Suspected pyroglutamic acidosis in a complex patient: A case report

We present the case of a 35-year-old female with multiple co-morbidities who developed unexplained high anion gap metabolic acidosis (HAGMA) despite marked improvement in sepsis parameters and other clinical conditions. Given the presence of known predisposing factors such as chronic therapeutic paracetamol (acetaminophen) use, chronic kidney disease, female gender, acute serious illness, undernutrition, and a dramatic improvement in acidosis following discontinuation of paracetamol without other overt explanations, we suspect acquired pyroglutamic acidosis (5-oxoproline acidosis) despite a lack of laboratory confirmation of 5-oxoproline. This case underlines the importance of recognizing this underdiagnosed cause of HAGMA.