Analysis of systemic lupus erythematosus-related interstitial pneumonia: a retrospective multicentre study

Noriyuki Enomoto, Ryoko Egashira, Kazuhiro Tabata, Mikiko Hashisako, Masashi Kitani, Yuko Waseda, Tamotsu Ishizuka, Satoshi Watanabe, Kazuo Kasahara, Shinyu Izumi, Akira Shiraki, Atsushi Miyamoto, Kazuma Kishi, Tomoo Kishaba, Chikatosi Sugimoto, Yoshikazu Inoue, Kensuke Kataoka, Yasuhiro Kondoh, Yutaka Tsuchiya, Tomohisa BabaHiroaki Sugiura, Tomonori Tanaka, Hiromitsu Sumikawa, Takafumi Suda

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30 Citations (Scopus)


Thoracic diseases in patients with systemic lupus erythematosus (SLE), especially interstitial pneumonia (SLE-IP), are rare and have been poorly studied. The aims of this multicentre study were to evaluate SLE-IP and elucidate its clinical characteristics and prognosis. Fifty-five patients with SLE-IP who had attended the respiratory departments of participating hospitals were retrospectively evaluated in this multicentre study. Clinical information, high-resolution computed tomography (HRCT), and surgical lung biopsy/autopsy specimens were analysed by respiratory physicians, pulmonary radiologists, and pulmonary pathologists. IP patterns on HRCT and lung specimens were classified based on the international classification statement/guideline for idiopathic interstitial pneumonias. The most frequent form of SLE-IP at diagnosis was chronic IP (63.6%), followed by subacute (20.0%), and acute IP (12.7%). Radiologically, the most common HRCT pattern was “Unclassifiable” (54%). Histologically, “Unclassifiable” was the most frequently found (41.7%) among 12 patients with histologically proven IP. Interestingly, accompanying airway diseases were present in nine of these patients (75%). In multivariate analysis, current smoking (hazard ratio [HR] 6.105, p = 0.027), thrombocytopenia (HR 7.676, p = 0.010), anti-double-strand DNA titre (HR 0.956, p = 0.027), and nonspecific interstitial pneumonia (NSIP) + organizing pneumonia (OP) pattern on HRCT (vs. NSIP, HR 0.089, p = 0.023) were significant prognostic factors. In conclusion, chronic IP was the most frequent form of IP in patients with SLE-IP, and “Unclassifiable” was the commonest pattern radiologically and histologically.

Original languageEnglish
Article number7355
JournalScientific reports
Issue number1
Publication statusPublished - 2019 Dec 1
Externally publishedYes

ASJC Scopus subject areas

  • General


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