Diagnosis and Treatment > Medication
Search for other papers by Shamaila Zaman in
Google Scholar
PubMed
Search for other papers by Bijal Patel in
Google Scholar
PubMed
Search for other papers by Paul Glynne in
Google Scholar
PubMed
Search for other papers by Mark Vanderpump in
Google Scholar
PubMed
Search for other papers by Ali Alsafi in
Google Scholar
PubMed
Search for other papers by Sairah Khan in
Google Scholar
PubMed
Search for other papers by Rashpal Flora in
Google Scholar
PubMed
Search for other papers by Fausto Palazzo in
Google Scholar
PubMed
Search for other papers by Florian Wernig in
Google Scholar
PubMed
Summary
Ectopic adrenocorticotropic hormone (ACTH) production is an uncommon cause of Cushing’s syndrome and, rarely, the source can be a phaeochromocytoma. A 55-year-old man presented following an episode of presumed gastroenteritis with vomiting and general malaise. Further episodes of diarrhoea, joint pains and palpitations followed. On examination, he was hypertensive with no clinical features to suggest hypercortisolaemia. He was subsequently found to have raised plasma normetanephrines of 3.98 nmol/L (NR <0.71) and metanephrines of 0.69 nmol/L (NR <0.36). An adrenal CT showed a 3.8 cm right adrenal nodule, which was not MIBG-avid but was clinically and biochemically consistent with a phaeochromocytoma. He was started on alpha blockade and referred for right adrenalectomy. Four weeks later, on the day of admission for adrenalectomy, profound hypokalaemia was noted (serum potassium 2.0 mmol/L) with non-specific ST-segment ECG changes. He was also diagnosed with new-onset diabetes mellitus (capillary blood glucose of 28 mmol/L). He reported to have gained weight and his skin had become darker over the course of the last 4 weeks. Given these findings, he underwent overnight dexamethasone suppression testing, which showed a non-suppressed serum cortisol of 1099 nmol/L. Baseline serum ACTH was 273 ng/L. A preliminary diagnosis of ectopic ACTH secretion from the known right-sided phaeochromocytoma was made and he was started on metyrapone and insulin. Surgery was postponed for 4 weeks. Following uncomplicated laparoscopic adrenalectomy, the patient recovered with full resolution of symptoms.
Learning points:
-
Phaeochromocytomas are a rare source of ectopic ACTH secretion. A high clinical index of suspicion is therefore required to make the diagnosis.
-
Ectopic ACTH secretion from a phaeochromocytoma can rapidly progress to severe Cushing’s syndrome, thus complicating tumour removal.
-
Removal of the primary tumour often leads to full recovery.
-
The limited literature suggests that the presence of ectopic Cushing’s syndrome does not appear to have any long-term prognostic implications.
Centre for Endocrine and Diabetes Sciences, University Hospital of Wales, Heath Park, Cardiff, UK
Search for other papers by J K Witczak in
Google Scholar
PubMed
Search for other papers by N Ubaysekara in
Google Scholar
PubMed
Search for other papers by R Ravindran in
Google Scholar
PubMed
Search for other papers by S Rice in
Google Scholar
PubMed
Search for other papers by Z Yousef in
Google Scholar
PubMed
Search for other papers by L D Premawardhana in
Google Scholar
PubMed
Summary
Graves’ disease is associated with tachydysrythmia, cardiac ischaemia and cardiomyopathy – all uncommon in young adults without previous cardiac disease. We present three young individuals who developed cardiac complications after periods of uncontrolled Graves’ disease. Subject 1: A 34-year-old female had severe thyrotoxic symptoms for weeks. Investigations showed fT4: 98.4 (11–25 pmol/L), fT3: 46.9 (3.1–6.8 pmol/L), TSH <0.01 (0.27–4.2 mU/L) and thyrotrophin receptor antibody (TRAb): 34.8 (<0.9 U//l). She had appropriate treatment but several weeks later she became breathless despite improving thyroid function. Echocardiography showed a pericardial effusion of 2.9 cm. She responded well to steroids and NSAIDs but developed active severe Graves’ orbitopathy after early total thyroidectomy. Subject 2: A 28-year-old male developed thyrotoxic symptoms (fT4: 38 pmol/L, fT3: 13.9 pmol/L, TSH <0.01 (for over 6 months) and TRAb: 9.3 U/L). One month after starting carbimazole, he developed acute heart failure (HF) due to severe dilated cardiomyopathy – EF 10–15%. He partially recovered after treatment – EF 28% and had early radioiodine treatment. Subject 3: A 42-year-old woman who had been thyrotoxic for several months (fT4: 54.3; fT3 >46.1; TSH <0.01; TRAb: 4.5) developed atrial fibrillation (AF) and heart failure. Echocardiography showed cardiomegaly – EF 29%. She maintains sinus rhythm following early total thyroidectomy (EF 50%). Significant cardiac complications may occur in previously fit young adults, who have had uncontrolled Graves’ disease for weeks to months. Cardiac function recovers in the majority, but early definitive treatment should be discussed to avoid Graves’ disease relapse and further cardiac decompensation.
Learning points:
-
Cardiac complications of Graves’ disease are uncommon in young adults without previous cardiac disease.
-
These complications may however occur if Graves’ disease had been poorly controlled for several weeks or months prior to presentation.
-
Persistent symptoms after adequate control should alert clinicians to the possibility of cardiac disease.
-
Specific treatment of Graves’ disease and appropriate cardiac intervention results in complete recovery in the majority and carries a good prognosis.
-
Early definitive treatment should be offered to them to prevent cardiac decompensation at times of further relapse.
