Updating the Rainbow of Flavors of Hypophysitis

Ananthakrishnan, Sonia

doi:10.4158/accr-2018-0164

Cited by 2 publications

(4 citation statements)

References 13 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“… 2 Systemic diseases such as sarcoidosis, granulomatous vasculitis, tuberculosis, and LCH can present similarly and extrapituitary manifestations usually provide clues to the diagnosis, although it can still prove challenging, as in our case. 1 , 2 , 3 , 6 …”

Section: Discussionmentioning

confidence: 99%

“… 6 , 9 Clinical presentation is heterogeneous, ranging from an indolent single-site disease or stable multisystem disease to disseminated aggressive disease with hematopoietic tissue involvement and high risk of mortality. 1 , 6 , 9 , 10 Infiltration of the pituitary stalk with central DI is among the most common endocrine manifestation, affecting 30% of adults with LCH and 40% of those with multisystem disease. 9 Anterior pituitary deficiencies are reported in 20% of patients with LCH, growth hormone deficiency being the most common, followed by gonadotropins, TSH, and ACTH.…”

Section: Discussionmentioning

confidence: 99%

“…Pituitary stalk lesions often present with central diabetes insipidus (DI) and varying forms of hypopituitarism termed as infundibulohypophysitis. 1 This poses a diagnostic challenge as multiple pathologies involving the infundibulum can cause a similar clinical picture. 2 Lymphocytic (ie, autoimmune) hypophysitis (LH) is a common cause of infundibulohypophysitis in younger women but is usually a presumptive diagnosis after the exclusion of systemic pathology.…”

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

A Case of Burnt-Out Langerhans Cell Histiocytosis Presenting as Postpartum Hypopituitarism

Karri

Hsieh

Binder

et al. 2021

AACE Clinical Case Reports

View full text Add to dashboard Cite

Objective To evaluate the case of a woman who presented with central hypogonadism and diabetes insipidus and further developed a persistent cough leading to an unexpected diagnosis of burnt-out Langerhans cell histiocytosis (LCH). Methods Clinical and laboratory endocrine evaluation, magnetic resonance imaging, high-resolution computed tomography, and open-lung biopsy results are discussed. Results A 28-year-old woman presented at 10 months postpartum with polydipsia, polyuria, and amenorrhea for 3 months. Her results showed a prolactin level of 25 μg/L (reference,<23.5 μg/L), estrogen level of 91 pmol/L (reference, 110-180 pmol/L), follicle-stimulating hormone level of 6 IU/L (reference, 2-20 IU/L), and luteinizing hormone level of 6 IU/L (reference, 2-70 IU/L). A water-deprivation test found a sodium concentration of 148 mmol/L (reference, 135-145 mmol/L), serum osmolality of 310 mmol/kg (reference, 275-295 mmol/kg), and urine osmolality of 107 mmol/kg (reference, 50-1450 mmol/kg) that improved to 142 mEq/L, 295 mmol/kg, and 535 mmol/kg, respectively, after desmopressin administration. Gadolinium-enhanced pituitary magnetic resonance imaging demonstrated a markedly thickened stalk with uniform enhancement. Chest high-resolution computed tomography confirmed bilateral upper-zone cystic lung disease suggestive of either pulmonary lymphangioleiomyomatosis or LCH. Eventual histology showed CD1a-positive burnt-out LCH. This differentiation was crucial as pulmonary lymphangioleiomyomatosis exacerbates with estrogen therapy and pregnancy, which the patient was able to successfully pursue without disease exacerbation. Conclusion The patient’s initial presentation was considered as lymphocytic hypophysitis, but subsequent cystic changes on high-resolution computed tomography led to a unifying definitive diagnosis of burnt-out LCH. This case highlights the importance of investigating for uncommon secondary causes of hypophysitis.

show abstract

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

A Case of Burnt-Out Langerhans Cell Histiocytosis Presenting as Postpartum Hypopituitarism

Karri

Hsieh

Binder

et al. 2021

AACE Clinical Case Reports

View full text Add to dashboard Cite

show abstract

“…Pituitary stalk lesions often present with central diabetes insipidus (DI) and varying forms of hypopituitarism termed as infundibulohypophysitis (1). This poses a diagnostic challenge since multiple pathologies involving the infundibulum can cause a similar clinical picture (2).…”

Section: Introductionmentioning

confidence: 99%

A Case of Burnt Out Langerhans Cell Histiocytosis Presenting as Post-Partum Hypopituitarism

Karri

Hsieh

Binder

et al. 2020

AACE Clinical Case Reports

View full text Add to dashboard Cite

Objective: Hypopituitarism in post-partum women is usually attributed to lymphocytic hypophysitis (LH), but can also be secondary to systemic pathology. We discuss a case of a lady presenting with central hypogonadism and diabetes insipidus, who further developed persistent cough, leading to an unexpected diagnosis of burnt-out Langerhans’ cell histiocytosis (LCH). Methods: Clinical and laboratory endocrine evaluation, magnetic resonance imaging (MRI), high-resolution computed tomography (HRCT) and open-lung biopsy results are discussed. Results: A 28 year old lady, presented at 10 months postpartum with polydipsia, polyuria and amenorrhoea for 3 months. Her results showed prolactin of 25 (reference,<23.5) μg/L , oestrogen of 24.8 (reference,30–49), follicular stimulating hormone 6 (reference, 2–20) IU/L and luteinizing hormone 6 (reference, 2–70) IU/L. Water deprivation test demonstrated sodium 148 (reference, 135–145) mEq/l, serum osmolality 310 (reference, 275–295) mmol/kg and urine osmolality 107 (reference,50–1450) mmol/kg improving to 142 mEq/L, 295mEq/L and 535 mEq/L respectively, after desmopressin. Gadolinium enhanced pituitary MRI demonstrated a markedly thickened stalk with uniform enhancement. Chest HRCT confirmed bilateral upper zone cystic lung disease suggestive of either pulmonary lymphangioleiomyomatosis (PLAM) or LCH. Eventual histology showed CD1a-positive burnt-out LCH. This differentiation was crucial as PLAM exacerbates with oestrogen therapy and pregnancy which she was able to successfully pursue without disease exacerbation. Conclusion: The patient’s initial presentation was considered as LH, but subsequent cystic changes on HRCT led to a unifying definitive diagnosis of burnt-out LCH. This case highlights the importance of investigating for uncommon secondary causes of hypophysitis.

show abstract

Updating the Rainbow of Flavors of Hypophysitis

Cited by 2 publications

References 13 publications

A Case of Burnt-Out Langerhans Cell Histiocytosis Presenting as Postpartum Hypopituitarism

A Case of Burnt-Out Langerhans Cell Histiocytosis Presenting as Postpartum Hypopituitarism

A Case of Burnt Out Langerhans Cell Histiocytosis Presenting as Post-Partum Hypopituitarism

Contact Info

Product

Resources

About