MENU ▼
Read by QxMD icon Read
search
OPEN IN READ APP
JOURNAL ARTICLE

Is MuSK myasthenia gravis linked to IgG4-related disease?

Pooja Raibagkar, Judith A Ferry, John H Stone
Journal of Neuroimmunology 2017 April 15, 305: 82-83
28284351
Immunoglobulin (Ig) G4-related disease (IgG4-RD) is an immune-mediated inflammatory condition that affects a wide variety of sites, including the nervous system, where it can involve the meninges or the pituitary gland, and cause perineural mass lesions. A large subset of acetylcholine receptor antibody (Ab)-negative myasthenia gravis (MG) patients has muscle-specific tyrosine kinase (MuSK) Abs, generally of the IgG4 subclass. There has not been any association found between IgG4-RD and MuSK MG yet. We report the first case of MuSK MG associated with lymphadenopathy with histopathology consistent with IgG4-RD. A 54-year-old woman with MuSK MG developed eight compression fractures related to steroid therapy. Eighteen months after initial presentation she was found to have retroperitoneal lymphadenopathy with biopsy findings consistent with IgG4-RD. She was started on rituximab with clinical improvement and ability to taper immunomodulatory agents for the first time. Our case raises number of questions regarding a potential link between MuSK MG and IgG4-RD which may shed further light on the pathophysiology and management of these diseases.

Comments

You need to log in or sign up for an account to be able to comment.

No comments yet, be the first to post one!

Related Papers

Available on the App Store

Available on the Play Store

Read Institutional Edition
Remove bar
Read by QxMD icon Read
28284351
×

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"