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1.
Int J Mol Sci ; 23(23)2022 Nov 29.
Artigo em Inglês | MEDLINE | ID: mdl-36499283

RESUMO

Autoimmune hypophysitis (AH) is an autoimmune disease of the pituitary for which the pathogenesis is incompletely known. AH is often treated with corticosteroids; however, steroids may lead to considerable side effects. Using a mouse model of AH (experimental autoimmune hypophysitis, EAH), we show that interleukin-1 receptor-associated kinase 1 (IRAK1) is upregulated in the pituitaries of mice that developed EAH. We identified rosoxacin as a specific inhibitor for IRAK1 and found it could treat EAH. Rosoxacin treatment at an early stage (day 0-13) slightly reduced disease severity, whereas treatment at a later stage (day 14-27) significantly suppressed EAH. Further investigation indicated rosoxacin reduced production of autoantigen-specific antibodies. Rosoxacin downregulated production of cytokines and chemokines that may dampen T cell differentiation or recruitment to the pituitary. Finally, rosoxacin downregulated class II major histocompatibility complex expression on antigen-presenting cells that may lead to impaired activation of autoantigen-specific T cells. These data suggest that IRAK1 may play a pathogenic role in AH and that rosoxacin may be an effective drug for AH and other inflammatory diseases involving IRAK1 dysregulation.


Assuntos
Hipofisite Autoimune , Quinases Associadas a Receptores de Interleucina-1 , Autoantígenos , Hipofisite Autoimune/terapia , Quinases Associadas a Receptores de Interleucina-1/antagonistas & inibidores , Animais , Camundongos
2.
Medicine (Baltimore) ; 101(43): e31338, 2022 Oct 28.
Artigo em Inglês | MEDLINE | ID: mdl-36316923

RESUMO

Rheumatic diseases have been reported to sometimes involve the pituitary gland. This study aims to characterize the clinical features and outcomes of patients with rheumatic disease-associated hypophysitis. We used the electronic medical record system in our hospital to identify nine patients with pituitary involvement in rheumatoid disease. We summarized the clinical characteristics, radiographic findings, treatments, and clinical outcomes of the 9 patients. We also performed a systematic literature review of systemic lupus erythematosus (SLE) cases with pituitary involvement published in PubMed and Wanfang databases from 1995 to 2021, and eight patients with complete information were selected. In the nine-patient cohort, the median age was 54 years, and the spectrum of rheumatic diseases included immunoglobulin G4-related disease (IgG4RD) (4/9), SLE (2/9), vasculitis (2/9), and Sjögren syndrome (SS) (1/9). All patients had pituitary abnormalities on radiological assessment, 6 developed diabetes insipidus (DI), and 8 presented with anterior pituitary hormone deficiencies in the disease duration. All the patients had multisystem involvement. As compared to hypophysitis with IgG4RD (IgG4-H), the age at onset of hypophysitis with SLE (SLE-H) patients was younger [(30.4 ± 16.4) years vs. (56.0 ± 0.8) years] and the disease duration was shorter [(14.0 ± 17.5) months vs. (71.0 ± 60.9) months] (P < .05). All patients were managed with glucocorticoids (GC) in combination with another immunosuppressant, and the majority of patients improved within 4 months. Six patients achieved disease remission while four required at least one hormone replacement therapy. Hypophysitis is a rare complication secondary to a variety of various rheumatic diseases that can occur at any stage. GC combined with additional immunosuppressants could improve patients' symptoms; however some patients also required long-term hormone replacement therapy in pituitary disorders.


Assuntos
Hipofisite Autoimune , Doenças do Colágeno , Hipofisite , Hipopituitarismo , Lúpus Eritematoso Sistêmico , Doenças da Hipófise , Doenças Reumáticas , Humanos , Pessoa de Meia-Idade , Adolescente , Adulto Jovem , Adulto , Hipofisite/complicações , Doenças da Hipófise/complicações , Doenças da Hipófise/diagnóstico , Hipopituitarismo/etiologia , Hipófise/diagnóstico por imagem , Glucocorticoides/uso terapêutico , Doenças Reumáticas/complicações , Doenças Reumáticas/tratamento farmacológico , Imunossupressores/uso terapêutico , Doenças do Colágeno/tratamento farmacológico , Lúpus Eritematoso Sistêmico/complicações , Lúpus Eritematoso Sistêmico/tratamento farmacológico , Hipofisite Autoimune/complicações , Hipofisite Autoimune/diagnóstico , Hipofisite Autoimune/tratamento farmacológico
3.
BMC Neurol ; 22(1): 371, 2022 Sep 29.
Artigo em Inglês | MEDLINE | ID: mdl-36175850

RESUMO

BACKGROUND: Autoimmune hypothalamitis is a very rare neuroendocrine disorder that causes central diabetes insipidus, headache, visual impairment, and sometimes cognitive impairment. Autoimmune hypothalamitis may occur in association with autoimmune hypophysitis, including lymphocytic hypophysitis, or in isolation. It is not known whether autoimmune hypothalamitis and autoimmune hypophysitis are consecutive diseases. CASE PRESENTATION: A 52-year-old woman developed autoimmune hypothalamitis 7 years after developing central diabetes insipidus due to lymphocytic hypophysitis, resulting in severe memory impairment. High-dose intravenous methylprednisolone therapy improved her cognitive function and decreased the size of the lesion. CONCLUSION: This case presented a unique clinical course, with a long period of time between the onset of autoimmune hypopituitaritis and the development of autoimmune hypothalamitis.


Assuntos
Hipofisite Autoimune , Diabetes Insípido Neurogênico , Diabetes Insípido , Diabetes Mellitus , Hipofisite Autoimune/complicações , Hipofisite Autoimune/diagnóstico , Hipofisite Autoimune/tratamento farmacológico , Diabetes Insípido/complicações , Diabetes Insípido/diagnóstico , Diabetes Insípido/tratamento farmacológico , Diabetes Insípido Neurogênico/complicações , Diabetes Insípido Neurogênico/diagnóstico , Diabetes Insípido Neurogênico/tratamento farmacológico , Feminino , Humanos , Hipopituitarismo , Imageamento por Ressonância Magnética , Transtornos da Memória/etiologia , Metilprednisolona/uso terapêutico , Pessoa de Meia-Idade
4.
Endocr Pract ; 28(9): 901-910, 2022 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-35779833

RESUMO

OBJECTIVE: Hypophysitis is considered a rare inflammatory disease of the pituitary gland. For a long time, primary autoimmune hypophysitis has stood out as the most relevant type of hypophysitis. However, with the advent of immunotherapy for the treatment of malignancies and identification of hypophysitis as an immune-related adverse event, hypophysitis has garnered increasing interest and recognition. Therefore, awareness, early recognition, and appropriate management are becoming important as the indication for immunomodulatory therapies broaden. METHODS: In this review, we discuss the epidemiology, diagnosis, and treatment of hypophysitis with a focus on recent data and highlight subtypes of particular interest while recognizing the gaps in knowledge that remain. RESULTS: Regardless of cause, symptoms and signs of hypophysitis may be related to mass effect (headache and visual disturbance) and hormonal disruption that warrant prompt evaluation. In the vast majority of cases, a diagnosis of hypophysitis can be made presumptively in the appropriate clinical context with radiologic findings consistent with hypophysitis and after the exclusion of other causes. CONCLUSION: Although subtle differences currently exist in management and outcome expectations between primary and secondary causes of hypophysitis, universally, treatment is aimed at symptom management and hormonal replacement therapy.


Assuntos
Hipofisite , Hipofisite Autoimune/diagnóstico , Hipofisite Autoimune/epidemiologia , Hipofisite Autoimune/etiologia , Cefaleia/patologia , Terapia de Reposição Hormonal , Humanos , Hipofisite/diagnóstico , Hipofisite/epidemiologia , Hipofisite/etiologia , Hipófise/patologia
5.
Rev Esp Patol ; 55(3): 203-206, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-35779888

RESUMO

Hypophysitis is a rare disease of pituitary gland, which, although it is usually a primary lesion, can also occur secondary to systemic conditions. Granulomatous hypophysitis is an inflammatory disease condition which accounts for less than 1% of all cellular lesions and can mimic adenoma. A 32-year-old woman presented with weight gain, galactorrhea and blurred vision. The MRI showed a cystic, nodular lesion in the intermediate lobe of the pituitary gland and the initial diagnosis was adenoma. She underwent surgery and the histopathology revealed granulomas composed of epithelioid histiocytes, multinuclear giant cells and mononuclear inflammatory cells. Inflammatory diseases of the pituitary gland are much less frequent than pituitary adenomas and idiopathic granulomatous hypophysitis is extremely rare. Histopathology and the ruling out of a systemic cause are the gold standards for its diagnosis.


Assuntos
Adenoma , Hipofisite Autoimune , Galactorreia , Hipofisite , Neoplasias Hipofisárias , Adulto , Feminino , Humanos , Neoplasias Hipofisárias/diagnóstico , Gravidez
6.
Rev. esp. patol ; 55(3): 203-206, jul.-sep. 2022. ilus
Artigo em Inglês | IBECS | ID: ibc-206796

RESUMO

Hypophysitis is a rare disease of pituitary gland, which, although it is usually a primary lesion, can also occur secondary to systemic conditions. Granulomatous hypophysitis is an inflammatory disease condition which accounts for less than 1% of all cellular lesions and can mimic adenoma. A 32-year-old woman presented with weight gain, galactorrhea and blurred vision. The MRI showed a cystic, nodular lesion in the intermediate lobe of the pituitary gland and the initial diagnosis was adenoma. She underwent surgery and the histopathology revealed granulomas composed of epithelioid histiocytes, multinuclear giant cells and mononuclear inflammatory cells.Inflammatory diseases of the pituitary gland are much less frequent than pituitary adenomas and idiopathic granulomatous hypophysitis is extremely rare. Histopathology and the ruling out of a systemic cause are the gold standards for its diagnosis.(AU)


La hipofisitis es una enfermedad rara de la glándula pituitaria, y a pesar de ser fundamentalmente una enfermedad primaria, puede ser también secundaria a enfermedades sistémicas. La hipofisitis granulomatosa es una enfermedad inflamatoria que representa menos del 1% de todas las lesiones celulares, y remedar al adenoma. Mujer de 32 años de edad ingresada en el hospital con aumento de peso, galactorrea y visión borrosa. La RM reflejó una lesión quística y nodular en el lóbulo intermedio de la glándula pituitaria, que fue operada con diagnóstico primario de adenoma. El examen microscópico reveló granulomas formados por histiocitos epitelioides, células gigantes multinucleares y células inflamatorias mononucleares. Las enfermedades inflamatorias de la glándula pituitaria son muy raras en comparación con los adenomas pituitarios. La hipofisitis granulomatosa idiopática es una de ellas. El criterio de referencia para este diagnóstico es realizar un examen histopatológico, y descartar una causa sistémica.(AU)


Assuntos
Humanos , Feminino , Adulto , Hipofisite Autoimune , Adenoma , Galactorreia , Neoplasias Hipofisárias , Hipofisite
7.
Intern Med ; 61(23): 3541-3545, 2022 Dec 01.
Artigo em Inglês | MEDLINE | ID: mdl-35569983

RESUMO

Pituitary inflammation due to IgG4-related disease is a rare condition and is sometimes accompanied by central diabetes insipidus. Central diabetes insipidus produces a strong thirst sensation, which may be difficult to distinguish when complicated by salivary insufficiency. A 45-year-old man was admitted to our department for a thorough examination of his thirst and polyuria. He had suddenly developed these symptoms more than one year earlier and visited an oral surgeon. Swelling of the left submandibular gland, right parotid gland, and cervical lymph nodes had been observed. Since his IgG4 level was relatively high at 792 mg/dL and a lip biopsy showed high plasmacytoid infiltration around the gland ducts, he had been diagnosed with IgG4-related disease. He had started taking 0.4 mg/kg/day of prednisolone, and his chief complaint temporarily improved. However, since the symptom recurred, he was referred to our institution. After admission, to examine the cause of his thirst and polyuria, we performed a water restriction test, vasopressin loading test, hypertonic saline loading test and pituitary magnetic resonance imaging. Based on the findings, we diagnosed him with central diabetes insipidus due to IgG4-related hypophysitis. We increased the dose of prednisolone to 0.6 mg/kg/day and started 10 µg/day of intranasal desmopressin. His symptoms were subsequently alleviated, and his serum IgG4 level finally normalized. We should remember that IgG4-related disease can be accompanied by hypophysitis and that central diabetes insipidus is brought about by IgG4-related hypophysitis. This case report should remind physicians of the fact that pituitary inflammation due to IgG4-related disease is very rare and can be masked by symptoms due to salivary gland inflammation, which can lead to pitfalls in the diagnosis in clinical practice.


Assuntos
Hipofisite Autoimune , Diabetes Insípido Neurogênico , Diabetes Insípido , Diabetes Mellitus , Doença Relacionada a Imunoglobulina G4 , Doenças da Hipófise , Sialadenite , Masculino , Humanos , Pessoa de Meia-Idade , Hipofisite Autoimune/complicações , Hipofisite Autoimune/diagnóstico , Diabetes Insípido Neurogênico/complicações , Diabetes Insípido Neurogênico/diagnóstico , Doença Relacionada a Imunoglobulina G4/complicações , Poliúria , Doenças da Hipófise/diagnóstico , Prednisolona/uso terapêutico , Sialadenite/complicações , Sialadenite/diagnóstico , Inflamação/complicações , Imunoglobulina G , Diabetes Insípido/complicações , Diabetes Insípido/diagnóstico
8.
Best Pract Res Clin Endocrinol Metab ; 36(3): 101663, 2022 05.
Artigo em Inglês | MEDLINE | ID: mdl-35525821

RESUMO

Recent advances in tumor immunology have enabled to develop immune checkpoint inhibitors and to save many patients with advanced cancer. Various endocrine diseases are caused by autoimmune mechanisms; it is important to understand that many endocrine organs are targeted by autoimmunity. In addition, some of malignant tumors ectopically produce hormones and lead to a clinically relevant ectopic hormone syndrome as an endocrine paraneoplastic syndrome. Thus, interplay between oncology, immunology, and endocrinology is important. We have defined a new clinical entity "paraneoplastic autoimmune hypophysitis" as "hypophysitis associated with autoimmunity against pituitary antigens caused by an ectopic expression of these antigens in the tumor and resulting in a breakdown of immune tolerance". To understand the pathophysiology of paraneoplastic autoimmune hypophysitis requires a fusion of the fields of oncology, immunology, and endocrinology; therefore, I propose a new concept of Onco-Immuno-Endocrinology.


Assuntos
Hipofisite Autoimune , Doenças do Sistema Endócrino , Endocrinologia , Hipofisite , Neoplasias , Doenças da Hipófise , Autoanticorpos , Hipofisite Autoimune/diagnóstico , Humanos , Hipofisite/diagnóstico , Hipofisite/etiologia , Doenças da Hipófise/complicações , Doenças da Hipófise/diagnóstico
9.
Acta Neurochir (Wien) ; 164(8): 2095-2103, 2022 08.
Artigo em Inglês | MEDLINE | ID: mdl-35532784

RESUMO

PURPOSE: IgG4-related hypophysitis (IgG4-RH) is a rare chronic inflammatory condition of the pituitary gland. This study reports the presentation, management and outcomes for patients with histologically proven IgG4-related hypophysitis. METHODS: A prospectively maintained electronic database was searched over a 14-year period from 1 January 2007 to 31 December 2020 at a single academic centre to identify all patients with a histological diagnosis of IgG4-RH. A retrospective case note review from electronic health records was conducted for each case to extract data on their presentation, management and outcomes. RESULTS: A total of 8 patients (5 male) with a median age of 51 years were identified. The most common presenting symptoms were headache (4/8; 50%), fatigue (3/8; 37.5%) and visual impairment (2/8; 25%). Three patients were initially treated with high-dose steroids aiming for reduction of the pituitary mass. However, ultimately all patients underwent transsphenoidal surgery. Post-operative changes included radiological reduction in pituitary mass in all patients that had imaging (7/7; 100%), improvement in vision (1/2; 50%), residual thick pituitary stalk (5/7; 71.4%), persistent anterior hypopituitarism (4/8; 50%) and panhypopopituitarism including diabetes insipidus (3/8; 37.5%). CONCLUSIONS: IgG4-RH is an increasingly recognised entity presenting with a variety of symptoms and signs. Clinical presentation is similar to other forms of hypophysitis. It is therefore important to consider IgG4-RH as a differential and to have a low threshold for pituitary biopsy, the diagnostic gold standard. The diagnosis of IgG4-RH will guide decisions for additional workup for IgG4-related disease, multi-disciplinary team involvement and follow-up.


Assuntos
Hipofisite Autoimune , Doenças da Hipófise , Hipofisite Autoimune/diagnóstico , Hipofisite Autoimune/patologia , Humanos , Imunoglobulina G/metabolismo , Imunoglobulina G/uso terapêutico , Masculino , Pessoa de Meia-Idade , Hipófise/diagnóstico por imagem , Hipófise/cirurgia , Estudos Retrospectivos
10.
Sci Rep ; 12(1): 4440, 2022 03 15.
Artigo em Inglês | MEDLINE | ID: mdl-35292721

RESUMO

Central diabetes insipidus (CDI) is a rare condition caused by various underlying diseases including inflammatory and autoimmune diseases, and neoplasms. Obtaining an accurate definitive diagnosis of the underlying cause of CDI is difficult. Recently, anti-rabphilin-3A antibodies were demonstrated to be a highly sensitive and specific marker of lymphocytic infundibuloneurohypophysitis (LINH). Here, we report a detailed case series, and evaluated the significance of anti-rabphilin-3A antibodies in differentiating the etiologies of CDI. A prospective analysis was conducted in 15 consecutive patients with CDI from 2013 to 2020 at a single referral center. Anti-rabphilin-3A antibodies were measured and the relationship between antibody positivity and the clinical/histopathological diagnoses was evaluated. Among 15 CDI patients, the positive anti-rabphilin-3A antibodies were found in 4 of 5 LINH cases, 3 of 4 lymphocytic panhypophysitis (LPH) cases, one of 2 sarcoidosis cases, and one intracranial germinoma case, respectively. Two Rathke cleft cyst cases and one craniopharyngioma case were negative. This is the first report of anti-rabphilin-3A antibodies positivity in CDI patients with biopsy-proven LPH. Measurement of anti-rabphilin-3A antibodies may be valuable for differentiating CDI etiologies.


Assuntos
Doenças Autoimunes , Hipofisite Autoimune , Diabetes Insípido Neurogênico , Diabetes Mellitus , Sarcoidose , Doenças Autoimunes/complicações , Diabetes Insípido Neurogênico/diagnóstico , Diabetes Insípido Neurogênico/etiologia , Feminino , Humanos , Masculino , Encaminhamento e Consulta , Sarcoidose/complicações
13.
J Pediatr Endocrinol Metab ; 35(5): 687-690, 2022 May 25.
Artigo em Inglês | MEDLINE | ID: mdl-35119230

RESUMO

OBJECTIVES: A highly invasive pathological diagnosis is necessary to differentiate central diabetes insipidus (CDI) with a thickened pituitary stalk. Lymphocytic infundibulo-hypophysitis (LIH) due to autoimmune involvement of the pituitary stalk is a differentiating disease, and anti-rabphilin-3A antibody (Rab3A-Ab) positivity was recently reported. CASE PRESENTATION: A 7-year-old boy was diagnosed with CDI after having polyuria for two months. He showed growth hormone deficiency with reduced growth rate. Brain magnetic resonance imaging (MRI) revealed a thickened pituitary stalk. The placental alkaline phosphatase level of the cerebrospinal fluid, tumor marker for germ cell tumors, was below the level of sensitivity. No skin or bone findings suggestive of Langerhans cell histiocytosis were detected. Eight months after CDI onset, Rab3A-Ab was positive, and MRI showed shrinking of the thickened pituitary stalk, leading to the diagnosis with LIH. CONCLUSIONS: Rab3A-Ab is a useful adjunctive diagnostic tool for childhood-onset LIH.


Assuntos
Hipofisite Autoimune , Diabetes Insípido Neurogênico , Diabetes Insípido , Diabetes Mellitus , Hipofisite Autoimune/diagnóstico , Criança , Diabetes Insípido/patologia , Diabetes Insípido Neurogênico/diagnóstico , Diabetes Insípido Neurogênico/etiologia , Diabetes Mellitus/patologia , Feminino , Humanos , Hipopituitarismo , Imageamento por Ressonância Magnética , Masculino , Hipófise/diagnóstico por imagem , Hipófise/patologia , Placenta/patologia , Gravidez
14.
J Clin Endocrinol Metab ; 107(4): 1170-1190, 2022 03 24.
Artigo em Inglês | MEDLINE | ID: mdl-35137155

RESUMO

CONTEXT: Lymphocytic hypophysitis (LyHy) is characterized by inflammation of the pituitary and or neuroinfundibulum and is uncommon. Treatment options include observation, high-dose glucocorticoids (HD-GCs) or surgery. Optimal first-line management strategy, however, remains unknown. OBJECTIVE: This work aimed to assess response to first-line treatment options (observation, HD-GCs, or surgery) of clinically relevant outcomes (symptomatic, hormonal, and radiographic improvement) among patients with LyHy. METHODS: A systematic review was conducted in 6 databases through 2020. Meta-analysis was conducted when feasible using a random-effects model. RESULTS: We included 33 studies reporting on 591 patients (423 women, 72%) with LyHy. Improvement/resolution of anterior pituitary dysfunction was highest when HD-GCs was first-line treatment. Surgery was associated with the greatest proportion of patients who had regression on imaging. Subgroup analysis comparing HD-GCs to observation showed the odds of anterior pituitary hormone recovery (OR 3.41; 95% CI, 1.68-6.94) or radiographic regression (OR 3.13; 95% CI, 1.54-6.36) were higher with HD-GCs, but so was the need for additional forms of treatment (OR 4.37; 95% CI, 1.70-11.22). No statistically significant difference was seen in recovery of diabetes insipidus (OR 0.9; 95% CI, 0.26-3.10). Certainty in these estimates was very low. CONCLUSION: Observation and use of HD-GCs both are successful first-line management strategies in LyHy. Although use of HD-GCs was associated with increased recovery of anterior pituitary hormone deficit, it also was associated with greater likelihood of additional treatment after withdrawal. Optimal dosing and duration of HD-GCs remains unknown.


Assuntos
Hipofisite Autoimune , Hipopituitarismo , Doenças da Hipófise , Hormônios Adeno-Hipofisários , Hipofisite Autoimune/complicações , Hipofisite Autoimune/tratamento farmacológico , Feminino , Glucocorticoides/uso terapêutico , Humanos , Hipopituitarismo/tratamento farmacológico , Masculino , Doenças da Hipófise/complicações , Doenças da Hipófise/terapia
15.
BMC Endocr Disord ; 22(1): 23, 2022 Jan 15.
Artigo em Inglês | MEDLINE | ID: mdl-35033046

RESUMO

BACKGROUND: The differential diagnosis of IgG4-related hypophysitis and other inflammatory diseases or tumors involving sellar region is challenging even after sellar biopsy. Sellar germinoma is usually infiltrated by lymphocytes or plasma cells, and may be confused with hypophysitis. CASE PRESENTATION: A 36-year-old man with diabetes insipidus, elevated serum IgG4 level (336 mg/dl), and sellar mass was suspected to have IgG4-related hypophysitis, and no other lesion of IgG4-related disease was detected. After treated by prednisone and mycophenolate mofetil, the serum IgG4 decreased to 214 mg/dl. However, after withdrawal of the drugs, the IgG4 level increased to 308 mg/dl. Endocrine assessments revealed panhypopituitarism, and the sellar mass enlarged. Transsphenoidal sellar exploration and biopsy was conducted. Pathological examination showed that the lesion was germinoma with lymphocytes and plasma cells infiltration, and IgG4-staining was positive (70/HPF, IgG4/IgG ratio = 10%). The patient was then treated by cisplatin and etoposide. After four cycles of chemotherapy, the serum IgG4 was 201 mg/dl, and the sellar mass was invisible. CONCLUSION: Sellar germinoma can mimic the clinical characteristics of IgG4-related hypophysitis. Poor response to glucocorticoids can be used as an exclusion criterion in the clinical diagnosis of IgG4-related hypophysitis.


Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Neoplasias Encefálicas/diagnóstico , Germinoma/diagnóstico , Sela Túrcica , Adulto , Hipofisite Autoimune/diagnóstico , Neoplasias Encefálicas/tratamento farmacológico , Cisplatino/administração & dosagem , Diagnóstico Diferencial , Etoposídeo/administração & dosagem , Germinoma/tratamento farmacológico , Humanos , Imunoglobulina G/sangue , Masculino
16.
Pituitary ; 25(2): 321-327, 2022 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-35088194

RESUMO

PURPOSE: To explore the clinical significance of anti-rabphillin-3A antibody for the differential diagnosis of lymphocytic panhypophysitis. METHODS AND RESULTS: A 58-year-old Japanese man developed uveitis of unknown cause in 2017. In 2019, he became aware of polyuria. In August 2020, he noticed transient diplopia and was diagnosed with right abducens nerve palsy. At the same time, he complained of fatigue and loss of appetite. Head magnetic resonance imaging demonstrated enlargement of the pituitary stalk and pituitary gland, corresponding to hypophysitis. Hormone stimulation tests showed blunted responses with respect to all anterior pituitary hormones. Central diabetes insipidus was diagnosed on the basis of a hypertonic saline loading test. Taking these findings together, a diagnosis of panhypopituitarism was made. Computed tomography showed enlargement of hilar lymph nodes. Biopsies of the hilar lymph nodes revealed non-caseating epithelioid cell granulomas that were consistent with sarcoidosis. Biopsy of the anterior pituitary revealed mild lymphocyte infiltration in the absence of IgG4-positive cells, non-caseating granulomas, or neoplasia. Western blotting revealed the presence of anti-rabphilin-3A antibody, supporting a diagnosis of lymphocytic panhypophysitis. Because the patient had no visual impairment or severe uveitis, we continued physiological hormone replacement therapy and topical steroid therapy for the uveitis. CONCLUSION: To the best of our knowledge, this is the first case of anti-rabphilin 3A antibody positive lymphocytic panhypophysitis comorbid with sarcoidosis, diagnosed by both pituitary and hilar lymph node biopsy. The utility of anti-rabphilin-3A antibody for the differential diagnosis of hypophysitis like this case should be clarified with further case studies.


Assuntos
Hipofisite Autoimune , Diabetes Insípido Neurogênico , Hipopituitarismo , Sarcoidose Pulmonar , Sarcoidose , Hipofisite Autoimune/diagnóstico , Hipofisite Autoimune/tratamento farmacológico , Diabetes Insípido Neurogênico/diagnóstico , Humanos , Hipopituitarismo/diagnóstico , Masculino , Pessoa de Meia-Idade , Hipófise/patologia , Sarcoidose/complicações , Sarcoidose/tratamento farmacológico , Sarcoidose/patologia , Sarcoidose Pulmonar/complicações , Sarcoidose Pulmonar/patologia
17.
Neuroendocrinology ; 112(2): 186-195, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-33744880

RESUMO

INTRODUCTION: The role of glucocorticoids in primary autoimmune hypophysitis (PAH) has been fraught with variability in regimens, leading to inconsistent outcomes in terms of anterior pituitary (AP) hormonal recovery. Hence, we aimed to compare the clinical, hormonal, and radiological outcomes of a standardized high-dose glucocorticoid therapy group (GTG) in PAH with a matched clinical observation group (COG). METHODS: Thirty-nine retrospective patients with PAH evaluated and treated at a single center in western India from 1999 to 2019 with a median follow-up duration of 48 months were subdivided into the GTG (n = 18) and COG (n = 21) and compared for the outcomes. RESULTS: Baseline demographic, hormonal, and radiological features matched between the groups, except pituitary height, which was significantly higher in GTG. Cortisol, thyroid, and gonadal axes were affected in 25 (64%), 22 (56%), and 21 (54%) patients, respectively, and central diabetes insipidus was seen in 7 (18%) patients. Panhypophysitis (PH) was the most common radiological subtype (n = 33, 84.6%). Resolution of mass effects was similar in both groups. Overall and complete AP hormonal recovery was significantly higher in the GTG than in the COG (12/14 [85.7%) vs. 6/14 [42.8%], p = 0.02; 10/14 [71.4%] vs. 1/14 [7.7%], p = 0.0007, respectively). Proportion of cases with empty sella were significantly higher in the COG (9/20 [45%] vs 1/17 [5.9%], p = 0.001). Among PH patients in the GTG (n = 17), we found duration from symptoms onset to treatment as the predictor of recovery. CONCLUSION: In a PH subtype-predominant PAH cohort, a standardized high-dose glucocorticoid regimen resulted in higher overall and complete AP hormonal recovery than that in the COG. Initiation of glucocorticoids in the early disease course may have been contributory.


Assuntos
Hipofisite Autoimune/tratamento farmacológico , Hipofisite Autoimune/metabolismo , Glucocorticoides/farmacologia , Adulto , Intervenção Médica Precoce , Feminino , Seguimentos , Glucocorticoides/administração & dosagem , Humanos , Masculino , Pessoa de Meia-Idade , Avaliação de Resultados em Cuidados de Saúde , Estudos Retrospectivos , Adulto Jovem
19.
Best Pract Res Clin Endocrinol Metab ; 36(3): 101601, 2022 05.
Artigo em Inglês | MEDLINE | ID: mdl-34876362

RESUMO

Pituitary autoimmunity is one of the principal causes of hypopituitarism. Additionally, hypophysitis is one of the immune-related adverse events associated with immunotherapy. Recent case-oriented research has revealed a novel type of autoimmune hypophysitis, anti-PIT-1 hypophysitis, related to isolated adrenocorticotropic hormone (ACTH) deficiency and immune checkpoint inhibitor-related hypophysitis, as a form of paraneoplastic syndrome. Under these conditions, the ectopic expression of pituitary antigens present in tumors evokes a breakdown of immune tolerance, resulting in the production of autoantibodies and autoreactive cytotoxic T cells that specifically harm pituitary cells. Consequently, an innovative clinical entity of paraneoplastic autoimmune hypophysitis has been purported. This novel concept and its underlying mechanisms provide clues for understanding the pathogenesis of autoimmune pituitary diseases and can be applied to other autoimmune diseases. This review discusses the etiology of paraneoplastic autoimmune hypophysitis and its future.


Assuntos
Doenças Autoimunes , Hipofisite Autoimune , Hipofisite , Doenças da Hipófise , Hormônio Adrenocorticotrópico/deficiência , Autoanticorpos , Doenças do Sistema Endócrino , Doenças Genéticas Inatas , Humanos , Hipoglicemia , Hipofisite/etiologia , Doenças da Hipófise/etiologia
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