No abstract

Pyroglutamic acidosis (PGA) is an underrecognized entity characterised by raised anion gap metabolic acidosis (RAGMA) and urinary hyper-excretion of pyroglutamic acid. It is frequently associated with chronic acetaminophen (APAP) ingestion. We report the case of a 73-year-old man with invasive pulmonary aspergillosis treated with voriconazole, and APAP for analgesia with a cumulative dose of 160g over 40 days. PGA was suspected as he developed severe RAGMA and which common causes were excluded. Diagnosis was confirmed by urinary organic acid analysis showing significant hyper-excretion of pyroglutamic acid. APAP was discontinued and N-acetylcysteine (NAC) was administered. His RAGMA rapidly resolved following treatment.