Commentary: Systemic lupus erythematosus retinopathy: Eye or multisystem involvement?

Le résumé fourni par la source

Systemic lupus erythematosus (SLE) has a wide range of protean manifestations with a variable course and prognosis. Though it is a multiorgan disease, it can be limited to one organ as well. Hence, the diagnosis and treatment are a challenge to the physician. Often, they focus exclusively on the serology evidence for diagnosing and monitoring the disease after excluding the competing diagnosis. The new SLE classification criteria were developed with support from both the American College of Rheumatology (ACR) and European Alliance of Associations for Rheumatology (EULAR). They have validated the criteria against a large number of cases, including many patients with manifestations that resemble SLE but who do not have SLE. The 2019 EULAR/ACR classification criteria for SLE include positive ANA as an obligatory entry criterion; followed by additive weighted criteria grouped in seven clinical (constitutional, hematologic, neuropsychiatric, mucocutaneous, serosal, musculoskeletal, and renal) and three immunological (antiphospholipid antibodies, complement proteins, and SLE-specific antibodies) domains, and weighted from 2 to 10. Patients accumulating ≥10 points are subclassified too.[1] To improve the long-term outcome management of SLE, the physician should aim at remission of disease, prevention of organ damage, reducing the adverse effects of the drugs, and improving the quality of life. Though complete remission is infrequent, treatment in SLE should aim at a low disease activity in all organ systems. According to EULAR, remission is defined as the absence of clinical activity with no use of glucocorticoids and immunosuppressive drugs.[2] However, low disease activity is defined as Systemic Lupus Disease Activity Index (SLEDAI) ≤4, Physician Global Assessment (PGA) ≤1 with glucocorticoid ≤7.5 mg of prednisone and well-tolerated immunosuppressive agents.[2] However, it is important to note that the classification criteria are not for the diagnosis or treatment decisions. Patients who do not fully meet these criteria should not be stopped from receiving appropriate therapies. This is also applicable to patients with ANA-negative SLE. Diagnosis of SLE remains the responsibility of the trained physician evaluating a patient. Ocular manifestations of SLE vary [Table 1] and can be vision-threatening. Ocular involvement in SLE correlates to systemic disease activity. Keratoconjunctivitis sicca is the most common ocular manifestation of SLE.Table 1: Ocular manifestations in SLESLE retinopathy is a sight-threatening ocular involvement in SLE. The classic sign of vasculitis in SLE is vascular sheathing, which can involve the arterioles and/or venules. Fundus fluorescein angiography is a sensitive tool in diagnosing retinal vessel and disc leaks.[6] Renal involvement in SLE will lead to secondary hypertension. When prolonged, it usually affects the retina and choroid and is characterized by retinal arterial narrowing, arteriovenous crossing changes, microaneurysms, intraretinal hemorrhages, hard exudates, disc edema, and multifocal serous or pigment epithelial detachment. Visual prognosis is associated with the pattern of retina involvement. Retinopathy, vascular occlusion, and optic neuropathy have a higher risk for morbidity. Active inflammation in the retina and choroid indicates vasculitis in other organs, especially cerebral vascular disease [Table 2].[7] Active CNS involvement in SLE is a significant cause of patient morbidity.Table 2: Association of SLE retinopathy with central nervous system (CNS)Therefore, SLE retinopathy is a guide in the management of the disease. Stafford-Brady et al. and Nguyen et al. have concluded that retinal involvement indicates poor survival prognosis.[8,10] The patients who developed SLE retinopathy had a poor overall survival rate as compared with patients without retinopathy within the same time period. Uvea specialists have an important role in the management of ocular involvement[12] in SLE. Stafford-Brady et al. have reported that 88% of patients with SLE retinopathy had active systemic disease.[8] Therefore, posterior segment manifestations in SLE retinopathy should prompt the specialist to communicate with the rheumatologist regarding the disease severity. Also, the ophthalmologist should be aggressive in the treatment as it may precede an extraocular involvement. The key element in reducing morbidity is early recognition and treatment by the rheumatologist and ophthalmologist. Immunological markers including antibodies against U1RNP, Sm, SS-A, Ro-52, and Ribosomal P were higher among those with SLE retinopathy. Antibody against Sm is relatively specific for the diagnosis of lupus. However, it does not correlate with disease activity or disease manifestation. But Seth et al.[6] have found an association between SLE retinopathy with this autoantibody. The role of antibodies to Sm in patients with SLE retinopathy needs further study. Due to the multisystem involvement, treatment of SLE is difficult. Treatment strategies for SLE include hydroxychloroquine, systemic corticosteroids, and early initiation of immunosuppressive therapy and/or biologics. Treatment of the ocular disease is based on systemic therapy. Though pan-retinal photocoagulation is beneficial for retinal neovascularization, anti-VEGF therapy is also beneficial in retinal neovascularization and macula ischemia. Early recognition of the disease by the rheumatologist and ophthalmologist, with coordinated treatment strategies, is the key to reducing the ocular morbidity associated with the disease. Also, the inclusion of ocular manifestations in the classification criteria for SLE will help in the earlier establishment of the diagnosis and therapeutic interventions and thereby reduce the mortality from this disease.

Ce résumé expose les affirmations des auteurs. BNTIC ne l’interprète pas comme une validation indépendante des résultats.