Hypersensitivity pneumonitis
- Overview
- Theory
- Diagnosis
- Management
- Follow up
- Resources
Treatment algorithm
Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups: see disclaimer
all patients
avoidance of antigen
Recommended in all patients.
Although there is limited information regarding effectiveness, clinically it is a sensible option.[6]Quirce S, Vandenplas O, Campo P, et al. Occupational hypersensitivity pneumonitis: an EAACI position paper. Allergy. 2016 Jun;71(6):765-79. http://onlinelibrary.wiley.com/doi/10.1111/all.12866/full http://www.ncbi.nlm.nih.gov/pubmed/26913451?tool=bestpractice.com [43]De Sadeleer LJ, Hermans F, De Dycker E, et al. Effects of corticosteroid treatment and antigen avoidance in a large hypersensitivity pneumonitis cohort: a single-centre cohort study. J Clin Med. 2019 Jan; 8(1): 14. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6352061 http://www.ncbi.nlm.nih.gov/pubmed/30577667?tool=bestpractice.com [67]Petnak T, Thongprayoon C, Baqir M, et al. Antigen identification and avoidance on outcomes in fibrotic hypersensitivity pneumonitis. Eur Respir J. 2022 Oct;60(4):2101336. https://publications.ersnet.org/content/erj/60/4/2101336 http://www.ncbi.nlm.nih.gov/pubmed/35236720?tool=bestpractice.com [68]Nishida T, Kawate E, Ishiguro T, et al. Antigen avoidance and outcome of nonfibrotic and fibrotic hypersensitivity pneumonitis. ERJ Open Res. 2022 Jan;8(1):00474-2021. https://pmc.ncbi.nlm.nih.gov/articles/PMC8819255 http://www.ncbi.nlm.nih.gov/pubmed/35141326?tool=bestpractice.com
The time it takes for symptoms to reduce is antigen-dependent, environment-dependent, and patient-dependent.
smoking cessation ± pulmonary rehabilitation ± supplemental oxygen
Treatment recommended for ALL patients in selected patient group
Patients who continue to smoke cigarettes should be counselled to quit.
Many patients will benefit from pulmonary rehabilitation programmes to improve their functional status and quality of life.[63]Dowman L, Hill CJ, May A, et al. Pulmonary rehabilitation for interstitial lung disease. Cochrane Database Syst Rev. 2021 Feb 1;2(2):CD006322. https://www.cochranelibrary.com/cdsr/doi/10.1002/14651858.CD006322.pub4/full http://www.ncbi.nlm.nih.gov/pubmed/34559419?tool=bestpractice.com [64]Rochester CL, Alison JA, Carlin B, et al. Pulmonary rehabilitation for adults with chronic respiratory disease: an official American Thoracic Society clinical practice guideline. Am J Respir Crit Care Med. 2023 Aug 15;208(4):e7-26. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10449064 http://www.ncbi.nlm.nih.gov/pubmed/37581410?tool=bestpractice.com
Severe hypoxaemia (PaO₂ ≤55 mmHg or oxygen saturation ≤89%) at rest or with exertion should be managed with supplemental oxygen. This is strongly recommended by the 2020 American Thoracic Society guideline on home oxygen therapy for adults with chronic lung disease.[65]Jacobs SS, Krishnan JA, Lederer DJ, et al. Home oxygen therapy for adults with chronic lung disease. An official American Thoracic Society clinical practice guideline. Am J Respir Crit Care Med. 2020 Nov 15;202(10):e121-41. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7667898 http://www.ncbi.nlm.nih.gov/pubmed/33185464?tool=bestpractice.com [Evidence C]1da311b4-fa5d-4612-a523-07652d21d38eguidelineCShould long-term oxygen be prescribed for adults with Interstitial Lung Disease (ILD) who have severe chronic resting room air hypoxaemia?[65]Jacobs SS, Krishnan JA, Lederer DJ, et al. Home oxygen therapy for adults with chronic lung disease. An official American Thoracic Society clinical practice guideline. Am J Respir Crit Care Med. 2020 Nov 15;202(10):e121-41. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7667898 http://www.ncbi.nlm.nih.gov/pubmed/33185464?tool=bestpractice.com
corticosteroid
Additional treatment recommended for SOME patients in selected patient group
Indications for corticosteroid treatment include persistent acute symptoms despite avoidance of antigen; moderate to severe respiratory impairment (e.g., hypoxaemia, lung function tests); and/or extensive lung involvement on imaging.
Patients with chronic fibrotic hypersensitivity pneumonitis (HP) are often given corticosteroids, for example, if there is evidence of inflammation such as bronchoalveolar lavage lymphocytosis or ground glass opacities.[43]De Sadeleer LJ, Hermans F, De Dycker E, et al. Effects of corticosteroid treatment and antigen avoidance in a large hypersensitivity pneumonitis cohort: a single-centre cohort study. J Clin Med. 2019 Jan; 8(1): 14. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6352061 http://www.ncbi.nlm.nih.gov/pubmed/30577667?tool=bestpractice.com [47]Costabel U, Miyazaki Y, Pardo A, et al. Hypersensitivity pneumonitis. Nat Rev Dis Primers. 2020 Aug 6;6(1):65. http://www.ncbi.nlm.nih.gov/pubmed/32764620?tool=bestpractice.com [57]Ejima M, Okamoto T, Suzuki T, et al. Efficacy of treatment with corticosteroids for fibrotic hypersensitivity pneumonitis: a propensity score-matched cohort analysis. BMC Pulm Med. 2021 Jul 19;21(1):243. https://bmcpulmmed.biomedcentral.com/articles/10.1186/s12890-021-01608-1 http://www.ncbi.nlm.nih.gov/pubmed/34281549?tool=bestpractice.com [58]De Sadeleer LJ, Hermans F, De Dycker E, et al. Impact of BAL lymphocytosis and presence of honeycombing on corticosteroid treatment effect in fibrotic hypersensitivity pneumonitis: a retrospective cohort study. Eur Respir J. 2020 Apr;55(4):1901983. https://publications.ersnet.org/content/erj/55/4/1901983
In patients with an acute presentation corticosteroids are given for 1-2 weeks, and can then usually be tapered off over about 6 weeks (alongside antigen avoidance). Patients with chronic fibrotic HP may require longer-term maintenance treatment. Follow your local protocols.
Primary options
prednisolone: 0.5 to 1 mg/kg/day orally initially, taper dose according to response
immunosuppressant
Additional treatment recommended for SOME patients in selected patient group
In patients with chronic fibrotic disease, an immunosuppressant such as azathioprine or mycophenolate may be started in patients with a suboptimal response to corticosteroids, or in patients who have a moderate to high corticosteroid dose requirement.[47]Costabel U, Miyazaki Y, Pardo A, et al. Hypersensitivity pneumonitis. Nat Rev Dis Primers. 2020 Aug 6;6(1):65. http://www.ncbi.nlm.nih.gov/pubmed/32764620?tool=bestpractice.com After starting the immunosuppressant, the corticosteroid dose is generally tapered, and then maintained at a low dose.
Observational studies suggest that immunosuppressants can improve lung function and reduce corticosteroid dose with patients with chronic or fibrotic hypersensitivity pneumonitis, but randomised clinical trials are needed.[59]Fiddler CA, Simler N, Thillai M, et al. Use of mycophenolate mofetil and azathioprine for the treatment of chronic hypersensitivity pneumonitis-a single-centre experience. Clin Respir J. 2019 Dec;13(12):791-4. http://www.ncbi.nlm.nih.gov/pubmed/31464073?tool=bestpractice.com [60]Casal A, Suárez-Antelo J, Gude F, et al. Use of mycophenolate mofetil for the treatment of fibrotic hypersensitivity pneumonitis. Am J Med Sci. 2025 Jan;369(1):24-34. http://www.ncbi.nlm.nih.gov/pubmed/39009283?tool=bestpractice.com [61]Terras Alexandre A, Martins N, Raimundo S, et al. Impact of Azathioprine use in chronic hypersensitivity pneumonitis patients. Pulm Pharmacol Ther. 2020 Feb;60:101878. http://www.ncbi.nlm.nih.gov/pubmed/31862300?tool=bestpractice.com [62]Morisset J, Johannson KA, Vittinghoff E, et al. Use of mycophenolate mofetil or azathioprine for the management of chronic hypersensitivity pneumonitis. Chest. 2017 Mar;151(3):619-25. https://pmc.ncbi.nlm.nih.gov/articles/PMC6026221 http://www.ncbi.nlm.nih.gov/pubmed/27816444?tool=bestpractice.com
Primary options
azathioprine: 1.5 to 2 mg/kg orally once daily, maximum 200 mg/day
OR
mycophenolate mofetil: 500-1500 mg orally twice daily
lung transplant
Additional treatment recommended for SOME patients in selected patient group
As for patients with other interstitial lung diseases, lung transplantation may be considered in eligible patients with progressive fibrotic disease.[1]Hamblin M, Prosch H, Vašáková M. Diagnosis, course and management of hypersensitivity pneumonitis. Eur Respir Rev. 2022 Feb 9;31(163):210169. https://publications.ersnet.org/content/errev/31/163/210169 http://www.ncbi.nlm.nih.gov/pubmed/35140104?tool=bestpractice.com [66]Kern RM, Singer JP, Koth L, et al. Lung transplantation for hypersensitivity pneumonitis. Chest. 2015 Jun;147(6):1558-65. https://pmc.ncbi.nlm.nih.gov/articles/PMC4451710 http://www.ncbi.nlm.nih.gov/pubmed/25412059?tool=bestpractice.com
Choose a patient group to see our recommendations
Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups. See disclaimer
Use of this content is subject to our disclaimer