The Drug-Induced Respiratory Disease Website

Alglucerase

Pulmonary arterial hypertension

May reverse with drug discontinuation. Generally similar to primary PHTn clinically, hemodynamically and on pathology. PMID 23997051. A few cases are thromboembolic in nature (PMID 24881082). Reviews at PMID 29508628, 30545968

Publications

Harats D, Pauzner R, Elstein D, Many A, Klutstein MW, Kramer MR, Farfel Z, Zimran A

Pulmonary hypertension in two patients with type I Gaucher disease while on alglucerase therapy.

Acta haematologica 1997;98;47-50 — 1997 — 47-50

Dawson A, Elias DJ, Rubenson D, Bartz SH, Garver PR, Kay AC, Bloor CM, Beutler E

Pulmonary hypertension developing after alglucerase therapy in two patients with type 1 Gaucher disease complicated by the hepatopulmonary syndrome.

Annals of internal medicine 1996 Dec 01;125;901-4 — 1996 Dec 01 — 901-4

Diagnosing DIRD

Drug and radiation history

Drug singularity - Correct identification of the drug

Consistent timing of exposure v. onset of symptoms

Clinical, imaging, BAL, pathological pattern consistent with the specific drug

Careful exlusion of another cause

Remission of symptoms with removal of drug

Recurrence with rechallenge (rarely advisable)

Causality assessment

Diagnosing DIRD

Identify causative drugs

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