An unusual cause of acute adrenal insufficiency.
J Tang, E Hui, A Abbara, A Ghaffar, D Papadopoulou, Z Win, K Meeran, K Wynne
Imperial Centre for Endocrinology
An 81 year old gentleman presented to the acute surgical team with severe constant epigastric pain radiating to his back. His only past medical history was of an oral squamous cell carcinoma radically treated 9years previously. He was on no regular medications and he did not consume alcohol. On examination, he was apyrexial and was very tender in the epigastrium, although not peritonitic. He had a one hour episode of sinus bradycardia (as low as 33bpm), with a differential blood pressure of 111/58 in the right arm and 153/84 in the left arm. His urea and electrolytes, thyroid function and amylase were all normal. He went on to have a CT scan of his chest and abdomen, which excluded an aortic aneurysm and did not demonstrate any evidence of pancreatitis. His intra-abdominal organs including adrenal glands were normal. He was treated with full ACS treatment for a possible inferior NSTEMI as a potential cause of bradycardia and abdominal pain, although his ECGs were normal and a12 hour troponin was negative. He went on to have an OGD, which demonstrated evidence of gastritis and he was discharged with a proton pump inhibitor therapy.
Two weeks later he re-presented with severe abdominal pain, predominantly in the left iliac fossa. He was haemodynamically stable (BP 130/57, pulse 82bpm). He had clinical and radiological evidence of a lower respiratory chest infection and his abdominal pain was felt to be secondary to diverticulitis for which he was started on antibiotics. He was noted to be hypoglycaemic the following morning with a BM recorded as 1.1mmol/l. His urea and electrolytes remained normal. A repeat CT abdomen demonstrated low density sub-acute expansion of the adrenal glands, which had developed since the previous CT scan. An MRI of his adrenal glands confirmed diffuse expansion with heterogenous signal, most consistent with evidence of recent adrenal haemorrhage.
A short synacthen test demonstrated primary adrenal failure (cortisol 139>>166>>175nmol/L, ACTH 50ng/l). Steroid replacement therapy with hydrocortisone was commenced and as he did not have a postural drop in blood pressure, he was not started on mineralocorticoid replacement. Other investigations including plasma renin, ANA, lupus anticoagulant, TB elispot and thrombophilia screen were all normal, although he was noted to have an IgA paraproteinaemia (13g/l) with a normal skeletal survey and bone profile most suggestive of MGUS.
The reported incidence of bilateral adrenal haemorrhage in general hospital autopsy studies is 0.14-1.8%, although it often remains undiagnosed during life (1). In contrast to autoimmune adrenalitis, mineralocorticoid function may be preserved. Major risk factors for adrenal haemorrhage include sepsis (especially meningococcal), operative stress, coagulopathy, including hypo or hyper-coagulable states (especially anti-phospholipid syndrome), malignancy and trauma, although it can rarely be idiopathic (2). Adrenal haemorrhage may represent a rare cause of an acute surgical abdomen. This gentleman has a number of contributory factors which may have led to his adrenal haemorrhage including sepsis, paraproteinaemia and the use of anticoagulants as part of his ACS treatment. This rare presentation must be bourne in mind especially whilst caring for hypotensive septic patients in the postoperative setting who exhibit signs consistent with hypoadrenalism.
(1) Xarli VP, Steele AA, Davis PJ. Adrenal hemorrhage in the adult. Medicine. 1978;57:211–221.
(2) Pictorial evolution of bilateral adrenal haemorrhage. Huelsen-Katz AM, Schouten BJ, Jardine DL, Soule SG, Liu H. Intern Med J2010 Jan;40(1):87-8.