation of connective tissue disease. Further studies are needed to investigate the role of RF, ACPA, and MPO-ANCA in development of pulmonary fibrosis.References:[1]Ghang B, Lee J, Chan Kwon O, Ahn SM, Oh JS, Hong S, et al. Clinical significance of autoantibody positivity in idiopathic pulmonary fibrosis. Respir Med. 2019;155:43-8.Disclosure of Interests:None declared

scholarly journals Subsequent Identification of a Connective Tissue Disease Amongst Patients with Idiopathic Pulmonary Fibrosis : A Long-term Observational Study in 527 Patients

2020 ◽  
Author(s):  
Byeong-zu Ghang ◽  
So Hye Nam ◽  
Jungsun Lee ◽  
Doo-Ho Lim ◽  
Soo Min Ahn ◽  
...  
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Connective Tissue ◽  
Pulmonary Fibrosis ◽  
Connective Tissue Disease ◽  
Female Gender ◽  
Survival Duration ◽  
Independent Risk Factors ◽  
Citrullinated Protein ◽  
Subsequent Identification

Abstract Background: Connective tissue disease (CTD) might occur during the course of idiopathic pulmonary fibrosis (IPF). Clinical factors associated with CTD development in IPF patients have still not been identified. We investigated which antibodies have a significant association with the development of CTD during the clinical course of IPF.Methods: We retrospectively reviewed the records of 527 patients with a first diagnosis of IPF between January 2007 and March 2014 and investigated the time to CTD development after IPF diagnosis in these patients.Results: CTD developed in 15 patients at a median of 2.1 years (range 1.2–4.8) after IPF diagnosis. All patients had anti-neutrophil cytoplasmic antibodies (ANCA) or autoantibodies that met the serology criteria for interstitial pneumonitis with autoimmune features. Survival duration for IPF patients with progression to CTD was 5.3 (3.8, 6.7) years, which was significantly longer than for IPF patients without progression to CTD [2.9 (1.7, 4.8), p = 0.001]. Independent risk factors for CTD development in IPF patients included female gender [adjusted hazard ratio (HR) 5.319, p = 0.0082], titer of rheumatoid factor (RF; adjusted HR, 1.006; p = 0.022), titer of anti-citrullinated protein antibody (ACPA; adjusted HR, 1.009; p = 0.0011), and titer of myeloperoxidase (MPO)-ANCA (adjusted HR, 1.02; p < 0.0001).Conclusions: Progression to CTD is uncommon in IPF patients. However, a significant number of IPF patients with high titers of RF, ACPA, or MPO-ANCA progressed to CTD. RF, ACPA, and MPO-ANCA might be significantly associated with CTD development in IPF patients.

scholarly journals Social deprivation in Scottish populations with pulmonary hypertension secondary to connective tissue disease and chronic thromboembolic disease

2020 ◽  
Vol 6 (4) ◽  
pp. 00297-2019 ◽  
Author(s):  
Michael McGettrick ◽  
Paul McCaughey ◽  
Alexander MacLellan ◽  
Melanie Brewis ◽  
A. Colin Church ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Connective Tissue ◽  
Connective Tissue Disease ◽  
Proportional Hazards ◽  
Social Deprivation ◽  
Population Distribution ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Cox Proportional Hazards ◽  
Functional Class ◽  
The Usa

Socioeconomic factors have been shown to have an adverse impact on survival in some respiratory diseases. Studies from the USA and China have suggested worse survival in idiopathic pulmonary arterial hypertension in low socioeconomic groups. We looked at the effect of deprivation on the outcomes in patients with connective tissue disease-associated pulmonary hypertension (CTDPH) and chronic thromboembolic pulmonary hypertension (CTEPH) in a retrospective observational study.Data were obtained from 232 patients with CTDPH and 263 with CTEPH who were under the care of the Scottish Pulmonary Vascular Unit, Glasgow, UK. We used Cox proportional hazards regression to assess for a relationship between deprivation and survival.We found no difference in survival across deprivation quintiles in the CTDPH (p=0.26) or CTEPH cohorts (p=0.18). We constructed multivariate models using enrolment time, age, sex and body mass index, with no significant change in findings. There was no difference between expected and observed population distribution of CTDPH (p=0.98) and CTEPH (p=0.36). Whilst there was no difference in presenting functional class in the CTDPH group, the CTEPH patients in more deprived quintiles presented in a worse functional class (p=0.032). There was no difference between quintiles of CTEPH patients who had distal or proximal disease (p=0.75), or who underwent surgery (p=0.5).Increased social deprivation is not associated with worse survival in patients with CTDPH and CTEPH managed in the Scottish National Health Service. Whilst there is no evidence of referral barriers in CTDPH, this may not be the case in CTEPH, as lower deprivation was associated with worse functional class at presentation.

Patient gender bias on the diagnosis of idiopathic pulmonary fibrosis

Thorax ◽  
2020 ◽  
Vol 75 (5) ◽  
pp. 407-412 ◽  
Author(s):  
Deborah Assayag ◽  
Julie Morisset ◽  
Kerri A Johannson ◽  
Athol U Wells ◽  
Simon L F Walsh
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Proportional Hazards ◽  
Usual Interstitial Pneumonia ◽  
Clinical Information ◽  
Cox Proportional Hazards ◽  
Diagnostic Confidence ◽  
Patient Gender ◽  
And Gender ◽  
Gender Influences

BackgroundPatient sex has clinical and prognostic implications in idiopathic pulmonary fibrosis (IPF). It is not known if sex-related and gender-related discrepancies exist when establishing a diagnosis of IPF. The aim was to determine how patient gender influences the diagnosis of IPF and the physician’s diagnostic confidence.MethodsThis study was performed using clinical cases compiled from a single centre, then scored by respiratory physicians for a prior study. Using clinical information, physicians were asked to provide up to five diagnoses, together with their diagnostic confidence. Logistic regression was used to assess the odds of receiving a diagnosis of IPF based on patient gender. Prognostic discrimination between IPF and non-IPF was used to assess diagnostic accuracy with Cox proportional hazards modelling.ResultsSixty cases were scored by 404 physicians. IPF was diagnosed more frequently in men compared with women (37.8% vs 10.6%; p<0.0001), and with greater mean diagnostic confidence (p<0.001). The odds of a male patient receiving an IPF diagnosis was greater than that of female patients, after adjusting for confounders (OR=3.05, 95% CI: 2.81 to 3.31), especially if the scan was not definite for the usual interstitial pneumonia pattern. Mortality was higher in women (HR=2.21, 95% CI: 2.02 to 2.41) than in men with an IPF diagnosis (HR=1.26, 95% CI: 1.20 to 1.33), suggesting that men were more often misclassified as having IPF.ConclusionPatient gender influences diagnosis of IPF: women may be underdiagnosed and men overdiagnosed with IPF.

scholarly journals Comparison of Characteristics of Connective Tissue Disease-Associated Interstitial Lung Diseases, Undifferentiated Connective Tissue Disease-Associated Interstitial Lung Diseases, and Idiopathic Pulmonary Fibrosis in Chinese Han Population: A Retrospective Study

2013 ◽  
Vol 2013 ◽  
pp. 1-7 ◽  
Author(s):  
Lin Pan ◽  
Yuan Liu ◽  
Rongfei Sun ◽  
Mingyu Fan ◽  
Guixiu Shi
Keyword(s):  
Interstitial Lung Disease ◽  
Idiopathic Pulmonary Fibrosis ◽  
Connective Tissue ◽  
Pulmonary Fibrosis ◽  
Lung Disease ◽  
Connective Tissue Disease ◽  
Lung Diseases ◽  
Interstitial Lung Diseases ◽  
Western China ◽  
Undifferentiated Connective Tissue Disease

Our study compared the prevalence and characteristics of patients with connective tissue disease-associated interstitial lung disease (CTD-ILD), undifferentiated connective tissue disease-associated interstitial lung disease (UCTD-ILD), or idiopathic pulmonary fibrosis (IPF) between January 2009 and December 2012 in West China Hospital, western China. Patients who met the criteria for ILD were included and were assigned to CTD-ILD, UCTD-ILD, or IPF group when they met the criteria for CTD, UCTD, or IPF, respectively. Clinical characteristics, laboratory tests, and high-resolution CT images were analyzed and compared among three groups. 203 patients were included, and all were Han nationality. CTD-ILD was identified in 31%, UCTD-ILD in 32%, and IPF in 37%. Gender and age differed among groups. Pulmonary symptoms were more common in IPF, while extrapulmonary symptoms were more common in CTD-ILD and UCTD-ILD group. Patients with CTD-ILD had more abnormal antibody tests than those of UCTD-ILD and IPF. Little significance was seen in HRCT images among three groups. A systematic evaluation of symptoms and serologic tests in patients with ILD can identify CTD-ILD, UCTD-ILD, and IPF.

scholarly journals Clinical Outcomes of Patients with Combined Idiopathic Pulmonary Fibrosis and Emphysema in the IPF-PRO Registry

Lung ◽  
2022 ◽  
Author(s):  
Hyun J. Kim ◽  
Laurie D. Snyder ◽  
Megan L. Neely ◽  
Anne S. Hellkamp ◽  
David L. Hotchkin ◽  
...  
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Clinical Outcomes ◽  
Proportional Hazards ◽  
Cox Proportional Hazards ◽  
Cox Proportional Hazards Models ◽  
Baseline Variable ◽  
Former Smokers ◽  
The Impact ◽  
Baseline Covariates

Abstract Purpose To assess the impact of concomitant emphysema on outcomes in patients with idiopathic pulmonary fibrosis (IPF). Methods The IPF-PRO Registry is a US registry of patients with IPF. The presence of combined pulmonary fibrosis and emphysema (CPFE) at enrollment was determined by investigators’ review of an HRCT scan. Associations between emphysema and clinical outcomes were analyzed using Cox proportional hazards models. Results Of 934 patients, 119 (12.7%) had CPFE. Compared with patients with IPF alone, patients with CPFE were older (median 72 vs 70 years); higher proportions were current/former smokers (88.2% vs 63.7%), used oxygen with activity (49.6% vs 31.9%) or at rest (30.8% vs 18.4%), had congestive heart failure (13.6% vs 4.8%) and had prior respiratory hospitalization (25.0% vs 16.7%); they had higher FVC (median 71.8 vs 69.4% predicted) and lower DLco (median 35.3 vs 43.6% predicted). In patients with CPFE and IPF alone, respectively, at 1 year, rates of death or lung transplant were 17.5% (95% CI: 11.7, 25.8) and 11.2% (9.2, 13.6) and rates of hospitalization were 21.6% (14.6, 29.6) and 20.6% (17.9, 23.5). There were no significant associations between emphysema and any outcome after adjustment for baseline variables. No baseline variable predicted outcomes better in IPF alone than in CPFE. Conclusion Approximately 13% of patients in the IPF-PRO Registry had CPFE. Physiologic characteristics and comorbidities of patients with CPFE differed from those of patients with IPF alone, but the presence of emphysema did not drive outcomes after adjustment for baseline covariates. Trial registration ClinicalTrials.gov, NCT01915511; registered August 5, 2013.

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