Systemic lupus erythematosus (SLE) is associated with a wide variety of ocular manifestations. Some of these include uveitis, occlusive retinal vasculitis, (epi)scleritis and keratitis.

Uveitis is an inflammation of the uvea, consisting of the iris, the ciliary body and the choroid. It is estimated that uveitis occurs in 0.48–0.8% of patients with systemic lupus erythematosus (SLE). It can be the presenting sign of SLE. All parts of the uvea can be involved in SLE-associated uveitis, ranging from anterior uveitis, often managed with topical therapy, to full blown occlusive retinal vasculitis, requiring aggressive immune suppression and sometimes retinal photocoagulation and surgery.^{1 2}

Scleritis is a painful inflammation of the sclera, sometimes leading to thinning and necrosis of the eye wall. Treatment is often with systemic immunosuppressants.³ Scleritis is easily distinguished from episcleritis, because the latter is much less painful and easily treated with topical steroids or non-steroidal anti-inflammatory drugs. It is estimated that around 1–2% of patients with SLE develop (epi)scleritis, whereas in (epi)scleritis patients around 3% is associated with SLE.^{4 5}

Dry eye disease (keratoconjunctivitis sicca) is a more frequent ocular manifestation of SLE compared to scleral involvement. Beyond dry eyes and scleral complications (including episcleritis and sclera involvement), SLE can affect various structures of the eye, causing uveal, lachrymal, retinal vascular, (epi)scleral involvement, and orbital and conjunctival disease can occur. This presentation will focus on the clinical manifestations of ocular features associated with SLE.

References

1. Kahwage PP, Ferriani MP, Furtado JM, et al. Uveitis in childhood-onset systemic lupus erythematosus patients: A multicenter survey. Clin Rheumatol. 2017;36(3):547–53. doi: 10.1007/s10067-016-3534-0.

2. Gallagher K, Viswanathan A, Okhravi N. Association of systemic lupus erythematosus with uveitis. JAMA Ophthalmol. 2015;133(10):1190–3. doi: 10.1001/jamaophthalmol.2015.2249.

3. Sivaraj RR, Durrani OM, Denniston AK, et al. Ocular manifestations of systemic lupus erythematosus. Rheumatology (Oxford). 2007;46(12):1757–62. doi: 10.1093/rheumatology/kem173.

4. Sainz de la Maza M, Molina N, Gonzalez-Gonzalez LA, et al. Clinical characteristics of a large cohort of patients with scleritis and episcleritis. Ophthalmology. 2012;119(1):43–50. doi: 10.1016/j.ophtha.2011.07.013.

5. Héron E, Gutzwiller-Fontaine M, Bourcier T. [scleritis and episcleritis: Diagnosis and treatment]. Rev Med Interne. 2014;35(9):577–85. doi: 10.1016/j.revmed.2014.02.004.

Learning Objectives At the end of this presentation participants will be able to:

• Identify common ocular manifestations associated with SLE

• Describe the different parts of the uvea and how uveitis can manifest in SLE patients

• Differentiate between scleritis and episcleritis in terms of pain level and treatment approach

• Explain the relative frequency of dry eye disease, scleral involvement, and uveitis in SLE patients

• Recognize that SLE can affect various structures of the eye beyond the ones covered in this text (e.g. uvea, lacrimal gland, retina, sclera, conjunctiva, and orbit)