Hypothalamic–pituitary sarcoidosis with vision loss and hypopituitarism: case series and literature review

Anthony, Jeremy R.; Esper, Gregory J.; Ioachimescu, Adriana G.

doi:10.1007/s11102-015-0678-x

Cited by 57 publications

(40 citation statements)

References 25 publications

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“…Imaging studies are usually characterised by sellar/suprasellar masses with or without pituitary stalk thickening. Hypothalamic and pituitary sarcoid involvement appears to be more common in men as opposed to other manifestations of sarcoidosis which have a female predilection 7. Earlier postmortem studies of patients with sarcoidosis complicated by hypopituitarism assumed that the hormone deficiency was due to pituitary gland destruction by granuloma formation,8 but subsequent studies showed responsiveness of pituitary gland to synthetic hypothalamic releasing factors suggesting that hypothalamic insufficiency may be the major cause of hypopituitarism in these patients 9…”

Section: Discussionmentioning

confidence: 99%

“…Hypogonadism appears to be the most common hormone deficiency associated with hypothalamic pituitary sarcoidosis 7 10 11. In a study of nine patients with hypothalamic–pituitary sarcoidosis, hypogonadism occurred in all the patients, diabetes insipidus in seven patients and hyperprolactinaemia in three patients 10.…”

Section: Discussionmentioning

confidence: 99%

“…Hypogonadism was the most common feature followed by TSH deficiency and hyperprolactinaemia, whereas diabetes insipidus occurred in half of the patients 11. There was no correlation of imaging with the pituitary hormone deficiency found and even though glucocorticoid treatment improved imaging abnormalities, it rarely resulted in resolution of the pituitary hormone deficiencies 7 10 11…”

Section: Discussionmentioning

confidence: 99%

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Systemic sarcoidosis with hypercalcaemia, hypothalamic–pituitary dysfunction and thyroid involvement

2016

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Sarcoidosis is a multisystem granulomatous disorder. The lungs are the principal organs affected, however, extrapulmonary involvement including disorders of the pituitary and thyroid glands has been reported but presentation with multiple endocrine manifestations is rare. We report the case of a 36-year-old African-American woman who presented with hypercalcaemia, abnormal thyroid function studies and secondary amenorrhoea. On workup including laboratory, radiological testing and biopsy she was diagnosed with sarcoidosis with multi-organ involvement. Endocrine manifestations included non-parathyroid hormone mediated hypercalcaemia related to sarcoidosis, thyroid involvement with sarcoidosis and hypothalamic-pituitary involvement with a sellar and suprasellar mass associated with secondary adrenal insufficiency, secondary hypogonadism, growth hormone deficiency and secondary hypothyroidism. We report to the best of our knowledge the first case of simultaneous multiple endocrine manifestations of sarcoidosis that included hypercalcaemia, hypopituitarism and sarcoidosis of the thyroid gland.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Systemic sarcoidosis with hypercalcaemia, hypothalamic–pituitary dysfunction and thyroid involvement

2016

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“…bilateral facial nerve palsy) is suggestive of NSOptic nerve involvement may have a more difficult disease course with refractory disease and relapse on corticosteroid dose reductionA pharynx, soft palate and vocal cord syndrome from glossopharyngeal and vagus nerve involvement is recognisedBasal meningitis may be the pathophysiological substrate of cranial neuropathiesFocal neurology, multifocal neurology or diffuse encephalopathy due to parenchymal lesions of the brain or brainstem [2, 8, 9, 18–22, 44]Lesions may be multiple and often enhance. Biopsy of mass lesions is recommended for a definitive diagnosisBehaviour change, confusional states and psychosis are reportedHypothamic and pituitary dysfunction [2, 6, 8, 9, 20, 22, 28, 29, 44–46]Usually of insidious onset, due to suprasellar inflammatory lesions. The most eminent symptoms are bitemporal visual failure, polydipsia and polyuria (diabetes insipidus), and galactorrhoeaSymptoms may arise from hypothalamic dysfunction, hypopituitarism or compression of the optic chiasm by mass effectAn aseptic meningitis is often seenMyopathy [6, 19, 20, 22, 38]Usually asymptomatic.…”

Section: Diagnosismentioning

confidence: 99%

Neurosarcoidosis: a clinical approach to diagnosis and management

2016

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Sarcoidosis is a rare but important cause of neurological morbidity, and neurological symptoms often herald the diagnosis. Our understanding of neurosarcoidosis has evolved from early descriptions of a uveoparotid fever to include presentations involving every part of the neural axis. The diagnosis should be suspected in patients with sarcoidosis who develop new neurological symptoms, those presenting with syndromes highly suggestive of neurosarcoidosis, or neuro-inflammatory disease where more common causes have been excluded. Investigation should look for evidence of neuro-inflammation, best achieved by contrast-enhanced brain magnetic resonance imaging and cerebrospinal fluid analysis. Evidence of sarcoidosis outside the nervous system should be sought in search of tissue for biopsy. Skin lesions should be identified and biopsies taken. Chest radiography including high-resolution computed tomography is often informative. In difficult cases, fluorodeoxyglucose positron emission tomography and gallium-67 imaging may identify subclinical disease and a target for biopsy. Symptomatic patients should be treated with corticosteroids, and if clinically indicated other immunosuppressants such as hydroxychloroquine, azathioprine, cyclophosphamide or methotrexate should be added. Anti-tumour necrosis factor alpha therapies may be considered in refractory disease but caution should be exercised as there is evidence to suggest they may unmask disease.

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“…Central nervous system is affected in 5-15% of cases, and neurosarcoidosis may be the first presenting feature of these systemic diseases (57). Lesions of sarcoidosis in the sellar region are found in the hypothalamus, the stalk and pituitary gland; are rare and account for 1% of sellar masses (58). The classic MRI feature is thickening of the stalk.…”

Section: Sarcoidosismentioning

confidence: 99%

DIAGNOSIS OF ENDOCRINE DISEASE: Expanding the cause of hypopituitarism

Pekić

Popović

2017

European Journal of Endocrinology

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Hypopituitarism is defined as one or more pituitary hormone deficits due to a lesion in the hypothalamic-pituitary region. By far, the most common cause of hypopituitarism associated with a sellar mass is a pituitary adenoma. A high index of suspicion is required for diagnosing hypopituitarism in several other conditions such as other massess in the sellar and parasellar region, brain damage caused by radiation and by traumatic brain injury, vascular lesions, infiltrative/immunological/inflammatory diseases (lymphocytic hypophysitis, sarcoidosis and hemochromatosis), infectious diseases and genetic disorders. Hypopituitarism may be permanent and progressive with sequential pattern of hormone deficiencies (radiation-induced hypopituitarism) or transient after traumatic brain injury with possible recovery occurring years from the initial event. In recent years, there is increased reporting of less common and less reported causes of hypopituitarism with its delayed diagnosis. The aim of this review is to summarize the published data and to allow earlier identification of populations at risk of hypopituitarism as optimal hormonal replacement may significantly improve their quality of life and life expectancy.Correspondence should be addressed to V Popovic

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Hypothalamic–pituitary sarcoidosis with vision loss and hypopituitarism: case series and literature review

Abstract: HP NS is a serious disease requiring multidisciplinary treatment and lifelong follow-up. Prospective multicentric studies are needed to determine a more standardized approach to HP NS and outline predictors of disease outcome.

Cited by 57 publications

References 25 publications

Systemic sarcoidosis with hypercalcaemia, hypothalamic–pituitary dysfunction and thyroid involvement

Systemic sarcoidosis with hypercalcaemia, hypothalamic–pituitary dysfunction and thyroid involvement

Neurosarcoidosis: a clinical approach to diagnosis and management

DIAGNOSIS OF ENDOCRINE DISEASE: Expanding the cause of hypopituitarism

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