Retinal Vasculitis in a Patient With Isaacs Syndrome and Inclusion Body Myositis

Overview

Journal J Vitreoretin Dis

Publisher Sage Publications

Date 2023 Apr 3

PMID 37006658

Authors

Luis Martinez-Velazquez

Paul Zhou

Francisco J Lopez-Font

Sandra Hoyek

Candace H Feldman

Anthony A Amato

Lucia Sobrin

Nimesh A Patel

Affiliations

Soon will be listed here.

Abstract

Purpose: To report a case of bilateral occlusive retinal vasculitis in a patient with autoimmunity.

Methods: A case was analyzed and a literature review performed.

Results: A 55-year-old woman with autoimmune diagnoses of Isaacs syndrome and inclusion body myositis (IBM) reported decreased vision for 3 months. A fundus examination showed peripheral intraretinal hemorrhages in the right eye and an inferotemporal subhyaloid hemorrhage with adjacent intraretinal hemorrhages and preretinal fibrosis in the left eye. Fluorescein angiography showed temporal peripheral leakage and capillary dropout in both eyes, consistent with occlusive vasculitis. Scatter laser treatment to peripheral areas of retinal nonperfusion was followed by an intravitreal bevacizumab injection. Four months later, vision had stabilized at 20/15 in both eyes and the peripheral leakage had resolved.

Conclusions: This patient developed retinal vasculitis associated with the rare autoimmune neuromuscular disorders of Isaacs syndrome and IBM. An extensive workup showed the most plausible mechanism for the vasculitis was autoimmunity with a history of previously elevated antibodies levels associated with the antiphospholipid syndrome.

References

Miserocchi E, Baltatzis S, Foster C . Ocular features associated with anticardiolipin antibodies: a descriptive study. Am J Ophthalmol. 2001; 131(4):451-6. DOI: 10.1016/s0002-9394(00)00884-9. View

Browne D, Gancher S, Nutt J, Brunt E, Smith E, Kramer P . Episodic ataxia/myokymia syndrome is associated with point mutations in the human potassium channel gene, KCNA1. Nat Genet. 1994; 8(2):136-40. DOI: 10.1038/ng1094-136. View

Cohen R, Lipper S, Dantzker D . Inclusion body myositis as a cause of respiratory failure. Chest. 1993; 104(3):975-7. DOI: 10.1378/chest.104.3.975. View

Ng C, Suresh S, Rosenbaum J, McDonald H, Cunningham Jr E . Occlusive retinal vasculitis associated with systemic sclerosis and antiphospholipid antibodies. Am J Ophthalmol Case Rep. 2021; 24:101206. PMC: 8449073. DOI: 10.1016/j.ajoc.2021.101206. View

George R, Walton R, Whitcup S, Nussenblatt R . Primary retinal vasculitis. Systemic associations and diagnostic evaluation. Ophthalmology. 1996; 103(3):384-9. DOI: 10.1016/s0161-6420(96)30681-7. View

Rosenbaum J, Ku J, Ali A, Choi D, Suhler E . Patients with retinal vasculitis rarely suffer from systemic vasculitis. Semin Arthritis Rheum. 2011; 41(6):859-65. PMC: 4062185. DOI: 10.1016/j.semarthrit.2011.10.006. View

Rana S, Ramanathan R, Small G, Adamovich B . Paraneoplastic Isaacs' syndrome: a case series and review of the literature. J Clin Neuromuscul Dis. 2012; 13(4):228-33. DOI: 10.1097/CND.0b013e318246197d. View

Maleki A, Cao J, Silpa-Archa S, Foster C . VISUAL OUTCOME AND POOR PROGNOSTIC FACTORS IN ISOLATED IDIOPATHIC RETINAL VASCULITIS. Retina. 2016; 36(10):1979-85. DOI: 10.1097/IAE.0000000000001019. View

Pelegrin L, Hernandez-Rodriguez J, Espinosa G, Llorenc V, Sainz-de-la-Maza M, Fontenla J . Characterization of isolated retinal vasculitis. Analysis of a cohort from a single center and literature review. Autoimmun Rev. 2017; 16(3):237-243. DOI: 10.1016/j.autrev.2017.01.006. View

10.

Naddaf E, Barohn R, Dimachkie M . Inclusion Body Myositis: Update on Pathogenesis and Treatment. Neurotherapeutics. 2018; 15(4):995-1005. PMC: 6277289. DOI: 10.1007/s13311-018-0658-8. View

11.

Ahmed A, Simmons Z . Isaacs syndrome: A review. Muscle Nerve. 2015; 52(1):5-12. DOI: 10.1002/mus.24632. View

12.

Huvard M, Pecen P, Palestine A . The Clinical Characteristics of Noninfectious Occlusive Retinal Vasculitis. Ophthalmol Retina. 2021; 6(1):43-48. DOI: 10.1016/j.oret.2021.04.012. View

13.

Sinha S, Newsom-Davis J, Mills K, Byrne N, Lang B, Vincent A . Autoimmune aetiology for acquired neuromyotonia (Isaacs' syndrome). Lancet. 1991; 338(8759):75-7. DOI: 10.1016/0140-6736(91)90073-x. View

14.

Arimura K, Sonoda Y, Watanabe O, Nagado T, Kurono A, Tomimitsu H . Isaacs' syndrome as a potassium channelopathy of the nerve. Muscle Nerve Suppl. 2002; 11:S55-8. DOI: 10.1002/mus.10148. View

15.

Rosenbaum J, Sibley C, Lin P . Retinal vasculitis. Curr Opin Rheumatol. 2016; 28(3):228-35. PMC: 4893321. DOI: 10.1097/BOR.0000000000000271. View