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Abstract Number: 2167

Incidence and Baseline Characteristics of Exacerbation in Patients with Interstitial Pneumonia with Autoimmune Features: A Single Center Large-Scale Cross-Sectional Cohort Study

Okinori Murata, Nobuhito Sasaki and Makoto Maemondo, Division of Pulmonary medicine, Allergy, and Rheumatology, Department of Internal Medicine, Iwate Medical University School of Medicine, Morioka, Japan

Meeting: 2018 ACR/ARHP Annual Meeting

Keywords: autoimmune diseases, Connective tissue diseases, interstitial lung disease and prognostic factors

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Session Information

Date: Tuesday, October 23, 2018

Title: Epidemiology and Public Health Poster III: SLE, SSc, APS, PsA, and Other Rheumatic Diseases

Session Type: ACR Poster Session C

Session Time: 9:00AM-11:00AM

Background/Purpose: Patients with idiopathic interstitial pneumonia (IIP) may have features of connective tissue diseases (CTDs). The term interstitial pneumonia with autoimmune features (IPAF) has been recently proposed for such patients. A few studies have been reported in prevalence of IPAF which was varied from 7.3% to 34.1% [1, 2]. Factors reported to indicate a poor prognosis in IPAF include age, smoking history, organizing pneumonia pattern in HRCT, anti-RNP antibody positivity, decline in %DLCO and presence of a multi-compartment feature within the morphological domain [2, 3]. To date, however, no study has comprehensively described prevalence of IPAF and factors of exacerbation. The aim of study was to identify of prevalence of IPAF in patients with interstitial lung disease and prognostic factors for exacerbation in patients with IPAF.

Methods: Six hundreds- and seventy-two patients who visited our department between April 2009 and March 2018 and were evaluated by chest HRCT scan. Then, they were clinically and radiologically diagnosed as having interstitial lung disease (ILD), IIP or connective tissue diseases associated ILD were enrolled. We applied IPAF criteria to these patients. Then, we purified 68 patients. The prognostic factors for exacerbation were prospectively calculated and statistically analyzed using clinical, laboratory and imaging data collected from medical records.

Results: Prevalence of IPAF was 10.1%. Of 68 patients with IPAF, 60% were women and mean age at diagnosis was 64.2 ± 13.8 years old. Mean observation period was 27.1 ± 29.6 months. Exacerbation rate was 25% (n=17). Overall death rate was 5.9% (n=4) and respiratory death rate was 2.9% (n=2). Comparison of characteristics at diagnosis between the exacerbation group and non-exacerbation group showed that the exacerbation group had a significantly elevated rate of smoking, KL-6, and SP-D (P=0.012, 0.0064, and 0.029, respectively).

Conclusion: Our large-scale cross-sectional cohort study identified unique prognostic factors of exacerbation.


Disclosure: O. Murata, None; N. Sasaki, None; M. Maemondo, None.

To cite this abstract in AMA style:

Murata O, Sasaki N, Maemondo M. Incidence and Baseline Characteristics of Exacerbation in Patients with Interstitial Pneumonia with Autoimmune Features: A Single Center Large-Scale Cross-Sectional Cohort Study [abstract]. Arthritis Rheumatol. 2018; 70 (suppl 9). https://acrabstracts.org/abstract/incidence-and-baseline-characteristics-of-exacerbation-in-patients-with-interstitial-pneumonia-with-autoimmune-features-a-single-center-large-scale-cross-sectional-cohort-study/. Accessed .
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