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HYPOPHYSITIS AND RETROPERITONEAL FIBROSIS ASSOCIATED WITH AUTOIMMUNE POLYGLANDULAR SYNDROME. REPORT OF ONE CASE

Hipofisitis linfoplasmocitaria y fibrosis retroperitoneal asociadas a un síndrome poliglandular autoinmune. Enfermedades relacionadas con IgC4. Caso Clínico

dc.contributoren-US
dc.contributorNingunaes-ES
dc.creatorGuarda, Francisco J; Departamento de Endocrinología, Hospital Clínico, Pontificia Universidad Católica de Chile, Santiago, Chile.
dc.creatorMéndez, Gonzalo P; Departamento de Anatomía Patológica, Escuela de Medicina, Hospital Clínico de la Pontificia Universidad Católica de Chile, Santiago, Chile.
dc.creatorEspinosa, Roberto; Servicio de Anatomía Patológica, Hospital Naval, Almirante Nef, Viña del Mar, Chile.
dc.creatorGejman, Roger; Departamento de Anatomía Patológica, Escuela de Medicina, Hospital Clínico de la Pontificia Universidad Católica de Chile, Santiago, Chile.
dc.creatorVega, Jorge; Departamento de Medicina; Escuela de Medicina Universidad de Valparaíso; Hospital Naval A. Nef; Servicio de Medicina; Sección de Nefrología, Valparaíso, Chile.
dc.date2018-11-13
dc.date.accessioned2019-11-11T18:27:49Z
dc.date.available2019-11-11T18:27:49Z
dc.identifierhttp://www.revistamedicadechile.cl/ojs/index.php/rmedica/article/view/6900
dc.identifier.urihttps://revistaschilenas.uchile.cl/handle/2250/111286
dc.descriptionWe report a 23 years 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 had a 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.en-US
dc.descriptionWe report a 23 years 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 had a 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.es-ES
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dc.languagespa
dc.publisherRevista Médica de Chilees-ES
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dc.sourceRevista Médica de Chile; Vol. 146, núm. 12 (2018): DICIEMBRE 2018es-ES
dc.source0034-9887
dc.subjectAutoimmune; Autoimmune Hypophysitis; Hypothyroidism; Polyendocrinopathies; Retroperitoneal Fibrosisen-US
dc.subjectAutoimmune; Autoimmune Hypophysitis; Hypothyroidism; Polyendocrinopathies; Retroperitoneal Fibrosises-ES
dc.titleHYPOPHYSITIS AND RETROPERITONEAL FIBROSIS ASSOCIATED WITH AUTOIMMUNE POLYGLANDULAR SYNDROME. REPORT OF ONE CASEen-US
dc.titleHipofisitis linfoplasmocitaria y fibrosis retroperitoneal asociadas a un síndrome poliglandular autoinmune. Enfermedades relacionadas con IgC4. Caso Clínicoes-ES
dc.typeinfo:eu-repo/semantics/article
dc.typeinfo:eu-repo/semantics/publishedVersion
dc.typees-ES


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