New clinical practice guidelines has been issued for treating hypersensitivity pneumonitis (HP) — an inflammatory lung disease triggered by allergens. The guidelines were issued by the American Thoracic Society, Asociación Latinoamericana de Tórax (ALAT), and the Japanese Respiratory Society, and come more than 30 years after the last guidance on the disease.

According to the Cleveland Clinic, hypersensitivity pneumonitis can be triggered by environmental allergens and causes inflammation of the lung tissue, which can lead to irreversible lung fibrosis over time.

HP can manifest as interstitial lung disease and is difficult to diagnose, the ATS says, adding that there has been little consensus in terms of disease definition, diagnostic criteria and diagnostic approach.

 “The clinician is often unable to distinguish features of fibrotic HP (f-HP) from those of idiopathic pulmonary fibrosis (IPF), and some patients meeting the  criteria for the diagnosis of IPF may in fact have f-HP with pulmonary fibrosis,” said Ganesh Raghu, MD, professor of medicine, University of Washington and director of the Center for Interstitial Lung Disease at University of Washington Medical center.  “The high rate of screen failures in patients participating in IPF clinical trials highlights this diagnostic challenge, as pulmonologists may be misdiagnosing patients with f-HP as having IPF, overlooking environmental factors that can contribute to the disease.” 

The guideline committee categorized HP into two clinical phenotypes, namely nonfibrotic and fibrotic HP, and made recommendations for reach. Their priority was to help clinicians make a confident and accurate diagnosis of HP. 



The following is a summary of the panel’s recommendations, which were formulated using the Grading of Recommendations, Assessment, Development, and Evaluation approach (GRADE):

Recommendation 1

For patients with newly identified ILD whose differential diagnosis includes non-fibrotic HP or fibrotic HP, the guideline committee makes no recommendation or suggestion for or against the use of a specific questionnaire to identify potential inciting agents of HP; instead, the guideline committee recommends the development and validation of a questionnaire. 

ATS: Pending the availability of a validated questionnaire, the guideline committee advocates that clinicians take a thorough history to identify potential exposures and sources in the patient’s environment that are known to be associated with HP.

Recommendation 2

For patients with newly identified ILD whose differential diagnosis includes non-fibrotic HP, the guideline committee suggests performing serum IgG testing that targets potential antigens associated with HP (suggestion, very low confidence in the estimated effects).

For patients with newly identified ILD whose differential diagnosis includes fibrotic HP, the guideline committee suggests performing serum IgG testing that targets potential antigens associated with HP (suggestion, very low confidence in the estimated effects).

Recommendation 3

For patients with newly identified ILD whose differential diagnosis includes non-fibrotic HP, the guideline committee recommends bronchoalveolar lavage with lymphocyte cellular analysis (recommendation, very low confidence in the estimated effects).

For patients with newly identified ILD whose differential diagnosis includes fibrotic HP, the guideline committee suggests bronchoalveolar lavage with lymphocyte cellular analysis (suggestion, very low confidence in the estimated effects).

Recommendation 4

For patients with newly identified ILD whose differential diagnosis includes non-fibrotic HP, the guideline committee suggests transbronchial forceps lung biopsy (suggestion, very low confidence in the estimated effects).

For patients with newly identified ILD whose differential diagnosis includes fibrotic HP, the guideline committee makes no recommendation or suggestion for or against transbronchial forceps lung biopsy.

Recommendation 5

For patients with newly identified ILD whose differential diagnosis includes non-fibrotic HP, the guideline committee makes no recommendation or suggestion for or against transbronchial cryobiopsy.

For patients with newly identified ILD whose differential diagnosis includes fibrotic HP, the guideline committee suggests transbronchial cryobiopsy (suggestion, very low confidence in estimated effects).

Recommendation 6

For patients with newly identified ILD whose differential diagnosis includes non-fibrotic HP, the guideline committee suggests surgical lung biopsy; this recommendation is intended for after alternative diagnostic options have been exhausted (suggestion, very low confidence in estimated effects).

For patients with newly identified ILD whose differential diagnosis includes fibrotic HP, the guideline committee suggests surgical lung biopsy; this recommendation is intended for after alternative diagnostic options have been exhausted (suggestion, very low confidence in estimated effects).



“These guidelines create a framework that we hope will standardize clinical care and facilitate research,” said Kevin C. Wilson, MD, professor of medicine at Boston University School of Medicine. Dr. Wilson also oversees development of clinical practice guidelines for the ATS. The ATS has published nearly 20 clinical practice guidelines on various conditions, ranging from allergy and asthma to TB, other pulmonary infections.