Associated disorders in myasthenia gravis: autoimmune diseases and their relation to thymectomy

Thorlacius, Sigurður; Aarli, Johan A.; Riise, Trond; Matre, R.; Johnsen, H J

doi:10.1111/j.1600-0404.1989.tb03881.x

Cited by 115 publications

(66 citation statements)

References 26 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…10 Myasthenia gravis (MG) is a well-recognized antibody-mediated disease affecting the neuromuscular junction, caused in around 85% of patients by immunoglobulin G (IgG)1-and IgG3-complement activating antibodies against the nicotinic acetylcholine receptor (AChR, AChR-Ab); for review, see Hoedemaekers et al 11 Both AQP4-Ab-positive NMOSD and AChR-Ab-positive MG are associated with other autoimmune diseases and autoantibodies, both organ-specific and systemic. 5,[12][13][14][15] Despite the rarity of MG and of NMO, several cases or small series of patients with both disorders have been reported over the years, most published prior to the identification of AQP4-Abs. In the English language literature, only 7 cases of AChR-Ab positive MG and AQP4-Ab-positive NMO/NMOSD have been reported.…”

mentioning

confidence: 99%

Myasthenia gravis and neuromyelitis optica spectrum disorder

et al. 2012

View full text Add to dashboard Cite

Objective:To describe 16 patients with a coincidence of 2 rare diseases: aquaporin-4 antibody (AQP4-Ab)-mediated neuromyelitis optica spectrum disorder (AQP4-NMOSD) and acetylcholine receptor antibody (AChR-Ab)-mediated myasthenia gravis (AChR-MG). Methods:The clinical details and antibody results of 16 patients with AChR-MG and AQP4-NMOSD were analyzed retrospectively.Results: All had early-onset AChR-MG, the majority with mild generalized disease, and a high proportion achieved remission. Fifteen were female; 11 were Caucasian. In 14/16, the MG preceded NMOSD (median interval: 16 years) and 11 of these had had a thymectomy although 1 only after NMOSD onset. In 4/5 patients tested, AQP4-Abs were detectable between 4 and 16 years prior to disease onset, including 2 patients with detectable AQP4-Abs prior to thymectomy. AChR-Abs decreased and the AQP4-Ab levels increased over time in concordance with the relevant disease. AChR-Abs were detectable at NMOSD onset in the one sample available from 1 of the 2 patients with NMOSD before MG. Conclusions:Although both conditions are rare, the association of MG and NMOSD occurs much more frequently than by chance and the MG appears to follow a benign course. AChR-Abs or AQP4-Abs may be present years before onset of the relevant disease and the antibody titers against AQP4 and AChR tend to change in opposite directions. Although most cases had MG prior to NMOSD onset, and had undergone thymectomy, NMOSD can occur first and in patients who have not had their thymus removed. Neurology ® 2012;78:1601-1607 GLOSSARY Ab ϭ antibodies; AChR-Ab ϭ acetylcholine receptor antibody; AChR-MG ϭ acetylcholine receptor antibody-mediated myasthenia gravis; AQP4-Ab ϭ aquaporin-4 antibody; AQP4-NMOSD ϭ aquaporin-4 antibody-mediated neuromyelitis optica spectrum disorder; IgG ϭ immunoglobulin G; IS ϭ immunosuppressive; LETM ϭ longitudinally extensive transverse myelitis; MG ϭ myasthenia gravis; NMO ϭ neuromyelitis optica; NMOSD ϭ neuromyelitis optica spectrum disorder; OCB ϭ oligoclonal bands; ON ϭ optic neuritis; SLE ϭ systemic lupus erythematosus; VGKC ϭ voltage-gated potassium channel.Neuromyelitis optica (NMO) is a recurrent inflammatory and demyelinating CNS disorder that affects predominantly the optic nerve and spinal cord.

show abstract

mentioning

confidence: 99%

Myasthenia gravis and neuromyelitis optica spectrum disorder

et al. 2012

View full text Add to dashboard Cite

show abstract

“…Thorlacius et al 10 e Oesterhuis 11 relataram que 2,2 a 8,3% dos pacientes com MG desenvolveram LES. A prevalência de MG em pacientes com LES não é conhecida.…”

Section: Discussionunclassified

“…Os sinais e sintomas de LES ou MG não foram diferentes daqueles observados em pacientes com formas puras das duas doenças. Todavia, pacientes com MG usualmente apresentaram formas mais severas de doença 10 .…”

Section: Discussionunclassified

See 1 more Smart Citation

Lupus eritematoso sistêmico associado a miastenia gravis: relato de caso

Carvalho

Abrahão

Assaf

1998

Arq. Neuro-Psiquiatr.

View full text Add to dashboard Cite

RESUMO -Os autores descrevem o caso de uma mulher branca de 24 anos de idade admitida com lupus eritematoso sistêmico (com 4 anos de evolução de doença) e início recente de miastenia gravis. São discutidos os principais diagnósticos diferenciais para a fraqueza muscular e a fadiga apresentadas por esta paciente. Uma revisão de literatura abordando a associação de miastenia gravis e lupus eritematoso é feita, com ênfase às características clínicas desses pacientes e ao papel do timoma e timectomia no desenvolvimento de lupus eritematoso em pacientes previamente miastênicos.PALAVRAS-CHAVE: lupus eritematoso sistêmico, miastenia gravis. Systemic lupus erythematosus and myasthenia gravis: case reportABSTRACT -We report the case of a 24-year-old white woman admitted with a four year diagnosis of systemic lupus erythematosus and the recent onset of myasthenia gravis discussing the main differential diagnosis of weakness and fatigue in this patient. A review of literature approaching the association of myasthenia gravis and systemic lupus erythematosus is also done with emphasis on the clinical characteristics of these patients and the role of thymoma and thymectomy in the development of systemic lupus erythematosus in myasthenic patients.KEY WORDS: systemic lupus erythematosus, myasthenia gravis.O lupus eritematoso sistêmico (LES) e a miastenia gravis (MG) são doenças pouco frequentes, mas não raras. A prevalência de LES é de 1,5 a 5 casos para cada 10000 habitantes enquanto a MG ocorre pelo menos uma vez em cada 10000 indivíduos. Desta forma, a probabilidade de um paciente desenvolver uma associação dessas duas doenças é extremamente baixa, mesmo quando recordamos que pacientes acometidos por uma primeira doença autoimune apresentam maior propensão para o desenvolvimento de uma segunda afecção desse mesmo grupo. Embora a associação de LES e MG tenha sido descrita na literatura, sua ocorrência não foi claramente explicada por estudos epidemiológicos. RELATO DO CASOUma mulher branca de 24 anos de idade foi admitida no hospital com insuficiência respiratória aguda. Visão dupla, alteração da fala e dificuldade para deglutir estavam presentes nos dois meses que precederam a admissão. Quatro anos antes desta internação, foi feito diagnóstico de lupus eritematoso sistêmico através da detecção de lesões discóides cutâneas (Fig 1 ), fotossensibilidade, anticorpo antinuclear positivo com título de 1:640 e padrão homogêneo, proteinúria não seletiva de 0,72g/L/24h e uma preparação positiva para pesquisa de

show abstract

“…Rheumatoid arthritis also was found in MG patients (4.2%). 6 This patient had been diagnosed with lupus nephritis that progressed to end-stage renal disease. After several years, she also had been diagnosed with MG.…”

Section: Discussionmentioning

confidence: 99%

Untitled

Hwang

Lee²,

Kim³

et al. 2014

Exp Clin Transplant

View full text Add to dashboard Cite

Myasthenia gravis is an autoimmune disease characterized by muscle weakness. Myasthenic crisis is a life-threatening complication of myasthenia gravis precipitated by several factors. We experienced a myasthenic crisis after a deceaseddonor kidney transplant in a 35-year-old woman who already had been diagnosed with myasthenia gravis. She received mechanical ventilatory support and intravenous immunoglobulin for treatment of the myasthenic crisis. During the early posttransplant period, she recovered with immediate graft function, and her graft functioning did not deteriorate during the myasthenic crisis. We suggest that physicians be aware of worsening of myasthenia gravis when patients with myasthenia gravis undergo a kidney transplant.

show abstract

Associated disorders in myasthenia gravis: autoimmune diseases and their relation to thymectomy

Cited by 115 publications

References 26 publications

Myasthenia gravis and neuromyelitis optica spectrum disorder

Myasthenia gravis and neuromyelitis optica spectrum disorder

Lupus eritematoso sistêmico associado a miastenia gravis: relato de caso

Untitled

Contact Info

Product

Resources

About