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. 2020 Jun;25(6):636-643.
doi: 10.1111/resp.13665. Epub 2019 Aug 6.

Long-term clinical course and outcome of interstitial pneumonia with autoimmune features

Ho Cheol Kim  1 , Joo Hee Lee  1 , Eun Jin Chae  2 , Joon Seon Song  3 , Jin Woo Song  1
Affiliations

Long-term clinical course and outcome of interstitial pneumonia with autoimmune features

Ho Cheol Kim et al. Respirology. 2020 Jun.

Abstract

Background and objective: Idiopathic interstitial pneumonia (IIP) with autoimmune features that does not fulfil connective tissue disease (CTD) criteria has been recently defined as interstitial pneumonia with autoimmune features (IPAF). However, its long-term clinical course and outcome are poorly understood.

Methods: We included consecutive patients diagnosed with IIP (n = 586) or CTD-related interstitial lung disease (CTD-ILD, n = 149). Some patients with IIP were reclassified as IPAF based on recent guidelines.

Results: The median follow-up period was 45 months. Among the IIP patients, 109 (18.6%) were reclassified as IPAF. Compared to the non-IPAF-IIP group, the IPAF group had slower diffusing capacity of the lung for carbon monoxide (DLCO ) and total lung capacity declines, and more frequent CTD development during follow-up periods. The prognosis of the IPAF was better than that of the non-IPAF-IIP and similar to that of the CTD-ILD. IPAF was associated with better prognosis in the IIP cohort on univariate but not on multivariate analysis. Usual interstitial pneumonia (UIP) pattern, old age and low DLCO independently predicted mortality in the IPAF group.

Conclusion: Compared to the non-IPAF-IIP group, the IPAF group had slower lung function declines and more frequent CTD development during follow-up. Although the prognosis of IPAF group was better than that of non-IPAF-IIP group and similar to that of CTD-ILD group, it showed poor prognosis in patients with old age, UIP pattern, and low DLCO .

Keywords: connective tissue disease-associated interstitial lung disease; idiopathic interstitial pneumonia; interstitial pneumonia with autoimmune features; prognosis.

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References

REFERENCES

    1. Kinder BW, Collard HR, Koth L, Daikh DI, Wolters PJ, Elicker B, Jones KD, King TE Jr. Idiopathic nonspecific interstitial pneumonia: lung manifestation of undifferentiated connective tissue disease? Am. J. Respir. Crit. Care Med. 2007; 176: 691-7.
    1. Fischer A, West SG, Swigris JJ, Brown KK, du Bois RM. Connective tissue disease-associated interstitial lung disease: a call for clarification. Chest 2010; 138: 251-6.
    1. Vij R, Noth I, Strek ME. Autoimmune-featured interstitial lung disease: a distinct entity. Chest 2011; 140: 1292-9.
    1. Corte TJ, Copley SJ, Desai SR, Zappala CJ, Hansell DM, Nicholson AG, Colby TV, Renzoni E, Maher TM, Wells AU. Significance of connective tissue disease features in idiopathic interstitial pneumonia. Eur. Respir. J. 2012; 39: 661-8.
    1. Fischer A, Antoniou KM, Brown KK, Cadranel J, Corte TJ, du Bois RM, Lee JS, Leslie KO, Lynch DA, Matteson EL et al. An official European Respiratory Society/American Thoracic Society research statement: interstitial pneumonia with autoimmune features. Eur. Respir. J. 2015; 46: 976-87.

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