- Author:
Paolo Maria LEONE
1
;
Luca RICHELDI
Author Information
- Publication Type:Review
- Keywords: Hypersensitivity Pneumonitis; Lung Diseases, Interstitial; Fibrosis; Diagnosis; Management; High Resolution Computed Tomography; Therapy; Steroids; Antigens; Nintedanib; Pirfenidone
- From:Tuberculosis and Respiratory Diseases 2020;83(2):122-131
- CountryRepublic of Korea
- Language:English
- Abstract: Hypersensitivity Pneumonitis (HP) one of the most common interstitial lung diseases (ILDs) is characterized by exposure to an inhaled inciting antigen that leads to a host immunologic reaction determining interstitial inflammation and architectural distortion. The underlying pathogenetic mechanisms are unclear. The absence of international shared diagnostic guidelines and the lack of a “gold-standard” test for HP combined with the presence of several clinical and radiologic overlapping features makes it particularly challenging to differentiate HP from other ILDs, also in expert contests. Radiology is playing a more crucial role in this process; recently the headcheese sign was recognized as a more specific for chronic-HP than the extensive mosaic attenuation. Several classification proposals and diagnostic models have been advanced by different groups, with no prospective validation. Therapeutic options for HP have been limited to antigen avoidance and immunosuppressant drugs over the last decades. Several questions about this condition remain unanswered and there is a need for more studies.