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. 2024 Nov 15;13(22):6870.
doi: 10.3390/jcm13226870.

The Clinical Significance of Interstitial Pneumonia with Autoimmune Features in Cryptogenic Organizing Pneumonia: A Prospective Multicenter Observational Study

Hisao Higo  1 , Hirohisa Ichikawa  2 , Yukako Arakawa  2 , Yoshihiro Mori  2 , Tomoki Tamura  3 , Shoichi Kuyama  3 , Chiaki Matsumoto  4 , Keisuke Sugimoto  4 , Noboru Hamada  5 , Toshimitsu Suwaki  5 , Junko Itano  6 , Yasushi Tanimoto  6 , Satoru Senoo  7 , Akihiko Taniguchi  7 , Yumi Inukai  1 , Machiko Arita  8 , Satoko Makimoto  9 , Katsuhide Kojima  9 , Takashi Matsushita  10 , Yoshinobu Maeda  11 , Nobuaki Miyahara  1   12
Affiliations

The Clinical Significance of Interstitial Pneumonia with Autoimmune Features in Cryptogenic Organizing Pneumonia: A Prospective Multicenter Observational Study

Hisao Higo et al. J Clin Med. .

Abstract

Background: There are cases of idiopathic interstitial pneumonias (IIPs) that do not meet the diagnostic criteria for connective tissue disease but have clinical features suggestive of autoimmune process. Interstitial pneumonia with autoimmune features (IPAF) was recently proposed as a research concept for these patients. Although several prospective studies on IPAF have been conducted, its clinical significance in cryptogenic organizing pneumonia (COP) remains unclear. Methods: Patients aged ≥20 years with suspected COP were prospectively enrolled between June 2018 and December 2022. Among the enrolled patients, those diagnosed with COP based on computed tomography (CT) and bronchoalveolar lavage (BAL) findings were compared between the IPAF and non-IPAF groups. Results: A total of 56 patients were enrolled in this study. Of these, 30 were diagnosed with COP and included in the analysis. Clinical and serological features were positive in two and six patients, respectively. Each feature was exclusive, and eight patients (26.7%) were diagnosed with IPAF. There were no differences between the IPAF and non-IPAF groups in terms of clinical features, including BAL findings, laboratory data, CT findings, and clinical course. During the one-year follow-up period, the frequency of COP exacerbation did not differ between the IPAF and non-IPAF groups, and no cases of systemic autoimmune disease or death occurred in either group. Conclusions: The COP characteristics of the IPAF and non-IPAF groups are similar in all aspects, and distinguishing between the two groups may be of little significance.

Keywords: bronchoalveolar lavage; connective tissue disease; cryptogenic organizing pneumonia; interstitial pneumonia with autoimmune features; prospective multicenter observational study.

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Conflict of interest statement

The authors declare no conflicts of interest.

Figures

Figure 1
Figure 1
Flow chart of the study. Of the 56 patients, 30 with cryptogenic organizing pneumonia were analyzed. Abbreviations: BAL, bronchoalveolar lavage; OP, organizing pneumonia; IPAF, interstitial pneumonia with autoimmune features.
Figure 2
Figure 2
Representative computed tomography images. (A–C) show cases from the IPAF group, and (D–F) show cases from the non-IPAF group. (A,B,D,E) depict cases with peripheral dominant consolidation, while images (C,F) show cases with a reversed halo sign (red arrowhead). Abbreviations: IPAF, interstitial pneumonia with autoimmune features.
Figure 3
Figure 3
The rate of corticosteroid use in the IPAF group and non-IPAF group during a one-year follow-up period. Abbreviations: IPAF, interstitial pneumonia with autoimmune features.
Figure 4
Figure 4
The rate of COP exacerbations in the IPAF group and non-IPAF group during the one-year follow-up period. Abbreviations: COP, cryptogenic organizing pneumonia; IPAF, interstitial pneumonia with autoimmune features.
See this image and copyright information in PMC

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