Outcomes of immunosuppressive therapy in chronic hypersensitivity pneumonitis

Adegunsoye, Ayodeji; Oldham, Justin M.; Pérez, Evans R. Fernández; Hamblin, Mark J.; Patel, Nina; Tener, Mitchell; Bhanot, Deepa; Robinson, Lacey B.; Bullick, Sam; Chen, Lena; Hsu, Scully; Churpek, Matthew M; Hedeker, Donald; Montner, Steven M.; Chung, Jonathan H.; Husain, Aliya N.; Noth, Imre; Strek, Mary E.; Vij, Rekha

doi:10.1183/23120541.00016-2017

Cited by 91 publications

(65 citation statements)

References 24 publications

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“…Identifying exposure to a ‘known cause’ of HP remains a key element, both in the diagnostic approach for patients with unexplained ILD and in the management of confirmed HP 4–8. Despite this, the optimal approach for establishing causation remains to be determined,9 with up to 63% of cases having no identifiable aetiological agent 10–17…”

Section: Introductionmentioning

confidence: 99%

Identifying causation in hypersensitivity pneumonitis: a British perspective

Barber

Burge²,

Feary

et al. 2019

BMJ Open Resp Res

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BackgroundEstablishing whether patients are exposed to a ‘known cause’ is a key element in both the diagnostic assessment and the subsequent management of hypersensitivity pneumonitis (HP).ObjectiveThis study surveyed British interstitial lung disease (ILD) specialists to document current practice and opinion in relation to establishing causation in HP.MethodsBritish ILD consultants (pulmonologists) were invited by email to take part in a structured questionnaire survey, to provide estimates of demographic data relating to their service and to rate their level of agreement with a series of statements. A priori ‘consensus agreement’ was defined as at least 70% of participants replying that they ‘Strongly agree’ or ‘Tend to agree’.Results54 consultants took part in the survey from 27 ILD multidisciplinary teams. Participants estimated that 20% of the patients in their ILD service have HP, and of these, a cause is identifiable in 32% of cases. For patients with confirmed HP, an estimated 40% have had a bronchoalveolar lavage for differential cell counts, and 10% a surgical biopsy. Consensus agreement was reached for 25 of 33 statements relating to causation and either the assessment of unexplained ILD or management of confirmed HP.ConclusionsThis survey has demonstrated that although there is a degree of variation in the diagnostic approach for patients with suspected HP in Britain, there is consensus opinion for some key areas of practice. There are several factors in clinical practice that currently act as potential barriers to identifying the cause for British HP patients.

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Section: Introductionmentioning

confidence: 99%

Identifying causation in hypersensitivity pneumonitis: a British perspective

Barber

Burge²,

Feary

et al. 2019

BMJ Open Resp Res

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“…56 Another study of 131 patients with CHP in which 93 received immunosuppressive therapy (prednisone: 44%; MMF: 35%; AZA: 21%), use of MMF or AZA was associated with a decreased incidence of treatment-emergent adverse events, and there was no difference in forced vital capacity (FVC) decline or survival when compared with therapy with prednisone alone. 57 On a case-by-cases basis, the duration of antiproliferative drug therapy can be extended beyond 6 or 12 months if the patient continues to demonstrate progressive clinical, physiological, and radiological improvement while off corticosteroids.…”

Section: Antiproliferative Drugsmentioning

confidence: 99%

Clinical Decision-Making in Hypersensitivity Pneumonitis: Diagnosis and Management

Pérez

Koelsch

Leone

et al. 2020

Semin Respir Crit Care Med

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This review provides an updated approach to the diagnosis and management of hypersensitivity pneumonitis (HP). The importance of using a multidisciplinary discussion to increase diagnostic and treatment confidence is emphasized. The role of Bayesian reasoning is highlighted throughout, underscoring the importance of hypothesis generation (differential diagnosis) and diagnostic test interpretation based on the probability of HP. Probability estimates of diagnostic certainty (i.e., a confident versus a working diagnosis) and treatment thresholds are carefully examined.Therapeutically, beyond antigen avoidance and newly available antifibrotic therapy for patients with a progressive fibrosing phenotype; the role, timing, and expected response to anti-inflammatory therapy in individual patients are unanswered questions. Since the evidence and validation of testing generally performed during the diagnostic work-up and longitudinal monitoring of HP is feeble at best, the viewpoints discussed are not intended to resolve current controversies but rather to provide a conceptual framework for evaluating discordant information when evaluating and caring for patients with HP.

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“…These treatments have been associated with reduced decline in FVC % pred, improved DLCO and transplant-free survival and a reduction in treatment-related AEs. Prospective randomised studies are needed to validate their effectiveness in these patients and to fully elucidate their role in the management of this disease [73,74].…”

Section: Hypersensitivity Pneumonitismentioning

confidence: 99%

Pharmacological management of progressive-fibrosing interstitial lung diseases: a review of the current evidence

Richeldi

Varone

Bergna³

et al. 2018

Eur Respir Rev

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A proportion of patients with interstitial lung diseases (ILDs) are at risk of developing a progressive-fibrosing phenotype, which is associated with a deterioration in lung function and early mortality. In addition to idiopathic pulmonary fibrosis (IPF), fibrosing ILDs that may present a progressive phenotype include idiopathic nonspecific interstitial pneumonia, connective tissue disease-associated ILDs, hypersensitivity pneumonitis, unclassifiable idiopathic interstitial pneumonia, ILDs related to other occupational exposures and sarcoidosis. Corticosteroids and/or immunosuppressive therapies are sometimes prescribed to patients with these diseases. However, this treatment regimen may not be effective, adequate on its own or well tolerated, suggesting that there is a pressing need for efficacious and better tolerated therapies. Currently, the only approved treatments to slow disease progression in patients with IPF are nintedanib and pirfenidone. Similarities in pathobiological mechanisms leading to fibrosis between IPF and other ILDs that may present a progressive-fibrosing phenotype provide a rationale to suggest that nintedanib and pirfenidone may be therapeutic options for patients with the latter diseases.This review provides an overview of the therapeutic options currently available for patients with fibrosing ILDs, including fibrosing ILDs that may present a progressive phenotype, and explores the status of the randomised controlled trials that are underway to determine the efficacy and safety of nintedanib and pirfenidone.

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Outcomes of immunosuppressive therapy in chronic hypersensitivity pneumonitis

Cited by 91 publications

References 24 publications

Identifying causation in hypersensitivity pneumonitis: a British perspective

Identifying causation in hypersensitivity pneumonitis: a British perspective

Clinical Decision-Making in Hypersensitivity Pneumonitis: Diagnosis and Management

Pharmacological management of progressive-fibrosing interstitial lung diseases: a review of the current evidence

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