Ocular Manifestations of Systemic Lupus Erythematosus: A Review of the Literature Neal V. Palejwala, Emory University Harpreet S. Walia, Emory University Steven Yeh, Emory University Journal Title: Autoimmune Diseases Volume: Volume 2012, Number 2012 Publisher: Hindawi Publishing Corporation | 2012-05-31, Pages 1-9 Type of Work: Article | Final Publisher PDF Publisher DOI: 10.1155/2012/290898 Permanent URL: http://pid.emory.edu/ark:/25593/d8q18 Final published version: http://www.hindawi.com/journals/ad/2012/290898/ Copyright information: © 2012 Neal V. Palejwala et al. This is an Open Access work distributed under the terms of the Creative Commons Attribution 3.0 Unported License (http://creativecommons.org/licenses/by/3.0/). Accessed September 23, 2021 5:51 AM EDT Hindawi Publishing Corporation Autoimmune Diseases Volume 2012, Article ID 290898, 9 pages doi:10.1155/2012/290898 Review Article Ocular Manifestations of Systemic Lupus Erythematosus: A Review of the Literature Neal V. Palejwala, Harpreet S. Walia, and Steven Yeh Section of Vitreoretinal Disease and Surgery, Department of Ophthalmology, Emory Eye Center, Emory University School of Medicine, Atlanta, 30322 GA, USA Correspondence should be addressed to Steven Yeh, [email protected] Received 5 May 2012; Accepted 31 May 2012 Academic Editor: Hiroshi Okamoto Copyright © 2012 Neal V. Palejwala et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. About one-third of patients suffering from systemic lupus erythematosus have ocular manifestations. The most common manifestation is keratoconjunctivitis sicca. The most vision threatening are retinal vasculitis and optic neuritis/neuropathy. Prompt diagnosis and treatment of eye disease is paramount as they are often associated with high levels of systemic inflammation and end-organ damage. Initial management with high-dose oral or IV corticosteroids is often necessary. Multiple “steroid-sparing” treatment options exist with the most recently studied being biologic agents. 1. Introduction as alleles that cause deficiency in complement components— C1q, C2, and C4 [5]—have all been linked to lupus. Systemic lupus erythematosus (SLE) is a chronic, autoim- mune, connective tissue disorder affecting multiple organ systems often with a relapsing and remitting clinical course. 3. Mechanism of Disease Prevalence, clinical manifestations, and morbidity vary sig- nificantly between the developing and industrialized worlds. SLE is a complex disease process demonstrating dysregula- While SLE is more common in people of African and Asian tion of the immune system at multiple levels. Autoantibodies descent, thrombotic complications are more common in against double-stranded DNA were first isolated from kidney Caucasian patients [1]. The highest prevalence has been specimens in patients with lupus nephritis in 1967 [6]. reported in Italy, Spain, Martinique, and the UK Afro- Other autoantibodies that have been implicated in disease Caribbean population [2]. The median age of onset is include anti-Ro, La, Sm, nucleosome, NMDA receptor, between the late teens and early 40s with a 9 times phospholipid, and α-actinin. Two major theories exist on higher incidence in women compared to men. Ocular how these autoantibodies cause tissue damage. The first manifestations—occurring in up to one third of patients— model suggests that anti-double-stranded DNA antibodies can be associated with significant morbidity and also a bind to circulating nucleosomes to form immune complexes marker for overall systemic disease activity. that then get deposited in end-organ capillary beds such as the renal glomerulus and activate immune/inflammatory responses [7]. The second hypothesizes that these autoanti- 2. Genetic Considerations bodies cross-react with normal renal proteins causing tissue destruction [8]. The source of autoantigens that trigger Concordance rates for SLE among monozygotic and dizy- the formation of the aforementioned antibodies is thought gotic twins are 25% and 2%, respectively, suggesting a sig- to arise from apoptotic cells. Normally, early complement nificant genetic contribution [3]. Major histocompatibility factors, such as C1q, bind cellular debris from apoptotic cells, complex genes, such as HLA-A1, B8, and DR3 [4], as well which facilitate phagocytosis by macrophages. Deficiency of 2 Autoimmune Diseases such complement factors is an independent risk factor for the Subcutaneous inflammation secondary to SLE was first development of SLE [5]. described by Kaposi in 1883, and the term “lupus ery- Mass production of autoantibodies relies on multiple fac- thematosus panniculitis” was coined in 1940 [14]. It is tor, which have each independently been targeted as potential most commonly encountered in the setting of discoid lupus immunotherapy in the treatment of lupus. Important steps erythematosus. Clinical findings include tender deep subcu- include T-cell activation via antigen binding to the T-cell taneous nodules usually involving the proximal extremities, receptor and proper costimulation; T-cell activation of B trunk, face, and scalp [15]. Orbital involvement is very cells; production of cytokines such as TNF-α,INF-γ, IL-10, rare and has only been reported in a handful of paper. and B-lymphocyte stimulator. Histopathology shows perivascular lymphocytic infiltration Medications, hormonal influences, and other factors [16]. Response to steroids can be quite dramatic in most such as sunlight have all been implicated in disease exac- cases [16–18]; however, few cases have shown a more erbation. Drug-induced lupus, most commonly due to virulent course with significant enophthalmos secondary to procainamide, hydralazine, and quinidine, usually presents fat atrophy [19] and even melting of orbital structures [20]. with disease involving the skin and joints with renal and CNS manifestations being much more rare [9]. Hormonal 5.2. Periorbita. Periorbital edema is an uncommon mani- replacement therapy has been associated with an increased festation of systemic and discoid lupus erythematosus with risk of mild-to-moderate flares [10]. an overall incidence of 4.8% [21]. It is most common in patients of African decent [22]. Violaceous swelling with overlying eczematous changes without any skin necrosis 4. Diagnostic Criteria is seen. Some cases can resemble chronic blepharitis [23]. Etiologies include nephrosis, increased vascular permeabil- According to the 1982 revised criteria for systemic lupus ity, dermal mucin deposits, and angioedema secondary erythematosus, a diagnosis of SLE can be made by the serial to C1 deficiency. Treatment options include observation or simultaneous presentation of at least 4 of the following [23], topical/intradermal/systemic corticosteroids [24], and 11 criteria: malar rash, discoid rash, photosensitivity, oral antimalarials [23]. ulcers, nonerosive arthritis, serositis, renal dysfunction, neu- rological derangements (i.e., seizures or psychosis), hemato- logic disorder (i.e., anemia, leukopenia, thrombocytopenia), 5.3. Eyelids. Typical lesions of discoid lupus erythematosus immunologic disorder (i.e., anti-DNA antibody, anti-Sm are slightly raised, scaly, and atrophic. Most commonly, they antibody, and false positive VDRL testing), and presence of occur on the head, face, neck, and other sun-exposed areas. ff antinuclear antibodies. Rarely does it a ect the eyelids. Histopathologic study shows a hyperkeratotic epithelium with liquefactive degeneration of the basal layer and a dense perivascular/periappendageal 5. Ocular Manifestations lymphocytic infiltration [25, 26]. Diagnosis in most cases ff is delayed because lesions are often mistaken for blephar- SLE can a ect the periorbita, ocular adnexa, eye, and optic itis and eczema. Patients most commonly present with nerve. The most common association is keratoconjunctivitis chronic erythema, blepharoconjunctivitis with inflammation sicca, while the most visually devastating sequelae occur of the meibomian glands. Long-term complications include secondary to optic nerve involvement and retinal vaso- madarosis, lid scarring, and cicatricial ectropion/entropion occlusion. [26, 27]. 5.1. Orbit. Orbital involvement is a rare manifestation of 5.4. Ocular Surface. The most common ocular manifestation SLE. Vasculitis, myositis, and panniculitis have all been of SLE is keratoconjunctivitis sicca with the majority of described. Signs and symptoms include proptosis, enoph- patients endorsing at least one dry eye symptom [28]. thalmos, orbital pain, blurred vision, chemosis, and restric- Dryness can occur from multiple etiologies. Most patients tion of extraocular movements. with SLE develop a secondary Sjogren’s syndrome. In their Orbital vasculitis leads to nonperfusion of the globe review of 283 SLE patients, Manoussakis et al. [29] identified and extraocular muscles. This has been shown to cause 9.2% who had developed Sjogren’s syndrome (SS). The SLE- irreversible vision loss secondary to ischemic injury to the SS group had a higher frequency of Raynaud’s phenomenon, optic nerve as well as elevated intraocular pressure from anti-Ro antibody, anti-La antibody, and rheumatoid factor neovascular glaucoma [11]. and a lower frequency of renal involvement, lymphadenopa- Orbital myositis is often initially misdiagnosed as bac- thy, and thrombocytopenia. These patients tend to undergo terial orbital cellulitis, as it usually presents
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