Severe distributive shock, neutrophilic dermatosis, and ST-elevation myocardial infarction in the setting of azathioprine hypersensitivity syndrome.

Background: Azathioprine is a purine synthesis inhibitor used as an immunosuppressive therapy for many immune-mediated diseases. Azathioprine hypersensitivity reaction is a rare, life-threatening adverse reaction characterized by a range of multisystem manifestations including fever, abdominal pain, arthralgias, erythematous cutaneous eruption, acute renal failure, neutrophilia, and more rarely, distributive shock. Although acute heart failure has been rarely described in association with azathioprine hypersensitivity syndrome, myocardial infarction has, to our knowledge, never been associated with this entity.

Methods: We describe a case of a 59-year-old male with Crohn's disease who developed severe azathioprine hypersensitivity syndrome that included distributive shock, neutrophilic dermatosis, and acute coronary syndrome with ST-elevation. Clinical improvement was seen after cessation of azathioprine and administration of glucocorticoid therapy.

Conclusions: Prompt recognition of azathioprine hypersensitivity syndrome, which can manifest as shock and neutrophilic dermatosis, is key to ensure rapid azathioprine cessation.