Clinical features and treatment status of antiacetylcholine receptor antibody-positive ocular myasthenia gravis
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CLINICAL INVESTIGATION
Clinical features and treatment status of antiacetylcholine receptor antibody‑positive ocular myasthenia gravis Yoshihiko Isshiki1 · Osamu Mimura1 · Fumi Gomi1 Received: 28 January 2020 / Accepted: 16 July 2020 © Japanese Ophthalmological Society 2020
Abstract Purpose To clarify the factors predictive of progression from ocular myasthenia gravis (OMG) to generalized MG (GMG) among patients with positive antiacetylcholine receptor antibodies (AChRAb) and to evaluate the efficacy of our OMG treatment protocol including tacrolimus (TAC). Study design Retrospective study. Methods Fifty-two patients with AChRAb positivity and ptosis and/or eye movement disturbance showing diurnal fluctuation and fatigability were included. First, we compared the clinical characteristics of patients without OMG progression (p-OMG) with those of patients whose OMG transformed to GMG (TMG). Second, we subdivided the p-OMG patients according to the time of approval of TAC into the Before group and the After group. We compared the dosage and period of prednisolone administration (PSL) of the 2 groups. Third, we evaluated the responses to OMG treatment using the Myasthenia Gravis Foundation of America Postintervention Status (MGFA-PS) scale. Results Forty-one patients were classified into the p-OMG group, and 11, into the TMG group. The AChRAb level (P = 0.0006) and prevalence of thymoma (P = 0.001) were significantly higher in the TMG group than in the p-OMG group. In the p-OMG group, the MG composite score (P ≤ 0.0001) and AChRAb level (P = 0.005) improved after treatment. The periods of PSL administration at ≥ 20 mg/day (P = 0.009) and at 10 to 19 mg/day (P = 0.002) were significantly shorter in the After group. At the last follow-up, 78.0% of the p-OMG group patients had achieved MGFA-PS minimal manifestations or better status with PSL ≤ 5 mg/day. Conclusions The TMG cases were characterized by higher AChRAb levels and presence of thymoma. The P-OMG patients could be managed with our treatment protocol. Combined use of TAC was efficacious in patients with steroid-dependent p-OMG. Keywords Antiacetylcholine receptor antibody · Generalized myasthenia gravis · Myasthenia gravis foundation of America postintervention status · Ocular myasthenia gravis · Tacrolimus
Introduction Myasthenia gravis (MG) is an autoimmune systemic disease that exhibits diverse symptoms such as easy muscle fatigability, ptosis, and diplopia. Many patients manifest ocular symptoms at the onset, and early changes are readily detected by ophthalmologists. Ocular MG (OMG) is one Corresponding Author: Yoshihiko Isshiki * Yoshihiko Isshiki y‑isshik@hyo‑med.ac.jp 1
Department of Ophthalmology, Hyogo College of Medicine, 1‑1 Mukogawa‑cho, Nishinomiya, Hyogo 663‑850, Japan
type of MG that causes only ocular symptoms but reportedly developed into secondary generalized myasthenia gravis (GMG) in 20% to 80% of patients [1–4]. Compared with GMG, OMG has lower positive rates of antiacetylcholine receptor antibody (AChRAb) [5–7] and antimuscle-s
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