Chronic Hypersensitivity Pneumonitis (CHP), an Interstitial Lung Disease (ILD) with Distinct Molecular Signatures

2020 ◽  
Author(s):  
Haruhiko Furusawa ◽  
Jonathan H. Cardwell ◽  
Tsukasa Okamoto ◽  
Avram D. Walts ◽  
Iain R. Konigsberg ◽  
...  
Keyword(s):  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Hypersensitivity Pneumonitis ◽  
Molecular Signatures ◽  
Chronic Hypersensitivity Pneumonitis

scholarly journals Chronic Hypersensitivity Pneumonitis, an Interstitial Lung Disease with Distinct Molecular Signatures

2020 ◽  
Vol 202 (10) ◽  
pp. 1430-1444
Author(s):  
Haruhiko Furusawa ◽  
Jonathan H. Cardwell ◽  
Tsukasa Okamoto ◽  
Avram D. Walts ◽  
Iain R. Konigsberg ◽  
...  
Keyword(s):  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Hypersensitivity Pneumonitis ◽  
Molecular Signatures ◽  
Chronic Hypersensitivity Pneumonitis

scholarly journals Centrilobular Fibrosis in Fibrotic (Chronic) Hypersensitivity Pneumonitis, Usual Interstitial Pneumonia, and Connective Tissue Disease–Associated Interstitial Lung Disease

2020 ◽  
Vol 144 (12) ◽  
pp. 1509-1516
Author(s):  
Andrew Churg
Keyword(s):  
Interstitial Lung Disease ◽  
Connective Tissue ◽  
Lung Disease ◽  
Interstitial Pneumonia ◽  
Connective Tissue Disease ◽  
Hypersensitivity Pneumonitis ◽  
Interstitial Fibrosis ◽  
Usual Interstitial Pneumonia ◽  
Diffuse Fibrosis ◽  
Chronic Hypersensitivity Pneumonitis

Context.— Various pulmonary diseases can produce centrilobular (peribronchiolar) fibrosis, which may be isolated or associated with other patterns of more diffuse fibrosis. The major forms of interstitial lung disease in which centrilobular fibrosis is found are fibrotic (chronic) hypersensitivity pneumonitis, connective tissue disease–associated interstitial lung disease, and (a disputed issue) usual interstitial pneumonia/idiopathic interstitial fibrosis. Objective.— To review recent literature that addresses separation of these entities. Data Sources.— Data comprised recent publications. Conclusions.— In a specially constructed multidisciplinary discussion exercise, it was found that peribronchiolar metaplasia affecting more than half the bronchioles or more than 2 foci of peribronchiolar metaplasia per square centimeter of biopsy area was strongly associated with a confident diagnosis of fibrotic hypersensitivity pneumonitis. Giant cells or granulomas were only found in cases with a greater than 50% diagnostic confidence in hypersensitivity pneumonitis. Conversely, greater numbers of fibroblast foci per square centimeter and increasing measured amounts of subpleural fibrosis favored a diagnosis of usual interstitial pneumonia. Recent data also suggest that centrilobular fibrosis can be found in usual interstitial pneumonia, although the presence of centrilobular fibrosis statistically favors an alternate diagnosis. Connective tissue disease is a major confounder because many patterns are very similar to fibrotic hypersensitivity pneumonitis or usual interstitial pneumonia. Genetic abnormalities, such as the MUC5B minor allele overlap, in these conditions and at this point cannot be used for discrimination. Thus, the separation of fibrotic hypersensitivity pneumonitis and usual interstitial pneumonia remains a difficult problem. Accurate biopsy diagnosis of all of these diseases requires correlation with imaging and clinical findings, and is crucial for treatment.

scholarly journals Airway-specific autoantibodies identify a subset of patients with fibrotic interstitial lung disease

2021 ◽  
Author(s):  
Karim Boustani ◽  
Poonam Ghai ◽  
Rachele Invernizzi ◽  
Richard J. Hewitt ◽  
Toby M. Maher ◽  
...  
Keyword(s):  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Survival Probability ◽  
Hypersensitivity Pneumonitis ◽  
Healthy Controls ◽  
Systemic Autoimmunity ◽  
Autoimmune Pathology ◽  
Chronic Hypersensitivity Pneumonitis ◽  
Autoantibody Profiles

Fibrotic interstitial lung disease (fILD) has previously been associated with the presence of autoantibody. While studies have focused on systemic autoimmunity, the role of local autoantibodies in the airway remains unknown. We therefore extensively characterised the airway and peripheral autoantibody profiles in patients with fILD and assessed association with disease severity and outcome. Bronchoalveolar lavage (BAL) was collected from a cohort of fILD patients and total airway antibody concentrations were quantified. An autoantigen microarray was used to measure IgG and IgA autoantibodies against 124 autoantigens in BAL from 40 idiopathic pulmonary fibrosis (IPF), 20 chronic hypersensitivity pneumonitis (CHP), 20 connective tissue disease-associated ILD (CTD-ILD) patients and 20 controls. A subset of patients with fILD but not healthy controls had a local autoimmune signature in their airways that was not present systemically, regardless of disease. The proportion of patients with IPF with a local autoantibody signature was comparable to that of CTD-ILD, which has a known autoimmune pathology, identifying a potentially novel subset of patients. The presence of an airway autoimmune signature was not associated with reduced survival probability or changes in lung function in the cohort as a whole. Patients with IPF had increased airway total IgA and IgG1 while subjects with CHP had increased airway IgA, IgG1 and IgG4. In patients with CHP, increased airway total IgA was associated with reduced survival probability. The presence of airway autoantibodies identifies a unique subset of patients with fILD and the mechanisms by which these autoantibodies contribute to disease requires further investigation.

scholarly journals ‘When you hear hooves, think zebras, not horses’; two challenging cases of interstitial lung disease (ILD)

2019 ◽  
Vol 12 (2) ◽  
pp. bcr-2018-224507
Author(s):  
Marissa O’Callaghan ◽  
Aurelie Fabre ◽  
Michael Keane ◽  
Timothy J McDonnell
Keyword(s):  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Lung Fibrosis ◽  
Lung Diseases ◽  
Hypersensitivity Pneumonitis ◽  
Usual Interstitial Pneumonia ◽  
Case Series ◽  
Imaging Features ◽  
Exertional Dyspnoea ◽  
Multidisciplinary Team Meeting

Our case series describes two siblings with complex fibrosing lung diseases. The first patient was initially given a diagnosis of sarcoidosis based on imaging and exclusion of alternative diagnoses. A number of years after diagnosis, he had rapid deterioration of his disease and following surgical lung biopsy, his lung fibrosis was re-classified as chronic hypersensitivity pneumonitis (cHP) with a usual interstitial pneumonia pattern. He subsequently underwent successful lung transplantation. The second patient presented with rapidly progressing exertional dyspnoea. His bloods, imaging, bronchoalveolar lavage and histology were discussed at our multidisciplinary team meeting. His histology was most in keeping with subacute on cHP with overlapping imaging features between the two siblings. He was treated accordingly but unfortunately succumbed to his illness shortly after diagnosis. These cases highlight the difficulties differentiating between the various interstitial lung disease (ILD) subtypes and the challenges in management while also increasing awareness of familial ILD.

Transbronchial Biopsy and Cryobiopsy in the Diagnosis of Hypersensitivity Pneumonitis among Patients with Interstitial Lung Disease

2021 ◽  
Vol 18 (1) ◽  
pp. 148-161 ◽  
Author(s):  
Hassan A. Chami ◽  
Javier Diaz-Mendoza ◽  
Abigail Chua ◽  
Abhijit Duggal ◽  
Alex R. Jenkins ◽  
...  
Keyword(s):  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Hypersensitivity Pneumonitis ◽  
Transbronchial Biopsy

LUNG TRANSPLANTATION IN AN ADOLESCENT WITH PULMONARY FIBROSIS AS A RESULT OF INTERSTITIAL LUNG DISEASE (HYPERSENSITIVITY PNEUMONITIS)

2020 ◽  
Vol 99 (6) ◽  
pp. 287-293
Author(s):  
N.S. Lev ◽  
◽  
Y.L. Mizernitskiy ◽  
P.A. Shatokha ◽  
O.S. Groznova ◽  
...  
Keyword(s):  
Interstitial Lung Disease ◽  
Pulmonary Fibrosis ◽  
Lung Transplantation ◽  
Lung Disease ◽  
Children And Adolescents ◽  
Hypersensitivity Pneumonitis ◽  
Clinical Observation ◽  
Clinical Importance ◽  
Large Group ◽  
Chronic Course

Interstitial lung disease (ILD) is a large group of diseases with a severe course and, as a rule, an unfavorable prognosis, since irreversible pulmonary fibrosis forms in the outcome of most ILD. Among ILD in children and adolescents, hypersensitivity pneumonitis (HP) is of significant clinical importance. The clinical observation presented in the article demonstrates the first successful experience in our country (2018) of transplanting both lungs to an adolescent with pulmonary fibrosis resulting from the HP chronic course. Lung transplantation has become the only way to prolong the patient's life.

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