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P M J Zelissen - One of the best experts on this subject based on the ideXlab platform.
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does recovery of adrenal function occur in patients with autoimmune addison s disease
Clinical Endocrinology, 2011Co-Authors: L C C J Smans, P M J ZelissenAbstract:OBJECTIVE: We earlier discovered partial recovery in a patient with autoimmune Addison's disease. The aim of this study was to assess the occurrence of adrenocortical recovery in patients with autoimmune Adrenalitis. DESIGN: Cross-sectional study. PATIENTS: Twenty-seven adult patients with autoimmune Addison's disease on stable glucocorticoid and mineralocorticoid replacement therapy (RT) attending the Department of Endocrinology of a university teaching hospital were included in this study. METHODS: Adrenocortical function was assessed by performing an adrenocorticotrophic hormone (ACTH) (250 μg Synacthen) stimulation test (SST) after interruption of current glucocorticoid and mineralocorticoid RT. A normal adrenal response was defined as a serum cortisol concentration ≥500 nm 30 or 60 min after stimulation. Partial recovery was defined as a cortisol concentration ≥100 and ≤500 nm after stimulation. RESULTS: In 17 patients (63%), serum cortisol concentrations remained undetectable 30 and 60 min after the administration of ACTH. None of the remaining 10 participants had a normal response. Only one patient reached a cortisol concentration of 100 nm after 60 min, but this could not be confirmed during a second SST. CONCLUSIONS: In this cross-sectional study among 27 patients with autoimmune Adrenalitis, no new cases of adrenocortical recovery were found.
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partial recovery of adrenal function in a patient with autoimmune addison s disease
Journal of Endocrinological Investigation, 2008Co-Authors: L C C J Smans, P M J ZelissenAbstract:Objective: To our knowledge, no case of remission in autoimmune Addison’s disease has previously been reported. We describe a patient with primary adrenal insufficiency caused by autoimmune Adrenalitis in whom partial remission was observed after 7 yr. Case: A 39-yr-old male was referred because of extreme fatigue, weight loss, anorexia, nausea, and bouts of fever. During physical examination hyperpigmentation was seen. Laboratory tests showed a plasma cortisol of 0.02 µmol/l (08:30 h). Cortisol failed to increase during the ACTH stimulation test (0.02 to 0.03 µmol/l) and ACTH was markedly elevated (920 pmol/l). Adrenal auto-antibodies were weakly positive. A CT-scan showed no evidence of calcifications or other abnormalities of the adrenal glands. The diagnosis of autoimmune Addison’s disease was made and replacement therapy with hydrocortisone and fludrocortisone was started. During the following years the dose of hydrocortisone was gradually decreased. Eventually, the patient decided to stop his medication completely. A repeated ACTH-stimulation test revealed a basal cortisol of 0.25 µmol/l and a peak cortisol of 0.30 µmol/l with a basal ACTH of 178 pmol/l. The patient did not have any complaints. Conclusion: Recovery of adrenal insufficiency, due to causes other than autoimmune Adrenalitis, has been reported in the past. If our case of partial recovery of autoimmune Adrenalitis is not unique this could have profound effects on treatment and follow-up of Addison’s disease.
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associated autoimmunity in addison s disease
Journal of Autoimmunity, 1995Co-Authors: P M J Zelissen, Egbert J E G Bast, Ronald J M CroughsAbstract:As the last extensive series of patients with Addison's disease and coincident autoimmune phenomena were published approximately two decades ago, we studied the cause of the disease, the prevalence of autoimmune disorders and the frequency of occurrence of autoantibodies in 91 patients (31 men and 60 women, mean age 45.3-years-old, range 12-77) with Addison's disease. The cause of Addison's disease in six patients was tuberculosis (6.6%), and autoimmune Adrenalitis was considered to be the cause in 83 patients (91.2%). In two patients (2.2%) other causes were responsible for Addison's disease. In 47% of the patients with autoimmune Addison's disease at least one other autoimmune disorder was present. Primary hypothyroidism had the highest prevalence (20.5%), followed by vitiligo (9.6%), non-toxic goiter (8.4%), premature menopause (7.3% of the women), Graves' disease (6%), pernicious anaemia (4.8%), Sjogren's disease (2.4%), hypoparathyroidism (1.2%), type 1 diabetes mellitus (1.2%) and coeliac disease (1.2%). The frequency of autoantibodies in the patients with autoimmune Addison's disease was: adrenal antibodies (82.7%), antibodies against microsomal antigens (58%), thyroglobulin antibodies (23.4%), parietal cell antibodies (19.8%), pancreatic islet cell antibodies (6.2%) and ovary antibodies (3.7% of the women). In comparison with other extensive series of patients with Addison's disease, we found the highest prevalence of autoimmune Adrenalitis as the cause of Addison's disease, the highest prevalence of hypothyroidism and vitiligo as concomitant autoimmune disorders and the lowest prevalence of type 1 diabetes mellitus.
Susanna I Lee - One of the best experts on this subject based on the ideXlab platform.
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abdominal immune related adverse events detection on ultrasonography ct mri and 18f fluorodeoxyglucose positron emission tomography
British Journal of Radiology, 2021Co-Authors: Mark Anderson, Vikram Kurra, William R Bradley, Aoife Kilcoyne, Amirkasra Mojtahed, Susanna I LeeAbstract:Immune checkpoint inhibitor and chimeric antigen receptor T-cell therapies are associated with a unique spectrum of complications termed immune-related adverse events (irAEs). The abdomen is the most frequent site of severe irAEs that require hospitalization with life-threatening consequences. Most abdominal irAEs such as enterocolitis, hepatitis, cholangiopathy, cholecystitis, pancreatitis, Adrenalitis, and sarcoid-like reaction are initially detected on imaging such as ultrasonography (US), CT, MRI and fusion 18F-fluorodeoxyglucose (FDG) positron emission tomography (PET)-CT during routine surveillance of cancer therapy. Early recognition and diagnosis of irAEs and immediate management with cessation of immune modulator cancer therapy and institution of immunosuppressive therapy are necessary to avert morbidity and mortality. Diagnosis of irAEs is confirmed by tissue sampling or by follow-up imaging demonstrating resolution. Abdominal radiologists reviewing imaging on patients being treated with anti-cancer immunomodulators should be familiar with the imaging manifestations of irAEs.
Daniel Rimoldi - One of the best experts on this subject based on the ideXlab platform.
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Seric 21-hydroxilase antibodies in patients with anti-microsomal fraction antibodies. Autoimmune polyendocrine syndrome
Medicina, 2007Co-Authors: Silvia Botta, Silvana Roveto, Daniel RimoldiAbstract:Autoimmune polyendocrine syndrome (APS) is the association of autoimmune endocrine diseases, with other autoimmune nonendocrine disorders. APS types 1, 2 and 4 include autoimmune Adrenalitis; this suggests the presence of autoantibodies. A specific serological marker for these is the anti 21- hydroxilase autoantibody (a21-OH). APS type 2 is the association of autoimmune Adrenalitis, to autoimmune thyroid disease and/or diabetes mellitus, all these are induced by autoantibodies. Alopecia, vitiligo, myasthenia and other manifestations can be minor components. We sought to establish the prevalence of seric a21-OH in patients with positive anti-microsomal fraction autoantibodies, autoimmune thyroid disease and/or non-endocrine autoimmune diseases. We also aimed to diagnose incomplete forms of APS and to follow up patients at risk of progression to complete forms of APS. A population of 72 patients and another of 60 controls with negative anti-microsomal fraction autoantibodies were studied. Elevated seric a21-OH were found in two patients. Patient A with 47 U/ml had autoimmune hypothyroidism and myasthenia; and patient B with 8.75 U/ml had autoimmune hypothyrodism and vitiligo; they both lacked adrenal insufficiency. Seric a21-OH had a prevalence of 2.8%. Regarding the adrenal component, patients A and B had an incomplete and latent APS type 2. Considering a21-OH as markers of latent endocrine autoimmune diseases and taking into account the eventual risk of developing clinical manifestations, periodic biochemical and clinical follow-ups are recommended.
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Anticuerpos anti 21 hidroxilasa séricos en pacientes con anticuerpos antifracción microsomal: Síndrome poliendocrino autoinmune Seric 21- hydroxilase antibodies in patients with anti-microsomal fraction antibodies: Autoimmune polyendocrine syndrome
Fundación Revista Medicina, 2007Co-Authors: Silvia Botta, Silvana Roveto, Daniel RimoldiAbstract:El síndrome poliendocrino autoinmune (SPA) es la asociación de enfermedades endocrinas autoinmunes con otros desórdenes autoinmunes no endocrinos. Los tipos 1, 2 y 4 presentan Adrenalitis autoinmune, esto indica la presencia de autoanticuerpos, y su marcador serológico específico es el anti 21 hidroxilasa (a21-OH). El SPA tipo 2 es la asociación de Adrenalitis, enfermedad tiroidea y/o diabetes mellitus inducidas por autoanticuerpos. Como componentes menores, pueden estar asociados entre otros, vitiligo, alopecia y miastenia. Nuestros objetivos fueron: establecer la prevalencia de a21-OH séricos en pacientes con anticuerpos anti fracción microsomal (AFM) positivos, enfermedad tiroidea autoinmune y/o afecciones endocrinas y no endocrinas autoinmunes; diagnosticar formas incompletas de SPA y estudiar individuos con probable riesgo de progresión a un SPA completo. Estudiamos 72 pacientes AFM positivos y 60 sujetos tomados como grupo control, AFM negativos. Hallamos a21-OH elevados en dos pacientes: A= 47 U/ml, hipotiroidismo autoinmune y miastenia; y B= 8.75 U/ml, hipotiroidismo autoinmune y vitiligo; ambos con ausencia de insuficiencia adrenal. La prevalencia de a21-OH encontrada fue del 2.8%. Las pacientes A y B corresponden a un SPA tipo 2 incompleto y latente en relación al componente adrenal. Considerando a los a21-OH marcadores de enfermedad autoinmune latente, el eventual riesgo de evolución hacia la afección clínica sugiere la necesidad de estrechos controles clínicos y bioquímicos periódicos.Autoimmune polyendocrine syndrome (APS) is the association of autoimmune endocrine diseases, with other autoimmune nonendocrine disorders. APS types 1, 2 and 4 include autoimmune Adrenalitis; this suggests the presence of autoantibodies. A specific serological marker for these is the anti 21- hydroxilase autoantibody (a21-OH). APS type 2 is the association of autoimmune Adrenalitis, to autoimmune thyroid disease and/or diabetes mellitus, all these are induced by autoantibodies. Alopecia, vitiligo, myasthenia and other manifestations can be minor components. We sought to establish the prevalence of seric a21-OH in patients with positive anti-microsomal fraction autoantibodies, autoimmune thyroid disease and/or non-endocrine autoimmune diseases. We also aimed to diagnose incomplete forms of APS and to follow up patients at risk of progression to complete forms of APS. A population of 72 patients and another of 60 controls with negative anti-microsomal fraction autoantibodies were studied. Elevated seric a21-OH were found in two patients. Patient A with 47 U/ml had autoimmune hypothyroidism and myasthenia; and patient B with 8.75 U/ml had autoimmune hypothyrodism and vitiligo; they both lacked adrenal insufficiency. Seric a21-OH had a prevalence of 2.8%. Regarding the adrenal component, patients A and B had an incomplete and latent APS type 2. Considering a21-OH as markers of latent endocrine autoimmune diseases and taking into account the eventual risk of developing clinical manifestations, periodic biochemical and clinical follow-ups are recommended
Mark Anderson - One of the best experts on this subject based on the ideXlab platform.
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abdominal immune related adverse events detection on ultrasonography ct mri and 18f fluorodeoxyglucose positron emission tomography
British Journal of Radiology, 2021Co-Authors: Mark Anderson, Vikram Kurra, William R Bradley, Aoife Kilcoyne, Amirkasra Mojtahed, Susanna I LeeAbstract:Immune checkpoint inhibitor and chimeric antigen receptor T-cell therapies are associated with a unique spectrum of complications termed immune-related adverse events (irAEs). The abdomen is the most frequent site of severe irAEs that require hospitalization with life-threatening consequences. Most abdominal irAEs such as enterocolitis, hepatitis, cholangiopathy, cholecystitis, pancreatitis, Adrenalitis, and sarcoid-like reaction are initially detected on imaging such as ultrasonography (US), CT, MRI and fusion 18F-fluorodeoxyglucose (FDG) positron emission tomography (PET)-CT during routine surveillance of cancer therapy. Early recognition and diagnosis of irAEs and immediate management with cessation of immune modulator cancer therapy and institution of immunosuppressive therapy are necessary to avert morbidity and mortality. Diagnosis of irAEs is confirmed by tissue sampling or by follow-up imaging demonstrating resolution. Abdominal radiologists reviewing imaging on patients being treated with anti-cancer immunomodulators should be familiar with the imaging manifestations of irAEs.
Jozelio Freire De Carvalho - One of the best experts on this subject based on the ideXlab platform.
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diagnosis and classification of addison s disease autoimmune Adrenalitis
Autoimmunity Reviews, 2014Co-Authors: Rodrigo Antonio Brandao Neto, Jozelio Freire De CarvalhoAbstract:Autoimmune Adrenalitis, or autoimmune Addison disease (AAD), is the most prevalent cause of primary adrenal insufficiency in the developed world. AAD is rare and can easily be misdiagnosed as other conditions. The diagnosis depends on demonstrating inappropriately low cortisol production and the presence of high titers of adrenal cortex autoantibodies (ACAs), along with excluding other causes of adrenal failure using other tests as necessary. The treatment corticosteroid replacement, and the prognosis following the treatment is the same as the normal population. Spontaneous recovery of adrenal function has been described but is rare.
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autoimmune addison disease or autoimmune Adrenalitis
2008Co-Authors: Jozelio Freire De Carvalho, Rodrigo Antonio Brandao NetoAbstract:Autoimmune Adrenalitis is the most common cause of adrenal failure in developed countries, accounting for about 70% of the cases, characterized by primary adrenal failure and circulating antibodies against enzymes of adrenal cortex. The clinical manifestations are similar to other causes of Addison disease and adrenal glands are usually small and atrophic. This chapter discusses the clinical manifestations, pathology, diagnosis, and treatment of this condition and proposes a diagnostic criteria for the disease.