Endocrine Abstracts

Primary adrenal insufficiency, Addison’s disease, is a rare endocrine disorder. Early diagnosis is often difficult and the presentation is commonly only recognised after a life threatening adrenal crisis. Fortunately, with appropriate early treatment further complications are uncommon in the acute setting. This case concerns a 19-year-old man who presented with lethargy, dyspnoea on exertion and a one month history of hyperpigmentation. He had a history of nocturnal enuresis for which he was taking desmopressin. On examination he had tanned skin, buccal pigmentation and borderline hypotension. Admission bloods revealed hyponatraemia and hyperkalaemia. After establishing a working diagnosis of first presentation with Addison’s disease, treatment was started including fluid resuscitation and intravenous hydrocortisone. The diagnosis of primary adrenal insufficiency was supported by inadequate response on short synacthen test and positive adrenal cortex antibodies. Two days after admission he developed sudden onset respiratory distress and hypoxia. Bi-basal crepitations were present. CTPA reported bilateral predominantly central ground glass and confluent opacities with no evidence of pulmonary embolus. Of note there was CT evidence of right heart strain. An echocardiogram showed good systolic function with estimated ejection fraction 60–65%. Pulmonary artery pressures were elevated. COVID-19 throat swab was negative. Troponin was mildly elevated though ECG showed sinus tachycardia with no ischaemic changes. He rapidly improved with CPAP for several days. Further endocrine investigations confirmed the diagnosis of primary adrenal failure (0900 h ACTH levels 688 ng/l (reference range <30 ng/l), aldosterone levels <60 pmol/l (reference range 90–700 pmol/l), grossly atrophic adrenals on CT). He was successfully converted to oral therapy prior to discharge. The reason he developed acute respiratory distress syndrome (ARDS) following treatment of adrenal insufficiency remains unknown. A literature review conducted to identify links between cases of Addison’s disease and ARDS reports few cases. One (1) presented a very similar, though more severe, history and course of illness. For our patient, previous desmopressin use may have been a contributor, exacerbating the rapid changes in fluid balance as steroid deficiency was corrected. This case highlights a rare, though potentially life-threatening, complication of acute adrenal crisis and the necessity for increased awareness of such cases among treating physicians.

References

1. Wolff, B., Machill, K., Schulzki, I., Schumacher, D. and Werner, D., 2007. Acute reversible cardiomyopathy with cardiogenic shock in a patient with Addisonian crisis: A case report. International Journal of Cardiology, 116(2), pp.e71–e73.