Case Presentation:
A 58 year old woman with a history of hypothyroidism on levothyroxine was directly admitted to our hospital after seeing her gastroenterologist with nausea, vomiting, weakness, and weight loss. She had a 6 month history of progressive weakness and intermittent episodes of emesis associated with palpitations and presyncopal episodes including an admission to an outside hospital one month before presentation with a normal CT abdomen/pelvis, HIDA scan, abdominal ultrasound, and EGD. On physical exam, her blood pressure was 100/60, no lymphadenopathy was appreciated, and her strength was decreased in her legs, particularly the proximal muscles. Extensive laboratory testing revealed a sodium of 131, potassium of 4.5, free T4 of 1.5, cortisol stimulation test with results of <1.5, < 1.5, and <1.5 at baseline and 30 and 60 minutes after infusion of cosyntropin, respectively. Further testing revealed a markedly elevated level of 21‐hydroxylase antibodies as well as elevated thyroperoxidase antibodies consistent with a diagnosis of profound primary adrenal insufficiency as part of the Autoimmune Polyglandular Syndrome, Type II (APS‐2), also known as Schmidt syndrome. Prior to discharge, patient was started on oral prednisone and her strength improved significantly.
Discussion:
Autoimmune adrenal insufficiency (Addison’s disease) is a rare disease found in 30 to 140 per million people in Western countries. APS‐2 is the most common polyglandular autoimmune syndrome usually presenting as adrenal insufficiency with hypothyroidism or type I diabetes mellitus. The pathogenesis of APS‐2 involves antibodies against steroidogenic enzymes (most commonly 21‐hydroxylase) on top of a genetic predisposition. Patients rarely present until over 90 % of adrenal cortex has been destroyed. Hospitalists often care for patients with autoimmune thyroid disease, but we rarely consider the diagnosis of concomitant adrenal insufficiency. While generalized weakness is one of the most common and nonspecific presenting symptoms encountered as a Hospitalist, proximal muscle weakness provides a more defined differential.
Conclusions:
This case highlights the importance of including endocrinopathies on the differential in patients with apparent GI illness and neurologic findings. Had such a consideration been made previously, this patient would not have suffered for as many months. While advanced radiologic testing (CT scans, ultrasounds) and endoscopies are often very useful, sometimes a careful review of the past history, a thorough exam, as well as pertinent laboratory studies can yield a diagnosis much sooner. In conclusion, this case serves to remind Hospitalists to consider adrenal insufficiency in the differential in patients with a myriad of presenting signs and symptoms.