Miastenia grave induzida por D-penicilamina em paciente com esclerose sistêmica progressiva

Marchiori, Paulo Eurípedes; Scaff, Milberto; Cossermelli, W; Assis, J. Lamartine de

doi:10.1590/s0004-282x1984000400010

Cited by 7 publications

(3 citation statements)

References 13 publications

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“…Based on data from the literature, DPA-induced MG occurs usually after 2–12 months but can be evident years after treatment initiation, especially in WD patients (6–96 months). The symptoms are generally ocular, with good response to pyridostigmine and clinical remission within one year after DPA treatment cessation: 70% of patients completely clinically recover, in contrast to idiopathic MG where 9.6 to 57.5% of patients will have remission during the first year [ 16 , 17 , 18 , 19 , 20 , 21 , 22 , 23 , 24 , 25 , 26 , 27 , 28 , 29 , 30 , 31 ]. If MG symptoms persist, it seems that DPA treatment is only a trigger factor for MG.…”

Section: Discussionmentioning

confidence: 99%

“…The appearance of both AChR-Abs and MuSK-Abs (like in our patient) indicates the general autoimmune reaction after DPA [ 30 , 31 , 32 , 33 ] as they are rarely both seen in idiopathic MG. The alternative explanation of this phenomenon is that DPA stimulates prostaglandin E1 synthetase, producing prostaglandin E1, which occupies an allosteric site on AChRs, hindering acetylcholine binding [ 29 , 30 ].…”

Section: Discussionmentioning

confidence: 99%

“…However, mostly in the course of RA treatment and only occasionally in WD [2,[15][16][17]. From several retrospective analyses of WD patients treated with anti-copper agents (including DPA), it appears that cases of DPA-induced MG are incidental, with only seven cases in the medical literature and three cases in a Polish cohort of 1000 WD patients (data not published), indicating that it occurs less frequently that previously suggested [15,18,[20][21][22][23][24][25][26][27][28][29].…”

Section: Discussionmentioning

confidence: 99%

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D-Penicillamine-Induced Myasthenia Gravis—A Probable Complication of Wilson’s Disease Treatment—A Case Report and Systematic Review of the Literature

Antos

Członkowska

Bembenek

et al. 2023

Life

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Wilson’s disease (WD) is a genetic disorder with copper accumulation in various tissues leading to related clinical symptoms (mainly hepatic and neuropsychiatric) which can be in 85% of patients successfully treated with anti-copper agents. However, during WD treatment neurological deterioration may occur in several patients. D-penicillamine (DPA) is one of the most frequently used drugs in WD treatment. Despite its efficacy, DPA can produce many adverse drug reactions, which should be recognized early. We present the case of a 51-year-old man diagnosed with the hepatic form of WD and initially treated with DPA in whom after 15 months of treatment, diplopia and evening ptosis occurred. WD treatment non-compliance as well as overtreatment were excluded. Supported by neurological symptoms, a positive edrophonium test, and high serum levels of antibodies against acetylcholine receptors (AChR-Abs), as well as low concentrations of antibodies against muscle-specific kinase (MuSK-Abs), the diagnosis of myasthenia gravis (MG), induced by DPA, was established. DPA was stopped; zinc sulfate for WD and pyridostigmine for MG symptoms were introduced. Diplopia and ptosis subsided after a few days, which supported our diagnosis. During a follow-up visit after 6 months, the patient did not present any MG symptoms. AChR-Abs level gradually decreased and MuSK-Abs were no longer detected. Pyridostigmine was stopped, and within 9 months of follow-up, the neurological symptoms of MG did not reoccur. The authors discussed the patient’s neurological deterioration, performed a systematic review of DPA-induced MG in WD and concluded that MG is a rare and usually reversible complication of DPA treatment. DPA-induced MG generally occurs 2–12 months after treatment initiation and ocular symptoms predominate. Response to pyridostigmine treatment is good and MG symptoms usually reverse within one year after DPA treatment cessation. However, symptoms may persist in some cases where DPA treatment is only a trigger factor for MG occurrence.

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