Use of Mycophenolate Mofetil or Azathioprine for the Management of Chronic Hypersensitivity Pneumonitis

Morisset, Julie; Johannson, Kerri A.; Vittinghoff, Eric; Aravena, Carlos; Elicker, Brett M.; Jones, Kirk D.; Fell, Charlene D.; Manganas, H.; Dubé, Bruno-Pierre; Wolters, Paul J.; Collard, Harold R.; Ryerson, Christopher J.; Ley, Brett

doi:10.1016/j.chest.2016.10.029

Cited by 198 publications

(154 citation statements)

References 21 publications

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“…Diffusion of carbon monoxide (DLCO) decline was not measured. Increase in DLCO in chronic HP can occur in response to treatment with no significant changes in FVC 10. Several patients claimed avoiding antigenic exposure without improvement.…”

Section: Discussionmentioning

confidence: 99%

Change in FVC and survival in chronic fibrotic hypersensitivity pneumonitis

Gimenez

Storrer

Kuranishi

et al. 2017

Thorax

138

104

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The predictive value of the decline in FVC by ≥10% on survival in patients with fibrotic hypersensitivity pneumonitis is unknown. Of 112 patients included, 66 (59%) had surgical lung biopsies. Patients with ≥10% decline in predicted FVC after 6-12 months had a significantly increased risk of all-cause mortality (median survival 53 months, 95% CI 37 to 69 vs 139 months, 95% CI 66 to 212 months, p=0.007). On multivariate analysis remained associated with increasing mortality: decline in FVC by ≥10% (HR 4.13, 95% CI 1.96 to 8.70, p=0.005), lower FVC% (HR 1.03, 95% CI 1.01 to 1.05, p=0.003) and with decreasing mortality improvement with antigen avoidance (HR 0.18, 95% CI 0.04 to 0.77, p=0.021).

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Section: Discussionmentioning

confidence: 99%

Change in FVC and survival in chronic fibrotic hypersensitivity pneumonitis

Gimenez

Storrer

Kuranishi

et al. 2017

Thorax

138

104

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“…However, in the current era, separation of UIP/IPF from CHP is crucial because the former is treated with antifibrotic agents (pirfenidone, nintedanib), 6 whereas CHP is treated with immunosuppressive agents. 7 The general definition of hypersensitivity pneumonitis (HP) includes exposure to a sensitizing antigen (sometimes called an inciting agent in the literature), and there is some evidence that removing a patient with CHP from antigen exposure improves survival, 8 although other reports deny that, 9,10 so correct diagnosis of CHP cases may be important to alert the clinician to look for a source of exposure. Patients with CHP have a considerably better prognosis after lung transplantation than do patients with UIP/IPF, apparently because patients with CHP are less susceptible to bronchiolitis obliterans.…”

mentioning

confidence: 99%

Pathology of Chronic Hypersensitivity Pneumonitis What Is It? What Are the Diagnostic Criteria? Why Do We Care?

Churg

Bilawich

Wright

2017

Archives of Pathology &Amp; Laboratory Medicine

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Context.— Chronic hypersensitivity pneumonitis (CHP) has emerged from obscurity during the past 15 years and is now recognized as a very common form of fibrosing interstitial pneumonia but one that is frequently misdiagnosed both clinically and on surgical lung biopsy as usual interstitial pneumonia/idiopathic pulmonary fibrosis (UIP/IPF) or fibrotic nonspecific interstitial pneumonia. Objective.— To review the pathologic features of CHP. Data Sources.— Clinical, pathology, and radiology literature were used. Conclusions.— Upper lobe–predominant fibrosis and/or air-trapping on computed tomography scan are features of CHP but not UIP/IPF; however, radiologic separation is possible in only about 50% of cases. Morphologically, CHP sometimes mimics UIP/IPF, but CHP often shows isolated foci of peribronchiolar (centrilobular) fibrosis, frequently associated with fibroblast foci, and in CHP, fibrosis may bridge from the centrilobular region to another bronchiole, an interlobular septum, or the pleura (“bridging fibrosis”). This set of findings is uncommon in UIP/IPF. In addition, CHP may produce a picture of fibrotic nonspecific interstitial pneumonia. Although giant cells/granulomas are usually present in subacute hypersensitivity pneumonitis, they are much less frequently found in CHP, and their absence does not contradict the diagnosis. This diagnostic separation is clinically important because CHP is treated differently than UIP/IPF is (immunosuppressive agents versus antifibrotic agents); further, there are some data to suggest that removing the patient from antigen exposure improves outcome, and there is evidence that patients with CHP have a much better survival prognosis after lung transplantation than do patients with UIP/IPF. In most cases, accurate diagnosis of CHP requires consultation among clinicians, radiologists, and pathologists.

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“…Treatment with MMF or AZA was associated with a statistically significant improvement in gas transfer values after one year of treatment in a recent study comparing the two regimens in patients with fibrotic HP [61]. More intensive immunosuppression has been used in cases of rapidly progressive non-IPF interstitial lung disease.…”

Section: Management Of Fibrotic Hpmentioning

confidence: 99%

Fibrotic Hypersensitivity Pneumonitis: Key Issues in Diagnosis and Management

Kouranos

Jacob

Nicholson

et al. 2017

JCM

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The diagnosis of hypersensitivity pneumonitis (HP) relies on the clinical evaluation of a number of features, including a history of significant exposure to potentially causative antigens, physical examination, chest CT scan appearances, bronchoalveolar lavage lymphocytosis, and, in selected cases, histology. The presence of fibrosis is associated with higher morbidity and mortality. Differentiating fibrotic HP from the idiopathic interstitial pneumonias can be a challenge. Furthermore, even in the context of a clear diagnosis of fibrotic HP, the disease behaviour can parallel that of idiopathic pulmonary fibrosis in a subgroup, with inexorable progression despite treatment. We review the current knowledge on the diagnosis, management, and prognosis of HP with particular focus on the fibrotic phenotype.

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Use of Mycophenolate Mofetil or Azathioprine for the Management of Chronic Hypersensitivity Pneumonitis

Abstract: Treatment with MMF or AZA is associated with improvements in Dlco in patients with cHP. Prospective randomized trials are needed to validate their effectiveness for cHP.

Cited by 198 publications

References 21 publications

Change in FVC and survival in chronic fibrotic hypersensitivity pneumonitis

Change in FVC and survival in chronic fibrotic hypersensitivity pneumonitis

Pathology of Chronic Hypersensitivity Pneumonitis What Is It? What Are the Diagnostic Criteria? Why Do We Care?

Fibrotic Hypersensitivity Pneumonitis: Key Issues in Diagnosis and Management

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