8220 Panhypopituitarism due to Granulomatosis with Polyangiitis - Wegener's Granulomatosis. A Very Rare Cause of Panhypopiturism: A Case Report

Abstract Disclosure: T.D. Bertolacini da Silva: None. V.S. da Silva: None. P.R. Fiorin: None. J.S. Freire: None. C.G. Braga: None. M. Canzian: None. C. Machado: None. Background: Granulomatosis with Polyangiitis (GPA) is a necrotizing granulomatous vasculitis and pituitary involvement is less than 1%. We report a case of pituitary involvement, with CDI, and later panhypopituitarism. Clinical Case: A 43-year-old man was admitted in a hospital due to a headache 3 months ago. CT showed an expansive lesion in posterior fossa and hydrocephaly. Submetted for withdrawal of a Schwannoma and peritoneal shunt, with ipsilateral peripheral facial paralysis. After 1 year, started with polyuria and polydipsia, CDI was diagnosed and DDAVP started. Pituitary MRI showed a small rounded image in the sella turcica, cystic, 0.8 x 0.5 x 0.8cm. After 6 months, readmitted on hospital after trauma in surgical scar, with abscess, accomplished debridement and started oxacillin, with good controls of electrolytes and diuresis: Na 144 mmol/L (137-145 mmol/L). Readmitted after 2 months for surgical wound infection, on that occasion hyponatremia was diagnosed. Other tests were requested: TSH < 0.0015 uIU/mL (0.49-5.58 uIU/mL/mL), free T4 0.68 ng/dL (0.89-1.53 ng/dL), repeated TSH 0.036 uIU/mL and free T4 0.63 ng/dL, central hypothyroidism has been confirmed and levothyroxine started. Pituitary MRI was repeated with enlarged adeno-pituitary: 1.9 x 1.7 x 1.1cm, extension to the suprasellar cistern, compression, and superior displacement of the optic chiasm. Other tests: GH 0.173 ng/mL (< 3 ng/mL), IGF1 66 ng/mL (58-219 ng/mL), testosterone < 7.30 ng/dL (12-59 ng/dL), LH 0.1 mIU/mL (1.9-12.5 mIU/mL), prolactin 0.66 ng/mL (2.1-17.7 ng/mL), ACTH 6.66 pg/mL (< 46.0 pg/mL), confirmed pan-hypopituitarism. After 1-month patient started with petechiae and persisted with hyponatremia, SIADH hypothesis made. Chest CT showed a solid pulmonary nodule, spiculated, 2.6 x 2.1 x 1.8cm, first biopsy was inconclusive. After repeating the chest CT there was persistence the pulmonary nodule 4.2 x 5.8 x 4.4cm. Videothoracoscopy was perfomed and anatomopathological confimed GPA and C-ANCA positive. The patient received prednisone, ivermectin, pulse therapy (methylprednisolone) and cyclophosphamide. After 8 months admitted on hospital due to pneumonia and exogenous Cushing's syndrome, new tests were requested: pituitary MRI showed a hypocontrasting and heterogeneous on the sellar cavity 0.4 x 0.7 x 1.0cm, showing reduction of the lesion after treatment of the rheumatological disease. Patient also received treatment with Rituximab and the last pituitary MRI: hypocontrasting lesion, sometimes with a cystic appearance in the sellar cavity. Currently, in use: testosterone replacement, desmopressin prednisone, levothyroxine, rosuvastatin/ezetimib, cholecalciferol, colchicin and ibandronate sodium. He presented with an improved quality of life. Conclusion: GPA should be considered in cases of CDI and hypopituitarism when sellar disorders were discarded, and if a vasculitis is suspected. Presentation: 6/1/2024

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Journal of the Endocrine Society
Published
2024-10-01
DOI
https://doi.org/10.1210/jendso/bvae163.1291
Primary Topic
Systemic Sclerosis and Related Diseases
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8220 Panhypopituitarism due to Granulomatosis with Polyangiitis - Wegener's Granulomatosis. A Very Rare Cause of Panhypopiturism: A Case Report

Mauro Canzian, Paula Regina Fiorin, Vanessa Santos Dantas da Silva, Juliana Souza Rodrigues Freire et al.
Journal of the Endocrine Society
Systemic Sclerosis and Related Diseases
article

8220 Panhypopituitarism due to Granulomatosis with Polyangiitis - Wegener's Granulomatosis. A Very Rare Cause of Panhypopiturism: A Case Report

Mauro Canzian, Paula Regina Fiorin, Vanessa Santos Dantas da Silva, Juliana Souza Rodrigues Freire, Tiago Donizeti Bertolacini da Silva, Cybelle Machado, Carolina Gabrielli dos Santos Almeida Braga
article en

Abstract

Abstract Disclosure: T.D. Bertolacini da Silva: None. V.S. da Silva: None. P.R. Fiorin: None. J.S. Freire: None. C.G. Braga: None. M. Canzian: None. C. Machado: None. Background: Granulomatosis with Polyangiitis (GPA) is a necrotizing granulomatous vasculitis and pituitary involvement is less than 1%. We report a case of pituitary involvement, with CDI, and later panhypopituitarism. Clinical Case: A 43-year-old man was admitted in a hospital due to a headache 3 months ago. CT showed an expansive lesion in posterior fossa and hydrocephaly. Submetted for withdrawal of a Schwannoma and peritoneal shunt, with ipsilateral peripheral facial paralysis. After 1 year, started with polyuria and polydipsia, CDI was diagnosed and DDAVP started. Pituitary MRI showed a small rounded image in the sella turcica, cystic, 0.8 x 0.5 x 0.8cm. After 6 months, readmitted on hospital after trauma in surgical scar, with abscess, accomplished debridement and started oxacillin, with good controls of electrolytes and diuresis: Na 144 mmol/L (137-145 mmol/L). Readmitted after 2 months for surgical wound infection, on that occasion hyponatremia was diagnosed. Other tests were requested: TSH < 0.0015 uIU/mL (0.49-5.58 uIU/mL/mL), free T4 0.68 ng/dL (0.89-1.53 ng/dL), repeated TSH 0.036 uIU/mL and free T4 0.63 ng/dL, central hypothyroidism has been confirmed and levothyroxine started. Pituitary MRI was repeated with enlarged adeno-pituitary: 1.9 x 1.7 x 1.1cm, extension to the suprasellar cistern, compression, and superior displacement of the optic chiasm. Other tests: GH 0.173 ng/mL (< 3 ng/mL), IGF1 66 ng/mL (58-219 ng/mL), testosterone < 7.30 ng/dL (12-59 ng/dL), LH 0.1 mIU/mL (1.9-12.5 mIU/mL), prolactin 0.66 ng/mL (2.1-17.7 ng/mL), ACTH 6.66 pg/mL (< 46.0 pg/mL), confirmed pan-hypopituitarism. After 1-month patient started with petechiae and persisted with hyponatremia, SIADH hypothesis made. Chest CT showed a solid pulmonary nodule, spiculated, 2.6 x 2.1 x 1.8cm, first biopsy was inconclusive. After repeating the chest CT there was persistence the pulmonary nodule 4.2 x 5.8 x 4.4cm. Videothoracoscopy was perfomed and anatomopathological confimed GPA and C-ANCA positive. The patient received prednisone, ivermectin, pulse therapy (methylprednisolone) and cyclophosphamide. After 8 months admitted on hospital due to pneumonia and exogenous Cushing's syndrome, new tests were requested: pituitary MRI showed a hypocontrasting and heterogeneous on the sellar cavity 0.4 x 0.7 x 1.0cm, showing reduction of the lesion after treatment of the rheumatological disease. Patient also received treatment with Rituximab and the last pituitary MRI: hypocontrasting lesion, sometimes with a cystic appearance in the sellar cavity. Currently, in use: testosterone replacement, desmopressin prednisone, levothyroxine, rosuvastatin/ezetimib, cholecalciferol, colchicin and ibandronate sodium. He presented with an improved quality of life. Conclusion: GPA should be considered in cases of CDI and hypopituitarism when sellar disorders were discarded, and if a vasculitis is suspected. Presentation: 6/1/2024

Journal of the Endocrine SocietyVol. 8(Supplement_1)
D’Or Institute for Research and Education (BR), Faculdade de Medicina do ABC (BR)
Good health and well-being
Openalex Percentile: Top 72%
Systemic Sclerosis and Related Diseases
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