Pulmonary Disease and Critical Care· Topic 16.2 Occupational and environmental lung disease· 16.2.1

Hypersensitivity pneumonitis

Hypersensitivity pneumonitis is a mixed type III/IV immune reaction to repeated inhalation of an organic antigen in a sensitized host, producing noncaseating granulomas and bronchiolocentric inflammation. Removal of the offending antigen is the essential first step in treatment; glucocorticoids are added for significant hypoxemia or symptom burden, and unremitting exposure can progress to fibrotic lung disease indistinguishable from idiopathic pulmonary fibrosis.

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What this article covers

  • Overview

    An immune reaction to a repeatedly inhaled organic antigen in a sensitized host: hypersensitivity pneumonitis (HP, also called extrinsic allergic alveolitis) is a mixed type III (immune complex) and…

  • Epidemiology

    An uncommon form of interstitial lung disease (ILD) overall, but among the more common identifiable causes of ILD once a specific exposure is elicited.

  • Risk factors

    Occupational exposure to moldy organic dust, classically farming and grain handling (farmer's lung).

  • Pathophysiology

    Repetitive inhalation of a complex protein or low molecular weight antigen in a sensitized person triggers a combined immune-complex (type III) and delayed cell-mediated (type IV) hypersensitivity reaction in the small airways and alveoli.

  • Clinical features

    Acute HP is the most easily recognized form: fever, cough, dyspnea, chest tightness, chills, headache, and malaise (a flulike illness) beginning 4 to 12 hours after a large exposure to the inciting antigen.

  • Differential diagnosis

    Idiopathic pulmonary fibrosis: lower-lobe, peripheral reticular opacities with honeycombing in an older patient without an identifiable exposure, versus HP's upper/mid-lung centrilobular micronodules and a positive exposure history.

  • Diagnosis

    HP has no single confirmatory test; the diagnosis rests on a compatible antigen-exposure history plus characteristic HRCT, supported by BAL lymphocytosis with a normal or decreased CD4:CD8 ratio, and…

  • Management

    Remove the patient from the offending antigen first, in every case regardless of severity: this is the essential first step and directly lowers the risk of acute exacerbation and fibrotic progression.

  • Complications

    Progressive pulmonary fibrosis with traction bronchiectasis and honeycomb change, the most serious long-term consequence of unrecognized or continued exposure.

  • Prognosis

    Acute HP has a favorable prognosis when the antigen is identified and removed promptly, with symptoms resolving within about 48 hours and no lasting lung damage in most patients.

  • Prevention

    Complete (100%) elimination of the identified antigen is the primary preventive measure, not partial reduction, since a sensitized patient predictably relapses on any re-exposure to the causative antigen, even at low levels.

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