Pathologic Findings and Prognosis in a Large Prospective Cohort of Chronic Hypersensitivity Pneumonitis

Wang, Ping; Jones, Kirk D.; Urisman, Anatoly; Elicker, Brett M.; Urbania, Thomas H.; Johannson, Kerri A.; Assayag, Deborah; Lee, Joyce; Wolters, Paul J.; Collard, Harold R.; Koth, Laura L.

doi:10.1016/j.chest.2017.02.011

Cited by 147 publications

(139 citation statements)

References 20 publications

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“…Churg et al 27 reported that a fibrotic NSIP picture is less common than a UIP-like picture (4 of 25 cases Many of these cases are not morphologically separable from idiopathic, fibrotic NSIP, but the presence of giant cells and granulomas or Schaumann bodies ( Figure 5) favors CHP; Wang et al 32 found them in 68% of their fibrotic NSIPpattern cases, and Churg et al 27 observed them in 100% (4 of 4) of such cases. The interstitial inflammatory infiltrate may be sparse or may be composed of lymphocytes, plasma cells, and a few eosinophils, and lymphoid aggregates with or without germinal centers may be present.…”

Section: Chronic Hp: Nsip-like Fibrosismentioning

confidence: 99%

“…Only 2 reports describe the outcome in patients with purely peribronchiolar fibrosis: Churg et al 27 found a median survival of 144 months, but Wang et al 32 found a median survival of 60 months, only minimally (and not statistically) better than their patients with UIP patterns. Both those reports had few patients, so those conclusions may be tenuous, but it is hard to understand why pure centrilobular fibrosis, which is probably the earliest lesion of CHP, should lead to the same poor prognosis as a UIP picture.…”

Section: Relationship Of Pathologic Findings In Chp Tomentioning

confidence: 99%

“…Table 3 summarizes the literature using reports where outcome by specific pathologic pattern was indicated and standard definitions have been adhered to (the NSIP and ''typical'' patterns described by Gaxiola et al 43 are excluded because the authors indicated that they included cases with and without fibrosis in those categories). Four reports 27,32,33,43 indicated that CHP with a UIP pattern had a poor prognosis, with median survivals of 36 to 50 month, and Trahan et al 35 reported survivals of 9 months each for 2 patients whose biopsies showed only a UIP pattern. All those numbers were similar to the survival rates for UIP/IPF.…”

Section: Relationship Of Pathologic Findings In Chp Tomentioning

confidence: 99%

“…Wang et al 32 reported that 100% of their CHP cases with a UIP pattern had fibroblast foci, and suggest that the frequency of fibroblast foci is greater in cases with a UIP pattern than in cases with only peribronchiolar fibrosis, in which they found fibroblast foci in 30%.…”

mentioning

confidence: 99%

“…However, Chiba et al 28 described giant cells or granulomas in only 4 of 16 UIP-like cases (25%). Wang et al 32 found them in 2 of 7 UIP-like cases (28%), but in almost all cases with only peribronchiolar fibrosis. These data are somewhat difficult to interpret because, in the past, giant cells and granulomas tended to be viewed as requirements for the pathologic diagnosis of CHP, but that is no longer true, so that case-selection criteria probably changed over time.…”

mentioning

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: Chronic Hp: Nsip-like Fibrosismentioning

confidence: 99%

Section: Relationship Of Pathologic Findings In Chp Tomentioning

confidence: 99%

Section: Relationship Of Pathologic Findings In Chp Tomentioning

confidence: 99%

mentioning

confidence: 99%

mentioning

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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Clinical radiological and pathological staging of children with hypersensitivity pneumonitis

Özsezen

Özcan

Emiralioğlu

et al. 2022

Pediatric Pulmonology

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Objective: Hypersensitivity pneumonitis (HP) is rare in the pediatric population. To date, there are no studies defining a correlation between clinical, radiological, and pathological findings in children with HP. The objective of this study is to define the clinical, and radiological characteristics and prognosis of childhood HP and to examine the clinical, radiological, and pathological correlation between HP stages.Methods: Patients with suspected HP and followed at two tertiary care hospitals between 2000 and 2020 were retrospectively evaluated. Computed tomography (CT) of the chest of patients was evaluated by a single radiologist. The interagreement between clinical and radiological severity of the patients was calculated with the κ test.Results: Fourteen children with suspected HP were identified. The results of 10 patients with the definitive diagnosis were as follows: one patient (10%) had acute, five patients (50%) had subacute, and four patients (40%) had chronic HP. The most prominent findings in chest CT were hilar, or hilar and subcarinal lymphadenopathy (80%), centrilobular nodules (60%), patchy or diffuse ground-glass opacities (50%), and cysts (50%). The interagreement between clinical and radiological severity of the patients was 100% (approximate significance: 0.003). The diagnosis of four patients with suspected HP who were unresponsive to standardized medical treatments or developed multisystem involvement was diagnosed with other diseases. One patient (10%) with definitive chronic HP died due to respiratory failure during follow up.Conclusion: Similar to adult HP, the prognosis is worse in children with existing fibrotic equivalents in chest CT. Patients who are not responding to standard medical treatments or develop multisystem involvement should be evaluated for other lung diseases.

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Chronic Hypersensitivity Pneumonitis

Ravaglia

Poletti

2019

Transbronchial Cryobiopsy in Diffuse Parenchymal Lung Disease

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Pathologic Findings and Prognosis in a Large Prospective Cohort of Chronic Hypersensitivity Pneumonitis

Cited by 147 publications

References 20 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?

Clinical radiological and pathological staging of children with hypersensitivity pneumonitis

Chronic Hypersensitivity Pneumonitis

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