Diagnosis and Treatment of Fibrotic Hypersensitivity Pneumonia. Where We Stand and Where We Need to Go

Salisbury, M.L.; Myers, Jeffrey L.; Belloli, Elizabeth A.; Kazerooni, Ella A.; Martínez, Fernando J.; Flaherty, Kevin R.

doi:10.1164/rccm.201608-1675pp

Cited by 193 publications

(217 citation statements)

References 91 publications

(124 reference statements)

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“…There are basically 2 views: one is that CHP is defined by time since the appearance of symptoms (variously described in the literature as greater than 3 months, 4 months, or 1 year) and/or the pattern of disease presentation (''recurrent,'' ''insidious,'' versus ''acute''); the other is that CHP is defined only by the presence of fibrosis either on imaging or from biopsy (reviewed in Salisbury et al 1 and Spagnolo et al 13 ). In our view, the evidence is clear (see Relationship of Pathologic Findings in CHP to Prognosis below) that the presence of fibrosis is associated with a considerably worse prognosis compared with patients with HP who do not have fibrosis (under this definition, the latter are labeled as having acute or subacute CHP, depending on presentation), and we will use CHP here to indicate only HP cases with fibrosis.…”

Section: Definition Of Chpmentioning

confidence: 99%

“…1 In current practice, whether a patient has CHP is a common, multidisciplinary discussion, and one about which there is often considerable disagreement. In a formal study, Walsh et al 2 asked 7 experienced multidisciplinary discussion groups, comprising clinicians, radiologists, and pathologists, to review the same 70 cases and examined the level of agreement.…”

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confidence: 99%

“…A major part of the problem in recognizing CHP is that many of these patients (at least 50% but probably more 1 ) have a fibrotic lung disease without a recognizable antigen exposure. Detection of circulating immunoglobulin G against specific antigens may or may not reveal the sensitizing agent, and the same is true of inhalation-challenge testing, which is performed in only a few laboratories and which may not produce a physiologic reaction in CHP.…”

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confidence: 99%

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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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Section: Definition Of Chpmentioning

confidence: 99%

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confidence: 99%

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confidence: 99%

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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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“…However, making the diagnosis of HP remains challenging due to variability of clinical findings and lack of consensus criteria [15–17]. A history of exposure has been shown to be the most powerful determinant of an HP diagnosis, which requires a thoughtful approach that may span multiple patient encounters [18, 19].…”

Section: Introductionmentioning

confidence: 99%

“…Two recent papers reached different conclusion in this regard [15, 23]. However, in patients without a clearly identifiable fibrogenic antigen or an HRCT that is nondiagnostic, additional invasive testing is required [24].…”

Section: Introductionmentioning

confidence: 99%

Utility of Bronchoalveolar Lavage and Transbronchial Biopsy in Patients with Hypersensitivity Pneumonitis

Adams

Newton

Batra

et al. 2018

Lung

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Lentil aspiration leading to likely hypersensitivity pneumonitis

Dhochak

Jat

Lodha

et al. 2019

Pediatric Pulmonology

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Rationale A variety of inhaled antigens have been implicated to cause hypersensitivity pneumonitis (HP). We observed that children force‐fed with lentil‐based weaning food had persistent respiratory symptoms and radiology similar to HP. Objectives To describe the clinical features of lentil HP. Methods We conducted a retrospective review of records of children with lentil HP attending Pediatric Chest Clinic at a tertiary care hospital in North India from 2008‐2018. We included case records with elevated immunoglobulin G (IgG) specific for lentil antigen. Measurements and Main Results Nine children (seven boys) were identified with median (IQR) age of onset of symptoms and diagnosis at 9 (6, 12) and 11 (10, 16) months, respectively. Chronic cough (100%), shortness of breath (89%), fever (78%), vomiting (56%), and wheezing (33%) were common symptoms. Fine crackles were heard in 33% of children, none had clubbing. CT scans showed nodular opacities and consolidation in 78% and 67% children, respectively. Bronchoalveolar lavage showed increased neutrophils and lymphocytes (67% and 33%, respectively). All children showed rapid remission with systemic steroids (prednisolone), starting at a median dose of 1 (1, 1.1) mg kg−1 day−1. One child had a clinical relapse which was treated with oral steroids again. IgG specific to lentil antigens was elevated in children with lentil HP (21‐>200 mgA/L) compared with children with other chronic respiratory illnesses (n = 7, <2‐11.4 mgA/L). Conclusions Lentil aspiration is an important cause of HP in infants of weaning age with force‐feeding practices. Further studies are needed to identify aspirated antigens which cause HP in aspiration prone children.

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Diagnosis and Treatment of Fibrotic Hypersensitivity Pneumonia. Where We Stand and Where We Need to Go

Cited by 193 publications

References 91 publications

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

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

Utility of Bronchoalveolar Lavage and Transbronchial Biopsy in Patients with Hypersensitivity Pneumonitis

Lentil aspiration leading to likely hypersensitivity pneumonitis

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