Case Presentation: A 46 year-old man with decompensated cirrhosis (MELD 45) presented with dizziness and confusion for two days. He denied any other new symptoms. Home medications included lactulose, propranolol, pantoprazole, folic acid and thiamine.
On arrival, the patient was normothermic (99.2 F), with bradycardia to 30-40 BPM and blood pressures of 80s/40s mmHg. He was severely jaundiced with mild asterixis, and was confused but oriented to person and place. Abdomen was nontender, and mildly distended; ultrasound demonstrated mild to moderate ascites without a tappable fluid pocket. EKG showed sinus bradycardia, and fingerstick glucose was 64 mg/dL. Labs were notable for creatinine of 2.6 mg/dL from 1.0, INR 4.2 from 2.8, and direct bilirubin 21 mg/dL from 18 one month prior. There was no leukocytosis, hemoglobin and platelets were at baseline, and lactate was 2.1 mg/dL.
The patient was given 2 liters of normal saline and 1 g/kg of albumin, as well as treated empirically for spontaneous bacterial peritonitis with ceftriaxone. When neither his bradycardia nor his hypotension improved, he was admitted to the medical ICU for undifferentiated shock. Glucagon was administered due to concern for beta blocker toxicity, without improvement. Atropine increased HR only to 40s-50s. On hospital day two, a cosyntropin stimulation test revealed adrenal insufficiency, and stress-dose steroids were initiated with improvement of heart rate to 70s and blood pressures to 130s/80s mmHg. Over time, he also responded well to midodrine for hepatorenal syndrome, with gradual improvement in his kidney function.
Discussion: RAI affects between 25 and 60% of patients with cirrhosis, regardless of etiology or stage of disease. Even in stable cirrhosis, RAI is a predictor of early mortality, and the presence of hepato-adrenal syndrome on hospital admission has been found to be associated with a higher probability of developing sepsis (27% versus 9%, P = 0.003), type 1 hepatorenal syndrome (16% versus 3%, P = 0.002), and death (22% versus 7%, P = 0.01). In light of the prevalence and prognostic implications of RAI in cirrhosis, some study authors have called for universal screening for the syndrome. There may also be a survival benefit to treating hepato-adrenal patients in septic shock with steroids, underscoring the need for prompt diagnosis and treatment of hepato-adrenal syndrome.
Conclusions: Relative adrenal insufficiency (RAI) is an important but often-overlooked prognostic indicator of morbidity and mortality in cirrhosis. RAI in decompensated cirrhosis is common, and has recently been named hepato-adrenal syndrome. It is critical that hepato-adrenal syndrome be considered when cirrhotic patients present with shock of unknown etiology, as prompt management may reduce mortality.