Hipofisitis linfoplasmocitaria y fibrosis retroperitoneal asociadas a un síndrome poliglandular autoinmune. Enfermedades relacionadas con IgG4. Caso clínico

We report a 23 year old woman presenting with a nephrotic syndrome due to minimal change disease, central diabetes insipidus, primary hypothyroidism, vitiligo and universal alopecia. Eleven years later, she presented secondary amenorrhea due to hypogonadotropic hypogonadism, with mild hyperprolactin...

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Autores principales:	Guarda,Francisco J., Méndez,Gonzalo P., Espinosa,Roberto, Gejman,Roger, Vega,Jorge
Lenguaje:	Spanish / Castilian
Publicado:	Sociedad Médica de Santiago 2018
Materias:	Autoimmune Autoimmune Hypophysitis Hypothyroidism Polyendocrinopathies Retroperitoneal Fibrosis Immunoglobulin G4-Related Disease
Acceso en línea:	http://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872018001201486
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spelling	oai:scielo:S0034-988720180012014862019-02-18Hipofisitis linfoplasmocitaria y fibrosis retroperitoneal asociadas a un síndrome poliglandular autoinmune. Enfermedades relacionadas con IgG4. Caso clínicoGuarda,Francisco J.Méndez,Gonzalo P.Espinosa,RobertoGejman,RogerVega,Jorge Autoimmune Autoimmune Hypophysitis Hypothyroidism Polyendocrinopathies Retroperitoneal Fibrosis Immunoglobulin G4-Related Disease We report a 23 year old woman presenting with a nephrotic syndrome due to minimal change disease, central diabetes insipidus, primary hypothyroidism, vitiligo and universal alopecia. Eleven years later, she presented secondary amenorrhea due to hypogonadotropic hypogonadism, with mild hyperprolactinemia and central adrenal insufficiency. A magnetic resonance imaging of the sella turcica showed a pituitary mass with suprasellar extension that was resected using a transsphenoidal approach. Pathology confirmed the presence of a lymphoplasmacytic hypophysitis. She needed a second surgical resection due to mass growth and neuro-ophthalmologic impairment. One year later, systemic lupus erythematosus, arterial hypertension and type 2 diabetes mellitus were diagnosed. Two years later, due to back pain, constipation and renal failure, retroperitoneal fibrosis was found, satisfactorily treated with glucocorticoids and colchicine. Hence, this clinical vignette shows the coexistence of autoimmune polyglandular syndrome with retroperitoneal fibrosis and lymphoplasmacytic hypophysitis. Tissue analysis showed the presence of IgG4 producing plasma cells in the pituitary and retroperitoneum, which constitute a basis for the diagnosis of IgG4 related disease.info:eu-repo/semantics/openAccessSociedad Médica de SantiagoRevista médica de Chile v.146 n.12 20182018-12-01text/htmlhttp://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872018001201486es10.4067/s0034-98872018001201486
institution	Scielo Chile
collection	Scielo Chile
language	Spanish / Castilian
topic	Autoimmune Autoimmune Hypophysitis Hypothyroidism Polyendocrinopathies Retroperitoneal Fibrosis Immunoglobulin G4-Related Disease
spellingShingle	Autoimmune Autoimmune Hypophysitis Hypothyroidism Polyendocrinopathies Retroperitoneal Fibrosis Immunoglobulin G4-Related Disease Guarda,Francisco J. Méndez,Gonzalo P. Espinosa,Roberto Gejman,Roger Vega,Jorge Hipofisitis linfoplasmocitaria y fibrosis retroperitoneal asociadas a un síndrome poliglandular autoinmune. Enfermedades relacionadas con IgG4. Caso clínico
description	We report a 23 year old woman presenting with a nephrotic syndrome due to minimal change disease, central diabetes insipidus, primary hypothyroidism, vitiligo and universal alopecia. Eleven years later, she presented secondary amenorrhea due to hypogonadotropic hypogonadism, with mild hyperprolactinemia and central adrenal insufficiency. A magnetic resonance imaging of the sella turcica showed a pituitary mass with suprasellar extension that was resected using a transsphenoidal approach. Pathology confirmed the presence of a lymphoplasmacytic hypophysitis. She needed a second surgical resection due to mass growth and neuro-ophthalmologic impairment. One year later, systemic lupus erythematosus, arterial hypertension and type 2 diabetes mellitus were diagnosed. Two years later, due to back pain, constipation and renal failure, retroperitoneal fibrosis was found, satisfactorily treated with glucocorticoids and colchicine. Hence, this clinical vignette shows the coexistence of autoimmune polyglandular syndrome with retroperitoneal fibrosis and lymphoplasmacytic hypophysitis. Tissue analysis showed the presence of IgG4 producing plasma cells in the pituitary and retroperitoneum, which constitute a basis for the diagnosis of IgG4 related disease.
author	Guarda,Francisco J. Méndez,Gonzalo P. Espinosa,Roberto Gejman,Roger Vega,Jorge
author_facet	Guarda,Francisco J. Méndez,Gonzalo P. Espinosa,Roberto Gejman,Roger Vega,Jorge
author_sort	Guarda,Francisco J.
title	Hipofisitis linfoplasmocitaria y fibrosis retroperitoneal asociadas a un síndrome poliglandular autoinmune. Enfermedades relacionadas con IgG4. Caso clínico
title_short	Hipofisitis linfoplasmocitaria y fibrosis retroperitoneal asociadas a un síndrome poliglandular autoinmune. Enfermedades relacionadas con IgG4. Caso clínico
title_full	Hipofisitis linfoplasmocitaria y fibrosis retroperitoneal asociadas a un síndrome poliglandular autoinmune. Enfermedades relacionadas con IgG4. Caso clínico
title_fullStr	Hipofisitis linfoplasmocitaria y fibrosis retroperitoneal asociadas a un síndrome poliglandular autoinmune. Enfermedades relacionadas con IgG4. Caso clínico
title_full_unstemmed	Hipofisitis linfoplasmocitaria y fibrosis retroperitoneal asociadas a un síndrome poliglandular autoinmune. Enfermedades relacionadas con IgG4. Caso clínico
title_sort	hipofisitis linfoplasmocitaria y fibrosis retroperitoneal asociadas a un síndrome poliglandular autoinmune. enfermedades relacionadas con igg4. caso clínico
publisher	Sociedad Médica de Santiago
publishDate	2018
url	http://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872018001201486
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Hipofisitis linfoplasmocitaria y fibrosis retroperitoneal asociadas a un síndrome poliglandular autoinmune. Enfermedades relacionadas con IgG4. Caso clínico

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