Autoimmune spontaneous chronic urticaria and generalized myasthenia gravis in a patient with polyglandular autoimmune syndrome type 3

Marchi, Sergio Umberto De; Cecchin, E

doi:10.1002/mus.24635

Cited by 7 publications

(6 citation statements)

References 27 publications

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“…Cases of MG associated with thyroid diseases (Hashimoto's disease and Graves' disease), rheumatoid arthritis, systemic lupus erythematosus, and insulindependent diabetes mellitus have also been described (12). There have been several reports of MG and APS (13)(14)(15)(16)(17)(18)(19). Six of eight patients had bulbar symptoms that tended to be severe, and three were intubated.…”

Section: Discussionmentioning

confidence: 99%

A Patient with Fulminant Myasthenia Gravis Is Seropositive for Both AChR and LRP4 Antibodies, Complicated by Autoimmune Polyglandular Syndrome Type 3

et al. 2020

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This article describes the first reported case of myasthenia gravis (MG) seropositive for both acetylcholine receptor antibody and low-density lipoprotein receptor-related protein 4 antibody, complicated by autoimmune polyglandular syndrome (APS) type 3. The patient exhibited myasthenic weakness restricted to the ocular muscles and ptosis. Severe clinical deterioration ensued with predominant bulbar symptoms. MG rapidly worsened, the patient was intubated, and agranulocytosis due to thiamazole was also present, so it was necessary to perform thyroidectomy with tracheostomy and thymectomy in two phases. Both the double-seropositive MG and the APS were involved in the patient's rapid deterioration.

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Section: Discussionmentioning

confidence: 99%

A Patient with Fulminant Myasthenia Gravis Is Seropositive for Both AChR and LRP4 Antibodies, Complicated by Autoimmune Polyglandular Syndrome Type 3

et al. 2020

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“…Foi também realizada ainda tomografia computadorizada do tórax que identificou a presença de hiperplasia tímica; e a eletroneuromiografia demonstrou teste de estimulação repetitiva positivo nas placas motoras dos nervos facial e acessório direito, com decremento maior que 10% do 2º ao 10º tempo do teste, padrão compatível com a doença. O paciente então mesmo recebeu uma dose inicial de piridostigmina (30mg 3x/dia) e evoluiu com melhora progressiva da força muscular, sendo posteriormente aumentada a dose e foi submetido a timectomia proporcionando estabilidade clínica da doença (TORRE, et al, 2018;SANDERS et al, 2016;DE MARCHI, 2015;DUMAN et. al., 2014;BRANCO et al, 2011).…”

Section: Discussionunclassified

Síndrome poliglandular autoimune tipo III: uma rara associação entre doença de graves, vitiligo e miastenia gravis

Tavares¹,

Kahwage²

2019

Acervo Saúde

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Introdução: A sindrome poliglandular autoimune (SPA) é caracterizada pela associação de doenças autoimunes órgão-específicas endócrinas e não-endócrinas, e se classificam em quatro tipos dependendo dos órgãos afetados. A SPA tipo III é definida como associação entre doença da tireoide autoimune com outras entidades autoimunes como doenças gastrointestinais, neuromusculares, pele, etc. Detalhamento do caso: Este relato descreve o caso clínico de um paciente masculino de 22 anos, apresentando associação de Doença de Graves, Vitiligo e Miastenia Gravis diagnosticada por critérios clínicos bem estabelecidos e exames complementares como marcadores de autoimunidade e exames de imagem, são mostradas as complicações apresentadas pelo paciente, o tratamento instituído e sua evolução clínica. Discussão: Compara-se o caso descrito com os achados relatados na literatura, abordando a epidemiologia, apresentação clínica, métodos diagnósticos e tratamentos disponíveis, ressaltando a importância da investigação clínica detalhada, no intuito de realizar diagnósticos mais precoces e tratar adequadamente os pacientes.

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“…HLA DRB-1 is found in this group of patients. APS 3 is the most common APS, likely due to the high prevalence of the combination of ATD and T1DM 20. If the autoimmune polyendocrinopathies do not fulfil the criteria of APS 1 to 3, the disease may be categorised as APS 4.…”

Section: Discussionmentioning

confidence: 99%

Unusual case of anti-N-methyl-D-aspartic acid-receptor (NMDA-R) encephalitis and autoimmune polyglandular syndrome (APS)

Frunza-Stefan¹,

Whitlatch

Rao

et al. 2018

BMJ Case Reports

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Anti-N-methyl-D-aspartic acid-receptor (NMDA-R) encephalitis is a novel disease discovered within the past 10 years. It is an autoimmune disease (AD) that has been associated with other ADs, such as Graves' disease. However, association with autoimmune polyglandular syndromes (APS) has not been previously described. A 58-year-old woman presented with altered mental status and an 8-month history of weight loss, apathy and somnolence. Laboratory evaluation confirmed Graves' disease with thyrotoxicosis and type 1 diabetes mellitus. Despite treatment, she continued to have a fluctuating mental status. Further diagnostic evaluation included an abdominal MRI that showed a cystic lobular left adnexal mass. Serum anti-NMDA-R antibodies were positive, raising concern for NMDA-R encephalitis. Bilateral salpingo-oophorectomy was performed, with pathology consistent with cystadenofibroma. She had a favourable recovery with marked clinical improvement. Anti-NMDA-R antibodies were negative 2 months following surgery. The concomitant occurrence of APS and anti-NMDA-R encephalitis suggests a shared mechanism of autoimmune pathophysiology.

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Autoimmune spontaneous chronic urticaria and generalized myasthenia gravis in a patient with polyglandular autoimmune syndrome type 3

Abstract: Patients with APS should have a more thorough evaluation to better clarify their autoimmune background. Early detection of autoantibodies and latent organ-specific dysfunction may help physicians take appropriate action to prevent full-blown disease.

Cited by 7 publications

References 27 publications

A Patient with Fulminant Myasthenia Gravis Is Seropositive for Both AChR and LRP4 Antibodies, Complicated by Autoimmune Polyglandular Syndrome Type 3

A Patient with Fulminant Myasthenia Gravis Is Seropositive for Both AChR and LRP4 Antibodies, Complicated by Autoimmune Polyglandular Syndrome Type 3

Síndrome poliglandular autoimune tipo III: uma rara associação entre doença de graves, vitiligo e miastenia gravis

Unusual case of anti-N-methyl-D-aspartic acid-receptor (NMDA-R) encephalitis and autoimmune polyglandular syndrome (APS)

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