Chronic Hypersensitivity Pneumonitis With a Usual Interstitial Pneumonia-Like Pattern

Chiba, Sahoko; Tsuchiya, Kimitake; Akashi, Takumi; Ishizuka, Masahiro; Okamoto, Tsukasa; Furusawa, Haruhiko; Tateishi, Tomoya; Kishino, Mitsuhiro; Miyazaki, Yasunari; Tateishi, Ukihide; Takemura, Tamiko; Inase, Naohiko

doi:10.1016/j.chest.2015.12.030

Cited by 70 publications

(62 citation statements)

References 20 publications

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“…Chiba et al 28 divided cases with a UIP pattern by the number of fibroblast foci per square centimeter and concluded that patients with fewer than 2.2 fibroblast foci/cm 2 did very well, with 70% survival at 100 months, whereas patients with more than 2.2 fibroblast foci/cm 2 had a median survival of 27 months. In a broad sense, that report is in accord with studies on UIP in which there was a survival difference by the number of fibroblast foci (eg, du Bois et al 48 ), but those reports usually do not show the type of dramatic difference described by Chiba et al, 28 and other authors have found no effect of numbers of fibroblast foci on survival. 49,50 The 2.2 fibroblast foci/cm 2 (which was the median in the Chiba et al 28 study) was a very small number of fibroblast foci, and the patients with more than 2.2 fibroblast foci/cm 2 had a median survival of 27 months.…”

Section: Relationship Of Pathologic Findings In Chp Tosupporting

confidence: 71%

“…Takemura et al 26 reported a remarkably good survival of 80% at 100 months for patient with CHPs with a UIP-like pattern, but they also recorded a median survival of 100 months for a comparison group of patients with UIP/ IPF, a survival that is not typical of UIP/IPF. Both the Chiba et al 28 and Takemura et al 26 studies raise a question of whether there is a subgroup of patients with CHP and a UIP-like pattern that do relatively well, whereas for most patients with a UIP-like pattern, it carries a poor prognosis.…”

Section: Relationship Of Pathologic Findings In Chp Tomentioning

confidence: 99%

“…Lymphoid aggregates are often present in CHP; Takemura et al 26 reported lymphoid aggregates in 17 of 22 CHP cases (72%) with a UIP pattern versus 5 of 13 UIP/IPF cases (38%), and Chiba et al 28 found lymphoid aggregates in 60% of a series of 16 cases (terminology in that article is confusing because the authors appear to be using the term follicles for aggregates.). Myers 29 explicitly noted that true lymphoid follicles can be found in CHP, but in our experience, they are uncommon and raise a question of connective tissue disease-associated interstitial lung disease (ILD) when present.…”

mentioning

confidence: 99%

“…Churg et al 27 found giant cells or granulomas in 16 of 18 cases (89%) with a UIPlike pattern and 3 of 3 cases (100%) with only peribronchiolar fibrosis, and Takemura et al 26 found giant cells in 15 of 22 cases (68%) and granulomas in 14 of 22 cases (64%). 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.…”

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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: Relationship Of Pathologic Findings In Chp Tosupporting

confidence: 71%

Section: Relationship Of Pathologic Findings In Chp Tomentioning

confidence: 99%

mentioning

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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“…Cellular Physiology and Biochemistry macrophages via the complement pathway [16]. These activated macrophages then release chemotactic factors, such as interleukin-8 (IL-8) and macrophage inflammatory protein-1α (MIP-1α), which consequentially induce the macrophages to proliferate at the inflammatory site.…”

Section: Cellular Physiology and Biochemistrymentioning

confidence: 99%

Associations of Pulmonary Fibrosis with Peripheral Blood Th1/Th2 Cell Imbalance and EBF3 Gene Methylation in Uygur Pigeon Breeder’s Lung Patients

Wu¹,

Luo²,

Pang³

et al. 2018

Cell Physiol Biochem

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Background/Aims Pigeon breeder’s lung (PBL) results from Th1/Th2 cell imbalance. B cells inhibit the immune activity of Th1, and EBF3 is a key B cell factor. This study explored the relationship between EBF3 and Th1/Th2 imbalance in chronic PBL cases complicated with pulmonary fibrosis (PF). Methods Twenty Uygur PBL+PF patients, 20 pigeon breeders without PBL or PF, and 20 healthy individuals without pigeon breeding history constituted the patient I, negative control, and normal control groups, respectively. Peripheral blood specimens and case backgrounds were collected between June 2016 and March 2017. EBF3 gene methylation was analyzed by matrix assisted laser desorption ionization-time of flight mass spectrometry. To compare different mechanisms of PF progression in PBL, samples from 20 Uygur PBL patients without PF (at acute and sub-acute stages) were collected between October 2017 and February 2018, constituting the patient II group. EBF3 mRNA expression was evaluated by real-time polymerase chain reaction. IFN-γ, IL-4 and IL-10 expression and Th1/Th2 imbalance in PBL were evaluated by enzyme-linked immunosorbent assay and flow cytometry. Results CpG-2 and general methylation rates in the patient I group were lower than those in the control groups (P˂0.017). The level of EBF3 mRNA expression in the patient I group was significantly higher than that in any other group. Compared with the control groups, the patient I group showed a significantly higher level of IL-4, whereas the patient II group showed a significantly lower level. IL-10 was also expressed more highly in the patient I group than in any other group (P< 0.01). Flow cytometry showed INF-γ dominance (Th1 cytokine) in PBL at the acute/sub-acute stage and IL-4 dominance (Th2 cytokine) at the chronic stage after PF occurred. The general methylation rate was negatively correlated with the mRNA level, with the latter being positively correlated with the IL-10 level and number of pigeons bred in the past 3 months. IL-4 expression was negatively correlated with INF-γ but positively correlated with PF area and duration of pigeon breeding history. Conclusions After PF occurs in chronic PBL, the inflammation type changes from Th1 dominance to Th2 dominance. During PBL development, IL-10 increases before IL-4 does, which may be associated with EBF3 hypomethylation and the involvement of B lymphocytes.

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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 With a Usual Interstitial Pneumonia-Like Pattern

Cited by 70 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?

Associations of Pulmonary Fibrosis with Peripheral Blood Th1/Th2 Cell Imbalance and EBF3 Gene Methylation in Uygur Pigeon Breeder’s Lung Patients

Clinical radiological and pathological staging of children with hypersensitivity pneumonitis

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