Browse

You are looking at 1 - 10 of 37 items

Mawson Wang Department of Endocrinology and Diabetes, Blacktown Hospital, Sydney, Australia
Blacktown Clinical School, School of Medicine, Western Sydney University, Sydney, Australia

Search for other papers by Mawson Wang in
Google Scholar
PubMed
Close
,
Benjamin Jonker Department of Neurosurgery, St. Vincent’s Hospital, Sydney, Australia

Search for other papers by Benjamin Jonker in
Google Scholar
PubMed
Close
,
Louise Killen Department of Pathology, St. Vincent’s Hospital, Sydney, Australia

Search for other papers by Louise Killen in
Google Scholar
PubMed
Close
,
Yvonne Bogum NSW Health Pathology East, Prince of Wales Hospital, Sydney, Australia

Search for other papers by Yvonne Bogum in
Google Scholar
PubMed
Close
,
Ann McCormack Department of Endocrinology, St. Vincent’s Hospital, Sydney, Australia
Garvan Institute of Medical Research, Sydney, Australia
St. Vincent’s Clinical School, Faculty of Medicine, UNSW Sydney, Sydney, Australia

Search for other papers by Ann McCormack in
Google Scholar
PubMed
Close
, and
Ramy H Bishay Department of Endocrinology and Diabetes, Blacktown Hospital, Sydney, Australia
Blacktown Clinical School, School of Medicine, Western Sydney University, Sydney, Australia

Search for other papers by Ramy H Bishay in
Google Scholar
PubMed
Close

Summary

Cushing’s disease is a rare disorder characterised by excessive cortisol production as a consequence of a corticotroph pituitary tumour. While the primary treatment is surgical resection, post-operative radiation therapy may be used in cases of ongoing inadequate hormonal control or residual or progressive structural disease. Despite improved outcomes, radiotherapy for pituitary tumours is associated with hypopituitarism, visual deficits and, rarely, secondary malignancies. We describe an unusual case of a 67-year-old female with presumed Cushing’s disease diagnosed at the age of 37, treated with transsphenoidal resection of a pituitary tumour with post-operative external beam radiotherapy (EBRT), ketoconazole for steroidogenesis inhibition, and finally bilateral adrenalectomy for refractory disease. She presented 30 years after her treatment with a witnessed generalised tonic-clonic seizure. Radiological investigations confirmed an extracranial mass infiltrating through the temporal bone and into brain parenchyma. Due to recurrent generalised seizures, the patient was intubated and commenced on dexamethasone and anti-epileptic therapy. Resection of the tumour revealed a high-grade osteoblastic osteosarcoma. Unfortunately, the patient deteriorated in intensive care and suffered a fatal cardiac arrest following a likely aspiration event. We describe the risk factors, prevalence and treatment of radiation-induced osteosarcoma, an exceedingly rare and late complication of pituitary irradiation. To our knowledge, this is the longest reported latency period between pituitary irradiation and the development of an osteosarcoma of the skull.

Learning points:

  • Cushing’s disease is treated with transsphenoidal resection as first-line therapy, with radiotherapy used in cases of incomplete resection, disease recurrence or persistent hypercortisolism.

  • The most common long-term adverse outcome of pituitary tumour irradiation is hypopituitarism occurring in 30–60% of patients at 10 years, and less commonly, vision loss and oculomotor nerve palsies, radiation-induced brain tumours and sarcomas.

  • Currently proposed characteristics of radiation-induced osteosarcomas include: the finding of a different histological type to the primary tumour, has developed within or adjacent to the path of the radiation beam, and a latency period of at least 3 years.

  • Treatment of osteosarcoma of the skull include complete surgical excision, followed by systemic chemotherapy and/or radiotherapy.

  • Overall prognosis in radiation-induced sarcoma of bone is poor.

  • Newer techniques such as stereotactic radiosurgery may reduce the incidence of radiation-induced malignancies.

Open access
Ravikumar Ravindran Section of Endocrinology, YYF Hospital, Ystrad Fawr Way, Caerphilly, UK

Search for other papers by Ravikumar Ravindran in
Google Scholar
PubMed
Close
,
Justyna Witczak Section of Endocrinology, YYF Hospital, Ystrad Fawr Way, Caerphilly, UK

Search for other papers by Justyna Witczak in
Google Scholar
PubMed
Close
,
Suhani Bahl Section of Endocrinology, YYF Hospital, Ystrad Fawr Way, Caerphilly, UK

Search for other papers by Suhani Bahl in
Google Scholar
PubMed
Close
,
Lakdasa D K E Premawardhana Section of Endocrinology, YYF Hospital, Ystrad Fawr Way, Caerphilly, UK
Centre for Endocrine and Diabetes Sciences, University Hospital of Wales, Cardiff, UK

Search for other papers by Lakdasa D K E Premawardhana in
Google Scholar
PubMed
Close
, and
Mohamed Adlan Section of Endocrinology, YYF Hospital, Ystrad Fawr Way, Caerphilly, UK

Search for other papers by Mohamed Adlan in
Google Scholar
PubMed
Close

Summary

A 53-year-old man who used growth hormone (GH), anabolic steroids and testosterone (T) for over 20 years presented with severe constipation and hypercalcaemia. He had benign prostatic hyperplasia and renal stones but no significant family history. Investigations showed – (1) corrected calcium (reference range) 3.66 mmol/L (2.2–2.6), phosphate 1.39 mmol/L (0.80–1.50), and PTH 2 pmol/L (1.6–7.2); (2) urea 21.9 mmol/L (2.5–7.8), creatinine 319 mmol/L (58–110), eGFR 18 mL/min (>90), and urine analysis (protein 4+, glucose 4+, red cells 2+); (3) creatine kinase 7952 U/L (40–320), positive anti Jo-1, and Ro-52 antibodies; (4) vitamin D 46 nmol/L (30–50), vitamin D3 29 pmol/L (55–139), vitamin A 4.65 mmol/L (1.10–2.60), and normal protein electrophoresis; (5) normal CT thorax, abdomen and pelvis and MRI of muscles showed ‘inflammation’, myositis and calcification; (6) biopsy of thigh muscles showed active myositis, chronic myopathic changes and mineral deposition and of the kidneys showed positive CD3 and CD45, focal segmental glomerulosclerosis and hypercalcaemic tubular changes; and (7) echocardiography showed left ventricular hypertrophy (likely medications and myositis contributing), aortic stenosis and an ejection fraction of 44%, and MRI confirmed these with possible right coronary artery disease. Hypercalcaemia was possibly multifactorial – (1) calcium release following myositis, rhabdomyolysis and acute kidney injury; (2) possible primary hyperparathyroidism (a low but detectable PTH); and (3) hypervitaminosis A. He was hydrated and given pamidronate, mycophenolate and prednisolone. Following initial biochemical and clinical improvement, he had multiple subsequent admissions for hypercalcaemia and renal deterioration. He continued taking GH and T despite counselling but died suddenly of a myocardial infarction.

Learning points:

  • The differential diagnosis of hypercalcaemia is sometimes a challenge.

  • Diagnosis may require multidisciplinary expertise and multiple and invasive investigations.

  • There may be several disparate causes for hypercalcaemia, although one usually predominates.

  • Maintaining ‘body image’ even with the use of harmful drugs may be an overpowering emotion despite counselling about their dangers.

Open access
Waralee Chatchomchaun Diabetes and Thyroid Center, Theptarin Hospital, Bangkok, Thailand

Search for other papers by Waralee Chatchomchaun in
Google Scholar
PubMed
Close
,
Yotsapon Thewjitcharoen Diabetes and Thyroid Center, Theptarin Hospital, Bangkok, Thailand

Search for other papers by Yotsapon Thewjitcharoen in
Google Scholar
PubMed
Close
,
Karndumri Krittadhee Diabetes and Thyroid Center, Theptarin Hospital, Bangkok, Thailand

Search for other papers by Karndumri Krittadhee in
Google Scholar
PubMed
Close
,
Veekij Veerasomboonsin Diabetes and Thyroid Center, Theptarin Hospital, Bangkok, Thailand

Search for other papers by Veekij Veerasomboonsin in
Google Scholar
PubMed
Close
,
Soontaree Nakasatien Diabetes and Thyroid Center, Theptarin Hospital, Bangkok, Thailand

Search for other papers by Soontaree Nakasatien in
Google Scholar
PubMed
Close
,
Sirinate Krittiyawong Diabetes and Thyroid Center, Theptarin Hospital, Bangkok, Thailand

Search for other papers by Sirinate Krittiyawong in
Google Scholar
PubMed
Close
,
Sriurai Porramatikul Diabetes and Thyroid Center, Theptarin Hospital, Bangkok, Thailand

Search for other papers by Sriurai Porramatikul in
Google Scholar
PubMed
Close
,
Ekgaluck Wanathayanoroj Diabetes and Thyroid Center, Theptarin Hospital, Bangkok, Thailand

Search for other papers by Ekgaluck Wanathayanoroj in
Google Scholar
PubMed
Close
,
Auchai Kanchanapituk Diabetes and Thyroid Center, Theptarin Hospital, Bangkok, Thailand

Search for other papers by Auchai Kanchanapituk in
Google Scholar
PubMed
Close
,
Pairoj Junyangdikul Department of Pathology, Samitivej Srinakarin Hospital, Bangkok Hospital Group, Bangkok, Thailand

Search for other papers by Pairoj Junyangdikul in
Google Scholar
PubMed
Close
, and
Thep Himathongkam Diabetes and Thyroid Center, Theptarin Hospital, Bangkok, Thailand

Search for other papers by Thep Himathongkam in
Google Scholar
PubMed
Close

Summary

In this case report, we describe a 37-year-old male who presented with fever and tender neck mass. Neck ultrasonography revealed a mixed echogenic multiloculated solid-cystic lesion containing turbid fluid and occupying the right thyroid region. Thyroid function tests showed subclinical hyperthyroidism. The patient was initially diagnosed with thyroid abscess and he was subsequently treated with percutaneous aspiration and i.v. antibiotics; however, his clinical symptoms did not improve. Surgical treatment was then performed and a pathological examination revealed a ruptured epidermoid cyst with abscess formation. No thyroid tissue was identified in the specimen. The patient was discharged uneventfully. However, at the 3-month and 1-year follow-ups, the patient was discovered to have developed subclinical hypothyroidism. Neck ultrasonography revealed a normal thyroid gland. This report demonstrates a rare case of epidermoid cyst abscess in the cervical region, of which initial imaging and abnormal thyroid function tests led to the erroneous diagnosis of thyroid abscess.

Learning points:

  • Epidermoid cyst abscess at the cervical region can mimic thyroid abscess.

  • Neck ultrasonography cannot distinguish thyroid abscess from epidermoid cyst abscess.

  • Thyroid function may be altered due to the adjacent soft tissue inflammation.

Open access
Carmina Teresa Fuss Division of Endocrinology and Diabetology, Department of Medicine I, University Hospital Würzburg, Würzburg, Germany

Search for other papers by Carmina Teresa Fuss in
Google Scholar
PubMed
Close
,
Stephanie Burger-Stritt Division of Endocrinology and Diabetology, Department of Medicine I, University Hospital Würzburg, Würzburg, Germany

Search for other papers by Stephanie Burger-Stritt in
Google Scholar
PubMed
Close
,
Silke Horn Division of Endocrinology and Diabetology, Department of Medicine I, University Hospital Würzburg, Würzburg, Germany

Search for other papers by Silke Horn in
Google Scholar
PubMed
Close
,
Ann-Cathrin Koschker Division of Endocrinology and Diabetology, Department of Medicine I, University Hospital Würzburg, Würzburg, Germany

Search for other papers by Ann-Cathrin Koschker in
Google Scholar
PubMed
Close
,
Kathrin Frey Division of Endocrinology and Diabetology, Department of Medicine I, University Hospital Würzburg, Würzburg, Germany

Search for other papers by Kathrin Frey in
Google Scholar
PubMed
Close
,
Almuth Meyer Division of Endocrinology and Diabetology, Department of Internal Medicine, Helios Klinikum Erfurt, Erfurt, Germany

Search for other papers by Almuth Meyer in
Google Scholar
PubMed
Close
, and
Stefanie Hahner Division of Endocrinology and Diabetology, Department of Medicine I, University Hospital Würzburg, Würzburg, Germany

Search for other papers by Stefanie Hahner in
Google Scholar
PubMed
Close

Summary

Standard treatment of hypoparathyroidism consists of supplementation of calcium and vitamin D analogues, which does not fully restore calcium homeostasis. In some patients, hypoparathyroidism is refractory to standard treatment with persistent low serum calcium levels and associated clinical complications. Here, we report on three patients (58-year-old male, 52-year-old female, and 48-year-old female) suffering from severe treatment-refractory postsurgical hypoparathyroidism. Two patients had persistent hypocalcemia despite oral treatment with up to 4 µg calcitriol and up to 4 g calcium per day necessitating additional i.v. administration of calcium gluconate 2–3 times per week, whereas the third patient presented with high frequencies of hypocalcemic and treatment-associated hypercalcemic episodes. S.c. administration of rhPTH (1–34) twice daily (40 µg/day) or rhPTH (1–84) (100 µg/day) only temporarily increased serum calcium levels but did not lead to long-term stabilization. In all three cases, treatment with rhPTH (1–34) as continuous s.c. infusion via insulin pump was initiated. Normalization of serum calcium and serum phosphate levels was observed within 1 week at daily 1–34 parathyroid hormone doses of 15 µg to 29.4 µg. Oral vitamin D and calcium treatment could be stopped or reduced and regular i.v. calcium administration was no more necessary. Ongoing efficacy of this treatment has been documented for up to 7 years so far. Therefore, we conclude that hypoparathyroidism that is refractory to both conventional treatment and s.c. parathyroid hormone (single or twice daily) may be successfully treated with continuous parathyroid hormone administration via insulin pump.

Learning points:

  • Standard treatment of hypoparathyroidism still consists of administration of calcium and active vitamin D.

  • Very few patients with hypoparathyroidism also do not respond sufficiently to standard treatment or administration of s.c. parathyroid hormone once or twice daily.

  • In those cases, continuous s.c. administration of parathyroid hormone via insulin pump may represent a successful treatment alternative.

Open access
Åke Sjöholm Division of Endocrinology and Diabetology, Department of Internal Medicine, Gävle Hospital, Gävle, Sweden

Search for other papers by Åke Sjöholm in
Google Scholar
PubMed
Close
,
Maria João Pereira Department of Medical Sciences, Uppsala University, Uppsala, Sweden

Search for other papers by Maria João Pereira in
Google Scholar
PubMed
Close
,
Thomas Nilsson Department of Medical Sciences, Uppsala University, Uppsala, Sweden

Search for other papers by Thomas Nilsson in
Google Scholar
PubMed
Close
,
Torbjörn Linde Department of Medical Sciences, Uppsala University, Uppsala, Sweden

Search for other papers by Torbjörn Linde in
Google Scholar
PubMed
Close
,
Petros Katsogiannos Department of Medical Sciences, Uppsala University, Uppsala, Sweden

Search for other papers by Petros Katsogiannos in
Google Scholar
PubMed
Close
,
Jan Saaf Department of Internal Medicine, Västmanland Hospital Köping, Köping, Sweden

Search for other papers by Jan Saaf in
Google Scholar
PubMed
Close
, and
Jan W Eriksson Department of Medical Sciences, Uppsala University, Uppsala, Sweden

Search for other papers by Jan W Eriksson in
Google Scholar
PubMed
Close

Summary

Type B insulin resistance syndrome (TBIRS) is a very rare autoimmune disorder with polyclonal autoantibodies against the insulin receptor, resulting in severe and refractory hyperglycemia. Described here is a patient who within a few months after the onset of autoimmune type 1 diabetes increased her insulin requirements more than 20-fold; despite this she had considerable difficulty maintaining a plasma glucose value of <40–60 mmol/L (720–1100 mg/dL). On suspicion of TBIRS, the patient was started on tapering dose of glucocorticoids to overcome the autoimmune insulin receptor blockade, resulting in an immediate and pronounced effect. Within days, insulin requirements decreased by 80–90% and plasma glucose stabilized around 7–8 mmol/L (126–144 mg/dL). The presence of antibodies to the insulin receptor was detected by immunoprecipitation and binding assays. After a 4-month remission on low maintenance dose prednisolone, the patient relapsed, which required repeated plasmaphereses and immune column treatments with temporarily remarkable effect. Mixed and transient results were seen with rituximab, mycophenolic acid and bortezomib, but the glycemic status remained suboptimal. Lack of compliance and recurrent infections may have contributed to this.

Learning points:

  • Type B insulin resistance syndrome (TBIRS) is a very rare autoimmune disorder with acquired polyclonal autoantibodies against the insulin receptor, resulting in severe and refractory hyperglycemia.

  • We describe here a young patient in whom, a few months after the onset of a regular autoimmune diabetes, insulin requirements in a short time increased more than 20-fold, but despite this, the plasma glucose level could be kept at <40–60 mmol/L only with considerable difficulty. Did this patient have TBIRS?

  • On suspicion of TBIRS, the patient was started on tapering glucocorticoids to overcome the autoimmune insulin receptor blockade, resulting in an immediate and pronounced effect; within days insulin requirements decreased by 80–90% and plasma glucose stabilized around 7–8 mmol/L.

  • The presence of antibodies to the insulin receptor was detected by immunoprecipitation and binding assays.

    After a 4-month remission on low maintenance dose prednisolone, the patient relapsed, which required repeated plasmaphereses with temporarily remarkable effect.

  • TBIRS should be considered in diabetic patients whose glycemia and/or insulin requirements are inexplicably and dramatically increased.

Open access
Shamaila Zaman Hammersmith Hospital, Imperial College Healthcare NHS Trust, London, UK

Search for other papers by Shamaila Zaman in
Google Scholar
PubMed
Close
,
Bijal Patel Hammersmith Hospital, Imperial College Healthcare NHS Trust, London, UK

Search for other papers by Bijal Patel in
Google Scholar
PubMed
Close
,
Paul Glynne The Physicians’ Clinic, London, UK

Search for other papers by Paul Glynne in
Google Scholar
PubMed
Close
,
Mark Vanderpump The Physicians’ Clinic, London, UK

Search for other papers by Mark Vanderpump in
Google Scholar
PubMed
Close
,
Ali Alsafi Hammersmith Hospital, Imperial College Healthcare NHS Trust, London, UK

Search for other papers by Ali Alsafi in
Google Scholar
PubMed
Close
,
Sairah Khan Hammersmith Hospital, Imperial College Healthcare NHS Trust, London, UK

Search for other papers by Sairah Khan in
Google Scholar
PubMed
Close
,
Rashpal Flora Hammersmith Hospital, Imperial College Healthcare NHS Trust, London, UK

Search for other papers by Rashpal Flora in
Google Scholar
PubMed
Close
,
Fausto Palazzo Hammersmith Hospital, Imperial College Healthcare NHS Trust, London, UK

Search for other papers by Fausto Palazzo in
Google Scholar
PubMed
Close
, and
Florian Wernig Hammersmith Hospital, Imperial College Healthcare NHS Trust, London, UK

Search for other papers by Florian Wernig in
Google Scholar
PubMed
Close

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.

Open access
Kaja Grønning Department of Endocrinology, Akershus University Hospital, Lorenskog, Norway

Search for other papers by Kaja Grønning in
Google Scholar
PubMed
Close
,
Archana Sharma Department of Endocrinology, Akershus University Hospital, Lorenskog, Norway

Search for other papers by Archana Sharma in
Google Scholar
PubMed
Close
,
Maria Adele Mastroianni Department of Haematology, Akershus University Hospital, Lorenskog, Norway

Search for other papers by Maria Adele Mastroianni in
Google Scholar
PubMed
Close
,
Bo Daniel Karlsson Department of Radiology, Akershus University Hospital, Lorenskog, Norway

Search for other papers by Bo Daniel Karlsson in
Google Scholar
PubMed
Close
,
Eystein S Husebye Department of Clinical Science and K.G. Jebsen Center of Autoimmune Disorders, University of Bergen, Bergen, Norway
Department of Medicine, Haukeland University Hospital, Bergen, Norway

Search for other papers by Eystein S Husebye in
Google Scholar
PubMed
Close
,
Kristian Løvås Department of Clinical Science and K.G. Jebsen Center of Autoimmune Disorders, University of Bergen, Bergen, Norway
Department of Medicine, Haukeland University Hospital, Bergen, Norway

Search for other papers by Kristian Løvås in
Google Scholar
PubMed
Close
, and
Ingrid Nermoen Department of Endocrinology, Akershus University Hospital, Lorenskog, Norway
Institute of Clinical Medicine, Faculty of Medicine, University of Oslo, Oslo, Norway

Search for other papers by Ingrid Nermoen in
Google Scholar
PubMed
Close

Summary

Primary adrenal lymphoma (PAL) is a rare cause of adrenal insufficiency. More than 90% is of B-cell origin. The condition is bilateral in up to 75% of cases, with adrenal insufficiency in two of three patients. We report two cases of adrenal insufficiency presenting at the age of 70 and 79 years, respectively. Both patients had negative 21-hydroxylase antibodies with bilateral adrenal lesions on CT. Biopsy showed B-cell lymphoma. One of the patients experienced intermittent disease regression on replacement dosage of glucocorticoids.

Learning points:

  • Primary adrenal lymphoma (PAL) is a rare cause of adrenal insufficiency.

  • Bilateral adrenal masses of unknown origin or in individuals with suspected extra-adrenal malignancy should be biopsied quickly when pheochromocytoma is excluded biochemically.

  • Steroid treatment before biopsy may affect diagnosis.

  • Adrenal insufficiency with negative 21-hydroxylase antibodies should be evaluated radiologically.

Open access
J K Witczak Section of Endocrinology, Department of Medicine, Prince Phillip Hospital
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
Close
,
N Ubaysekara Centre for Endocrine and Diabetes Sciences, University Hospital of Wales, Heath Park, Cardiff, UK

Search for other papers by N Ubaysekara in
Google Scholar
PubMed
Close
,
R Ravindran Centre for Endocrine and Diabetes Sciences, University Hospital of Wales, Heath Park, Cardiff, UK

Search for other papers by R Ravindran in
Google Scholar
PubMed
Close
,
S Rice Section of Endocrinology, Department of Medicine, Prince Phillip Hospital

Search for other papers by S Rice in
Google Scholar
PubMed
Close
,
Z Yousef Department of Cardiology, University Hospital of Wales, Heath Park, Cardiff, UK

Search for other papers by Z Yousef in
Google Scholar
PubMed
Close
, and
L D Premawardhana Centre for Endocrine and Diabetes Sciences, University Hospital of Wales, Heath Park, Cardiff, UK

Search for other papers by L D Premawardhana in
Google Scholar
PubMed
Close

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.

Open access
Misaki Aoshima Departments of Endocrinology Diabetes and Metabolism, Hamamatsu Medical Center, Hamamatsu, Shizuoka, Japan

Search for other papers by Misaki Aoshima in
Google Scholar
PubMed
Close
,
Koji Nagayama Departments of Endocrinology Diabetes and Metabolism, Hamamatsu Medical Center, Hamamatsu, Shizuoka, Japan

Search for other papers by Koji Nagayama in
Google Scholar
PubMed
Close
,
Kei Takeshita Departments of Endocrinology Diabetes and Metabolism, Hamamatsu Medical Center, Hamamatsu, Shizuoka, Japan

Search for other papers by Kei Takeshita in
Google Scholar
PubMed
Close
,
Hiroshi Ajima Departments of Endocrinology Diabetes and Metabolism, Hamamatsu Medical Center, Hamamatsu, Shizuoka, Japan

Search for other papers by Hiroshi Ajima in
Google Scholar
PubMed
Close
,
Sakurako Orikasa Departments of Endocrinology Diabetes and Metabolism, Hamamatsu Medical Center, Hamamatsu, Shizuoka, Japan

Search for other papers by Sakurako Orikasa in
Google Scholar
PubMed
Close
,
Ayana Iwazaki ²Departments of Endocrinology Diabetes and Metabolism, Seirei Hamamatsu General Hospital, Hamamatsu, Shizuoka, Japan

Search for other papers by Ayana Iwazaki in
Google Scholar
PubMed
Close
,
Hiroaki Takatori Department of Rheumatology, Hamamatsu Medical Center, Hamamatsu, Shizuoka, Japan

Search for other papers by Hiroaki Takatori in
Google Scholar
PubMed
Close
, and
Yutaka Oki Department of Family and Community Medicine, Hamamatsu University School of Medicine, Hamamatsu, Shizuoka, Japan

Search for other papers by Yutaka Oki in
Google Scholar
PubMed
Close

Summary

Patients treated with immunosuppressive drugs, especially methotrexate (MTX), rarely develop lymphoproliferative disorders (LPDs), known as MTX-related LPD (MTX–LPD). The primary site of MTX–LPD is often extranodal. This is the first reported case of MTX–LPD in the pituitary. A 65-year-old woman was admitted to our hospital with symptoms of oculomotor nerve palsy and multiple subcutaneous nodules. She had been treated with MTX for 11 years for rheumatoid arthritis. Computed tomography showed multiple masses in the orbit, sinuses, lung fields, anterior mediastinum, kidney, and subcutaneous tissue. Brain magnetic resonance imaging revealed a sellar mass. She was diagnosed with hypopituitarism and central diabetes insipidus based on endocrine examination. Although pituitary biopsy could not be performed, we concluded that the pituitary lesion was from MTX–LPD, similar to the lesions in the sinuses, anterior mediastinum, and subcutaneous tissue, which showed polymorphic LPD on biopsy. MTX was discontinued, and methylprednisolone was administered to improve the neurologic symptoms. After several weeks, there was marked improvement of all lesions, including the pituitary lesion, but the pituitary function did not improve. When pituitary lesions are caused by MTX–LPD, the possibility of anterior hypopituitarism and central diabetes insipidus needs to be considered. Further studies are needed to investigate the effectiveness of early diagnosis and treatment of MTX–LPD in restoring pituitary dysfunction.

Learning points

  • Pituitary lesions from MTX–LPD may cause hypopituitarism and central diabetes insipidus.

  • Pituitary metastasis of malignant lymphoma and primary pituitary lymphoma, which have the same tissue types with MTX–LPD, have poor prognosis, but the lesions of MTX–LPD can regress only after MTX discontinuation.

  • In cases of pituitary lesions alone, a diagnosis of MTX–LPD may be difficult, unless pituitary biopsy is performed. This possibility should be considered in patients treated with immunosuppressive drugs.

  • Pituitary hypofunction and diabetes insipidus may persist, even after regression of the lesions on imaging due to MTX discontinuation.