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. 2019 Oct;26(10):1296-e84.
doi: 10.1111/ene.13979. Epub 2019 Jun 2.

Clinical analysis of Chinese anti-low-density-lipoprotein-receptor-associated protein 4 antibodies in patients with myasthenia gravis

M Li  1 , J Han  2 , Y Zhang  2 , J Lv  2 , J Zhang  2 , X Zhao  2 , L Ren  1 , H Fang  2 , J Yang  3 , Y Zhang  3 , X Cui  4 , Q Zhang  4 , Q Li  5 , Y Du  5 , F Gao  2
Affiliations

Clinical analysis of Chinese anti-low-density-lipoprotein-receptor-associated protein 4 antibodies in patients with myasthenia gravis

M Li et al. Eur J Neurol. 2019 Oct.

Abstract

Background and purpose: Low-density-lipoprotein-receptor-associated protein 4 (LRP4) autoantibodies have recently been detected in myasthenia gravis (MG), but little is known about the clinical characteristics associated with this serological type. In this study, the clinical features of Chinese patients with anti-LRP4 antibody-positive MG were characterized.

Methods: A total of 2172 MG serum samples were collected from patients in various parts of China. An enzyme-linked immunosorbent assay was used to detect acetylcholine receptor (AChR) antibody and titin antibody, and cell-based assays were used to detect muscle-specific kinase antibody and LRP4 antibody. Clinical data for patients with MG were collected from different provinces in China.

Results: In total, 16 (0.8%) patients with LRP4-MG were found amongst 2172 total patients, including three patients with AChR/LRP4-MG. Additionally, 13 (2.9%) patients with LRP4-MG were found amongst 455 patients with double seronegative MG. The ratio of males to females for these 13 patients was 1:1.6, and 53.8% patients were children. A total of 91.7% of cases exhibited initial ocular involvement, and 58.3% of cases exhibited simple eye muscle involvement. Responses to acetylcholinesterase inhibitors and prednisone were observed.

Conclusion: The expanded sample confirmed that the positive rate of LRP4 antibodies in China is lower than that in western countries. Our results highlighted the differences between LRP4-MG and other antibody groups. Children and female patients with LRP4-MG have a higher prevalence, often involving the ocular muscles and limb muscles. The clinical symptoms are mild, and satisfactory responses to treatment are often achieved.

Keywords: autoantibody; double seronegative (dSN); low-density-lipoprotein-receptor-associated protein 4 (LRP4); myasthenia gravis (MG); triple seronegative (TSN).

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