Differences in clinical features of acute exacerbation between connective tissue disease-associated interstitial pneumonia and idiopathic pulmonary fibrosis | Zendy

Enomoto Noriyuki | Zendy; Oyama Yoshiyuki | Zendy; Enomoto Yasunori | Zendy; Yasui Hideki | Zendy; Karayama Masato | Zendy; Kono Masato | Zendy; Hozumi Hironao | Zendy; Suzuki Yuzo | Zendy; Furuhashi Kazuki | Zendy; Fujisawa Tomoyuki | Zendy; Inui Naoki | Zendy; Nakamura Yutaro | Zendy; Suda Takafumi | Zendy

Open Access

Differences in clinical features of acute exacerbation between connective tissue disease-associated interstitial pneumonia and idiopathic pulmonary fibrosis

Author(s) -

Enomoto Noriyuki,

Oyama Yoshiyuki,

Enomoto Yasunori,

Yasui Hideki,

Karayama Masato,

Kono Masato,

Hozumi Hironao,

Suzuki Yuzo,

Furuhashi Kazuki,

Fujisawa Tomoyuki,

Inui Naoki,

Nakamura Yutaro,

Suda Takafumi

Publication year - 2018

Publication title -

chronic respiratory disease

Language(s) - English

Resource type - Journals

SCImago Journal Rank - 0.929

H-Index - 41

eISSN - 1479-9731

pISSN - 1479-9723

DOI - 10.1177/1479972318809476

Subject(s) - medicine , idiopathic pulmonary fibrosis , ctd , honeycombing , connective tissue disease , usual interstitial pneumonia , exacerbation , gastroenterology , idiopathic interstitial pneumonia , fibrosis , stage (stratigraphy) , interstitial lung disease , disease , lung , paleontology , oceanography , biology , autoimmune disease , geology

Acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) is a devastating condition that frequently occurs in the advanced stage of IPF. However, the clinical features in AE of connective tissue disease-associated interstitial pneumonia (AE-CTD-IP) have not been well-established. The aim of this study was to clarify the clinical features of AE-CTD-IP and to compare them with those of AE-IPF. Fifteen AE-CTD-IP patients and 48 AE-IPF patients who were diagnosed and treated at our hospital were retrospectively studied. Compared with AE-IPF patients, AE-CTD-IP patients had a significantly higher %FVC (median, 94.8 vs. 56.3%; p < 0.001) and a lower extent of honeycombing on HRCT ( p = 0.020) within 1 year before AE. At AE, AE-CTD-IP patients showed higher white blood cell counts (12.0 vs. 9.9 × 10 3 /μL; p = 0.023), higher CRP (10.2 vs. 6.7 mg/dL; p = 0.027), and longer period from admission to the beginning of AE treatment (4 vs. 1 days; p = 0.003) than AE-IPF patients. In addition, patients with AE-CTD-IP had poor prognosis as in those with AE-IPF (log-rank; p = 0.171). In conclusion, AE-CTD-IP occurred even in the early stage of IP and had more inflammatory status than in AE-IPF.

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