Lymphocytic hypophysitis associated with Behcet's disease: A case report and review of the literature

Lymphocytic hypophysitis (LH) is a rare inflammatory disorder involving the pituitary gland, often with other autoimmune diseases combined. The coexistence of LH and Behçet\'s disease (BD) is a rare combination and only one case was reported in the previous literatures. A 50-year-old man was ad...

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Vydáno v:Neuro-endocrinology letters Ročník 39; číslo 1; s. 43
Hlavní autoři: Tao, Tingting, Zhang, Ziyi, Li, Hong
Médium: Journal Article
Jazyk:angličtina
Vydáno: Sweden 01.03.2018
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ISSN:0172-780X
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Abstract Lymphocytic hypophysitis (LH) is a rare inflammatory disorder involving the pituitary gland, often with other autoimmune diseases combined. The coexistence of LH and Behçet\'s disease (BD) is a rare combination and only one case was reported in the previous literatures. A 50-year-old man was admitted into Sir Run Run Shaw Hospital presented with frontal headache and fatigue which had lasted for four months. Endocrinological inspection indicated anterior pituitary dysfunction. Magnetic resonance imaging (MRI) revealed homogeneous pituitary enlargement and thickened pituitary stalk. Administration of glucocorticoids could effectively relieve headache and reduced pituitary mass volume. Oral aphthosis, skin lesions and positive pathergy tests were recognized later, which were characteristic features of BD. Although the diagnosis of BD is mainly dependent on clinical manifestations, PT is still a useful diagnostic tool 0f high specificityfor BD. And this male patient was diagnosed with both LH and BD afterwards. Then he was treated with cyclophosphamide and medium doses of methylprednisolone and remained in good conditions at the follow-up. LH and BD might share a common underlying autoimmune pathogenesis. The presentation of endocrinologic disturbances such as anterior pituitary dysfunction with typical features of skin lesions should prompt further investigation of possible comorbid autoimmune disease involving multiple organ systems. Early diagnosis and close monitoring are vitally important to ensure a stable endocrinologic status.
AbstractList Lymphocytic hypophysitis (LH) is a rare inflammatory disorder involving the pituitary gland, often with other autoimmune diseases combined. The coexistence of LH and Behçet\'s disease (BD) is a rare combination and only one case was reported in the previous literatures. A 50-year-old man was admitted into Sir Run Run Shaw Hospital presented with frontal headache and fatigue which had lasted for four months. Endocrinological inspection indicated anterior pituitary dysfunction. Magnetic resonance imaging (MRI) revealed homogeneous pituitary enlargement and thickened pituitary stalk. Administration of glucocorticoids could effectively relieve headache and reduced pituitary mass volume. Oral aphthosis, skin lesions and positive pathergy tests were recognized later, which were characteristic features of BD. Although the diagnosis of BD is mainly dependent on clinical manifestations, PT is still a useful diagnostic tool 0f high specificityfor BD. And this male patient was diagnosed with both LH and BD afterwards. Then he was treated with cyclophosphamide and medium doses of methylprednisolone and remained in good conditions at the follow-up. LH and BD might share a common underlying autoimmune pathogenesis. The presentation of endocrinologic disturbances such as anterior pituitary dysfunction with typical features of skin lesions should prompt further investigation of possible comorbid autoimmune disease involving multiple organ systems. Early diagnosis and close monitoring are vitally important to ensure a stable endocrinologic status.
Lymphocytic hypophysitis (LH) is a rare inflammatory disorder involving the pituitary gland, often with other autoimmune diseases combined. The coexistence of LH and Behçet\'s disease (BD) is a rare combination and only one case was reported in the previous literatures. A 50-year-old man was admitted into Sir Run Run Shaw Hospital presented with frontal headache and fatigue which had lasted for four months. Endocrinological inspection indicated anterior pituitary dysfunction. Magnetic resonance imaging (MRI) revealed homogeneous pituitary enlargement and thickened pituitary stalk. Administration of glucocorticoids could effectively relieve headache and reduced pituitary mass volume. Oral aphthosis, skin lesions and positive pathergy tests were recognized later, which were characteristic features of BD. Although the diagnosis of BD is mainly dependent on clinical manifestations, PT is still a useful diagnostic tool 0f high specificityfor BD. And this male patient was diagnosed with both LH and BD afterwards. Then he was treated with cyclophosphamide and medium doses of methylprednisolone and remained in good conditions at the follow-up. LH and BD might share a common underlying autoimmune pathogenesis. The presentation of endocrinologic disturbances such as anterior pituitary dysfunction with typical features of skin lesions should prompt further investigation of possible comorbid autoimmune disease involving multiple organ systems. Early diagnosis and close monitoring are vitally important to ensure a stable endocrinologic status.Lymphocytic hypophysitis (LH) is a rare inflammatory disorder involving the pituitary gland, often with other autoimmune diseases combined. The coexistence of LH and Behçet\'s disease (BD) is a rare combination and only one case was reported in the previous literatures. A 50-year-old man was admitted into Sir Run Run Shaw Hospital presented with frontal headache and fatigue which had lasted for four months. Endocrinological inspection indicated anterior pituitary dysfunction. Magnetic resonance imaging (MRI) revealed homogeneous pituitary enlargement and thickened pituitary stalk. Administration of glucocorticoids could effectively relieve headache and reduced pituitary mass volume. Oral aphthosis, skin lesions and positive pathergy tests were recognized later, which were characteristic features of BD. Although the diagnosis of BD is mainly dependent on clinical manifestations, PT is still a useful diagnostic tool 0f high specificityfor BD. And this male patient was diagnosed with both LH and BD afterwards. Then he was treated with cyclophosphamide and medium doses of methylprednisolone and remained in good conditions at the follow-up. LH and BD might share a common underlying autoimmune pathogenesis. The presentation of endocrinologic disturbances such as anterior pituitary dysfunction with typical features of skin lesions should prompt further investigation of possible comorbid autoimmune disease involving multiple organ systems. Early diagnosis and close monitoring are vitally important to ensure a stable endocrinologic status.
Author Tao, Tingting
Li, Hong
Zhang, Ziyi
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  givenname: Ziyi
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  fullname: Zhang, Ziyi
  organization: Department of Endocrinology, Sir Run Run Shaw Hospital, School of Medicine, Zhejiang University, Hangzhou, China
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  givenname: Hong
  surname: Li
  fullname: Li, Hong
  organization: Department of Endocrinology, Sir Run Run Shaw Hospital, School of Medicine, Zhejiang University, Hangzhou, China
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SubjectTerms Autoimmune Hypophysitis - complications
Autoimmune Hypophysitis - diagnostic imaging
Autoimmune Hypophysitis - drug therapy
Behcet Syndrome - complications
Behcet Syndrome - diagnostic imaging
Behcet Syndrome - drug therapy
Fatigue - drug therapy
Fatigue - etiology
Glucocorticoids - therapeutic use
Headache - drug therapy
Headache - etiology
Humans
Magnetic Resonance Imaging
Male
Middle Aged
Pituitary Diseases - complications
Pituitary Diseases - diagnostic imaging
Pituitary Diseases - drug therapy
Pituitary Gland - diagnostic imaging
Pituitary Gland, Anterior
Pituitary Hormones - blood
Title Lymphocytic hypophysitis associated with Behcet's disease: A case report and review of the literature
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