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Enrichment of serum IgG4 in MuSK myasthenia gravis patients

Vergoossen, Dana L. E. (author)
Leiden Univ, Dept Human Genet, Med Ctr LUMC, Einthovenweg 20, NL-2300 RC Leiden, Netherlands.
Ruiter, Annabel M. (author)
Leiden Univ, Dept Neurol, Med Ctr LUMC, Albinusdreef 2, NL-2333 ZA Leiden, Netherlands.
Keene, Kevin R. (author)
Leiden Univ, Dept Neurol, Med Ctr LUMC, Albinusdreef 2, NL-2333 ZA Leiden, Netherlands.
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Niks, Erik H. (author)
Leiden Univ, Dept Neurol, Med Ctr LUMC, Albinusdreef 2, NL-2333 ZA Leiden, Netherlands.
Tannemaat, Martijn R. (author)
Leiden Univ, Dept Neurol, Med Ctr LUMC, Albinusdreef 2, NL-2333 ZA Leiden, Netherlands.
Strijbos, Ellen (author)
Leiden Univ, Dept Neurol, Med Ctr LUMC, Albinusdreef 2, NL-2333 ZA Leiden, Netherlands.
Lipka, Alexander F. (author)
Leiden Univ, Dept Neurol, Med Ctr LUMC, Albinusdreef 2, NL-2333 ZA Leiden, Netherlands.
van der Zijde, Els C. Jol (author)
Leiden Univ, Willem Alexander Childrens Hosp, Med Ctr LUMC, Albinusdreef 2, NL-2333 ZA Leiden, Netherlands.
van Tol, Maarten J. D. (author)
Leiden Univ, Willem Alexander Childrens Hosp, Med Ctr LUMC, Albinusdreef 2, NL-2333 ZA Leiden, Netherlands.
Bakker, Jaap A. (author)
Leiden Univ, Dept Clin Chem, Med Ctr LUMC, Albinusdreef 2, NL-2333 ZA Leiden, Netherlands.
Wevers, Brigitte A. (author)
Leiden Univ, Dept Clin Chem, Med Ctr LUMC, Albinusdreef 2, NL-2333 ZA Leiden, Netherlands.
Westerberg, Elisabet (author)
Uppsala universitet,Klinisk neurofysiologi
Tong, Olivia C. (author)
Univ Calif Davis, Dept Neurol, 1515 Newton Court, Davis, CA 95618 USA.
Richman, David P. (author)
Univ Calif Davis, Dept Neurol, 1515 Newton Court, Davis, CA 95618 USA.
Illa, Isabel (author)
Hosp St Pau UAB, Neuromuscular Dis Neurol Dept, Ave Pare Claret 167, Barcelona 08025, Spain.
Rostedt Punga, Anna (author)
Uppsala universitet,Klinisk neurofysiologi
Evoli, Amelia (author)
Univ Cattolica Sacro Cuore, Dept Neurol, Largo A Gemelli 1, I-00168 Rome, Italy.
van der Maarel, Silvere M. (author)
Leiden Univ, Dept Human Genet, Med Ctr LUMC, Einthovenweg 20, NL-2300 RC Leiden, Netherlands.
Verschuuren, Jan J. (author)
Leiden Univ, Dept Neurol, Med Ctr LUMC, Albinusdreef 2, NL-2333 ZA Leiden, Netherlands.
Huijbers, Maartje G. (author)
Leiden Univ, Dept Human Genet, Med Ctr LUMC, Einthovenweg 20, NL-2300 RC Leiden, Netherlands.;Leiden Univ, Dept Neurol, Med Ctr LUMC, Albinusdreef 2, NL-2333 ZA Leiden, Netherlands.
Borgesf, Lucia S. (author)
Univ Calif Davis, Dept Neurol, 1515 Newton Court, Davis, CA 95618 USA.
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Leiden Univ, Dept Human Genet, Med Ctr LUMC, Einthovenweg 20, NL-2300 RC Leiden, Netherlands Leiden Univ, Dept Neurol, Med Ctr LUMC, Albinusdreef 2, NL-2333 ZA Leiden, Netherlands. (creator_code:org_t)
Elsevier, 2022
2022
English.
In: Journal of Neuroimmunology. - : Elsevier. - 0165-5728 .- 1872-8421. ; 373
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  • Journal article (peer-reviewed)
Abstract Subject headings
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  • Muscle-specific kinase (MuSK) myasthenia gravis (MG) is a neuromuscular autoimmune disease belonging to a growing group of IgG4 autoimmune diseases (IgG4-AIDs), in which the majority of pathogenic autoantibodies are of the IgG4 subclass. The more prevalent form of MG with acetylcholine receptor (AChR) antibodies is caused by IgG1-3 autoantibodies. A dominant role for IgG4 in autoimmune disease is intriguing due to its antiinflammatory characteristics. It is unclear why MuSK autoantibodies are predominantly IgG4. We hypothesized that MuSK MG patients have a general predisposition to generate IgG4 responses, therefore resulting in high levels of circulating IgG4. To investigate this, we quantified serum Ig isotypes and IgG subclasses using nephelometric and turbidimetric assays in MuSK MG and AChR MG patients not under influence of immunosuppressive treatment. Absolute serum IgG1 was increased in both MuSK and AChR MG patients compared to healthy donors. In addition, only MuSK MG patients on average had significantly increased and enriched serum IgG4. Although more MuSK MG patients had elevated serum IgG4, for most the IgG4 serum levels fell within the normal range. Correlation analyses suggest MuSK-specific antibodies do not solely explain the variation in IgG4 levels. In conclusion, although serum IgG4 levels are slightly increased, the levels do not support ubiquitous IgG4 responses in MuSK MG patients as the underlying cause of dominant IgG4 MuSK antibodies.

Subject headings

MEDICIN OCH HÄLSOVETENSKAP  -- Klinisk medicin -- Klinisk laboratoriemedicin (hsv//swe)
MEDICAL AND HEALTH SCIENCES  -- Clinical Medicine -- Clinical Laboratory Medicine (hsv//eng)
MEDICIN OCH HÄLSOVETENSKAP  -- Medicinska och farmaceutiska grundvetenskaper -- Immunologi inom det medicinska området (hsv//swe)
MEDICAL AND HEALTH SCIENCES  -- Basic Medicine -- Immunology in the medical area (hsv//eng)

Keyword

Immunoglobulins
IgG4
Autoimmune disease
Serum
MuSK
Myasthenia gravis

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