Antigen avoidance is critical for disease management of hypersensitivity pneumonitis (HP). Pharmacotherapy should be considered in cases where complete antigen avoidance is difficult or the causative antigen cannot be identified, but there are no established guidelines for its use in HP. Systemic corticosteroids are considered the mainstay of pharmacological treatment, since they seem to accelerate initial recovery. However, their use has little effect on the long-term outcome and should be reserved for patients with acute and rapidly worsening disease with a likely prominent inflammatory component. Steroid-sparing immunosuppressive drugs should be considered for longer-term management. Higher lymphocytosis in bronchoalveolar lavage has been associated with better response to treatment. Antifibrotic medications that target downstream fibrosis have a role in patients who progress despite these initial therapies. Although fibrotic HP and idiopathic pulmonary fibrosis share common clinical features and biopathology, anti-fibrotic drugs are considered second-line in the setting of progressive fibrotic HP disease. Due to the standardization of diagnosis based on recent clinical practice guidelines, further studies furnishing evidence for optimal pharmacotherapy of HP are expected.

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Steroid, Immunosuppressant, Anti-fibrotic Drug

  • Hiromi Tomioka

摘要

Antigen avoidance is critical for disease management of hypersensitivity pneumonitis (HP). Pharmacotherapy should be considered in cases where complete antigen avoidance is difficult or the causative antigen cannot be identified, but there are no established guidelines for its use in HP. Systemic corticosteroids are considered the mainstay of pharmacological treatment, since they seem to accelerate initial recovery. However, their use has little effect on the long-term outcome and should be reserved for patients with acute and rapidly worsening disease with a likely prominent inflammatory component. Steroid-sparing immunosuppressive drugs should be considered for longer-term management. Higher lymphocytosis in bronchoalveolar lavage has been associated with better response to treatment. Antifibrotic medications that target downstream fibrosis have a role in patients who progress despite these initial therapies. Although fibrotic HP and idiopathic pulmonary fibrosis share common clinical features and biopathology, anti-fibrotic drugs are considered second-line in the setting of progressive fibrotic HP disease. Due to the standardization of diagnosis based on recent clinical practice guidelines, further studies furnishing evidence for optimal pharmacotherapy of HP are expected.