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. 2022 Nov 12;12(11):2767.
doi: 10.3390/diagnostics12112767.

Factors Predictive for Immunomodulatory Therapy Response and Survival in Patients with Hypersensitivity Pneumonitis-Retrospective Cohort Analysis

Katarzyna B Lewandowska  1 , Inga Barańska  2 , Małgorzata Sobiecka  1 , Piotr Radwan-Rohrenschef  1 , Małgorzata Dybowska  1 , Monika Franczuk  3 , Adriana Roży  4 , Agnieszka Skoczylas  5 , Iwona Bestry  2 , Jan Kuś  1 , Witold Z Tomkowski  1 , Monika Szturmowicz  1
Affiliations

Factors Predictive for Immunomodulatory Therapy Response and Survival in Patients with Hypersensitivity Pneumonitis-Retrospective Cohort Analysis

Katarzyna B Lewandowska et al. Diagnostics (Basel). .

Abstract

Hypersensitivity pneumonitis (HP) is one of the interstitial lung diseases with clearly established diagnostic criteria. Nevertheless, pharmacologic treatment recommendations are still lacking. Most specialists use steroids as first-line drugs, sometimes combined with an immunosuppressive agent. Aim: The aim of the present retrospective study was to establish predictive factors for treatment success and survival advantage in HP patients. Methods: We analyzed the short-term treatment outcome and overall survival in consecutive HP patients treated with prednisone alone or combined with azathioprine. Results: The study group consisted of 93 HP patients, 54 (58%) with fibrotic HP and 39 (42%) with non-fibrotic HP. Mean (± SD) VCmax % pred. and TL,co % pred. before treatment initiation were 81.5 (±20.8)% and 48.3 (±15.7)%, respectively. Mean relative VCmax and TL,co change after 3−6 months of therapy were 9.5 (±18.8)% and 21.4 (±35.2)%, respectively. The short-term treatment outcomes were improvement in 49 (53%) patients, stabilization in 16 (17%) patients, and progression in 28 (30%) patients. Among those with fibrotic HP, improvement was noted in 19 (35%) cases. Significant positive treatment outcome predictors were fever after antigen exposure, lymphocyte count in broncho-alveolar lavage fluid (BALF) exceeding 54%, RV/TLC > 120% pred., and ill-defined centrilobular nodules in high-resolution computed tomography (HRCT). An increased eosinophil count in BALF and fibrosis in HRCT were significant negative treatment outcome predictors. The presence of fibrosis in HRCT remained significant in a multivariate analysis. A positive response to treatment, as well as preserved baseline VCmax (% pred.) and TLC (% pred.), predicted longer survival, while fibrosis in HRCT was related to a worse prognosis. Conclusion: Immunomodulatory treatment may be effective in a significant proportion of patients with HP, including those with fibrotic changes in HRCT. Therefore, future trials are urgently needed to establish the role of immunosuppressive treatment in fibrotic HP.

Keywords: hypersensitivity pneumonitis; lung fibrosis; pulmonary function tests; treatment.

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Conflict of interest statement

The authors declare no conflict of interest.

Figures

Figure 1
Figure 1
Kaplan–Meier survival curves in three different treatment outcome group: 0—progression, 1—stable disease, 2—improvement.
Figure 2
Figure 2
Kaplan–Meier survival curves according to the HRCT fibrosis score.
See this image and copyright information in PMC

References

    1. Raghu G., Remy-Jardin M., Ryerson C.J., Myers J.L., Kreuter M., Vasakova M., Bargagli E., Chung J.H., Collins B.F., Bendstrup E., et al. Diagnosis of Hypersensitivity Pneumonitis in Adults. An Official ATS/JRS/ALAT Clinical Practice Guideline. Am. J. Respir. Crit. Care Med. 2020;202:e36–e69. doi: 10.1164/rccm.202005-2032ST. - DOI - PMC - PubMed
    1. Jędrych M.E., Szturmowicz M., Bestry I., Kuś J. Hypersensitivity pneumonitis: Diagnostic criteria, treatment, prognosis and prevention. Med. Pr. 2016;67:517–527. doi: 10.13075/mp.5893.00406. - DOI - PubMed
    1. Vasakova M., Selman M., Morell F., Sterclova M., Molina-Molina M., Raghu G. Hypersensitivity Pneumonitis: Current Concepts of Pathogenesis and Potential Targets for Treatment. Am. J. Respir. Crit. Care Med. 2019;200:301–308. doi: 10.1164/rccm.201903-0541PP. - DOI - PubMed
    1. Walsh S.L.F., Sverzellati N., Devaraj A., Wells A.U., Hansell D.M. Chronic hypersensitivity pneumonitis: High resolution computed tomography patterns and pulmonary function indices as prognostic determinants. Eur. Radiol. 2012;22:1672–1679. doi: 10.1007/s00330-012-2427-0. - DOI - PubMed
    1. Szturmowicz M., Barańska I., Skoczylas A., Jędrych M.E., Demkow U. Correlation of bronchoalveolar lavage lymphocyte count with the extent of lung fibrosis and with plethysmographic lung volumes in patients with newly recognized hypersensitivity pneumonitis. Cent. Eur. J. Immunol. 2020;45:276–282. doi: 10.5114/ceji.2020.101246. - DOI - PMC - PubMed