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Front Neurol. 2022 May 9;13:873599. doi: 10.3389/fneur.2022.873599. eCollection 2022.

ABSTRACT

OBJECTIVE: This study aimed to summarize the clinical characteristics and prognosis of patients with anti- acetylcholine receptor (AChR) positive myasthenia gravis (MG) with a combination of anti-LRP4 or Titin antibodies.

METHODS: A total of 188 patients with generalized MG before immunotherapy were retrospectively collected and then divided into three groups: single anti-AChR positive-MG (AChR-MG, 101 cases), anti-AChR combined with anti-low-density lipoprotein receptor-related protein four-positive MG (AChR+LRP4-MG, 29 cases), and anti-AChR combined with anti-Titin-positive MG (AChR+Titin-MG, 58 cases). Clinical manifestations, therapeutic responses to immunotherapy, and follow-up information were analyzed.

RESULTS: Of the 188 seropositive MG patients, 29 (15.4%) were positive for both AChR and LRP4 antibodies, and 58 (30.9%) were positive for both AChR and Titin antibodies. The mean disease onset ages in the three groups were 47.41 ± 7.0, 49.81 ± 9.2, and 48.11 ± 6.5 years, respectively. AChR+LRP4-MG showed female predominance (27.6% were males and 72.4% were females), with mild overall clinical symptoms. The AChR+Titin-MG group showed shorter times for conversion to generalized MG (5.14 ± 0.0 months) than the AChR-MG group (11.69 ± 0.0 months) and the AChR+LRP4-MG group (13.08 ± 0.5 months; P < 0.001 in both cases). Furthermore, AChR+Titin-MG group had increased bulbar dysfunction, higher incidences of thymoma (32.8 vs. 19.8% and 3.4%, P=0.035), more severe quantitative MG scores, as assessed by both QMG scores [15.5 (11.75-22.5) vs. 13 (8-19), P = 0.005; and 9 (6-14) P < 0.001], and MG-ADL scores [10 (8-13) vs. 8 (5-13), P = 0.018; and 6 (4-8), P < 0.001]. Treatment for AChR+Titin-MG was largely dependent on corticosteroids and immunosuppressive agents (56.7 vs. 19.2% and 16.7%, p = 0.028). The rates of achieving s(MMS) or better within 2 years following immunotherapy in the three groups were 51.5, 62.1, and 51.7%, respectively (P = 0.581).

CONCLUSION: Clinical symptoms of anti-AChR positive MG combined with Titin antibody were more severe and progressed faster than those in the AChR + LRP4 and AChR groups. Regardless of antibody status, all patients responded well to immunotherapy and had relatively good prognoses.

PMID:35614931 | PMC:PMC9124862 | DOI:10.3389/fneur.2022.873599


Source: https://pubmed.ncbi.nlm.nih.gov/3561493 ... 3&v=2.17.6