When attacked by organisms and other foreign molecules, the body’s immune system responds with an impressive defense.
But when the body mistakes its own cells for invaders, the results can be devastating. In autoimmune diseases, the body has a misguided immune response in which it manufactures T cells and antibodies directed against its own cells and organs.1

In this column, the first of a two-part series, we focus on systemic lupus erythematosus (SLE), a common chronic autoimmune disease that affects multiple systems in the body.1,2

In SLE and other autoimmune diseases, the immune system’s recognition apparatus breaks down—specifically, misguided T cells and autoantibodies contribute to the development of these conditions.1,2 They begin to destroy healthy cells and tissues, leaving the body unable to perform vital functions and making it vulnerable to attack from actual pathogens.

Let’s review some basics of this complex disease, potential signs and symptoms, and how it affects the eye specifically.

Note the multiple cotton wool infarcts and flame hemorrhages in this patient with systemic lupus erythematosus.
The Impact of Lupus
In 1851, doctors coined the name “lupus erythematosus” for a disease frequently characterized by a facial rash that looked like the bite of a wolf (lupus means wolf; erythema means redness).4

There are several categories of lupus, including:

  • SLE
  • Discoid
  • Subacute cutaneous lupus erythematosus
  • Drug-induced
  • Neonatal

Of these subtypes, SLE is the most common and serious. Estimates of its prevalence vary considerably, but one sizable national review suggested that 161,000 Americans have definite SLE—while as many as 322,000 have definite or probable SLE.5    

While the disease can affect a wide patient demographic, it does discriminate. SLE affects women more frequently than men and is more common in blacks, Hispanics, Asians and Native Americans than in whites.6 Its onset usually occurs between ages 15 and 45, but it sometimes appears earlier or later in life.6

Episcleritis in another patient with SLE. 
Etiology and Patient History
Perform a thorough history and review of systems for patients suspected of having SLE, at minimum covering the following symptoms and signs:2,3

  • Alopecia
  • Anemia
  • Arthritis
  • Edema
  • Fatigue
  • Fever
  • Pleurisy
  • Photosensitivity
  • Seizures
  • Skin rashes
  • Ulcers of the mouth or nose.

The specific etiology of SLE is unknown, although clinicians and researchers consider it to be multifactorial.2 Previous and current investigations suggest a role for genetic, hormonal, immunologic and environmental factors. This wide range of contributing factors may help explain SLE’s variable clinical manifestations.7-9

The Eye in SLE

Reported Ocular Complications of SLE7-9
Ocular Anatomy    
SLE Complication
Discoid rash, blepharitis
Ocular surface
Keratoconjunctivitis sicca, recurrent corneal erosions
Episclera/sclera Episcleritis and scleritis of variable type and severity
Anterior chamber    Uveitis, frequently accompanies episcleritis/scleritis
Posterior segment 
Cotton-wool infarct, retinal hemorrhages, hard exudates, retinal vascular occlusions, vasculitis, proliferative retinopathy
Choroid               Ischemia, effusions
Optic nerve 
Optic neuritis, ischemic optic neuropathy
Oculomotor disorders     Secondary to vasculitic or ischemic events
Pupil disorders      
Horner’s syndrome, tonic and light-near dissociation of pupils
Visual pathway 
Retrochiasmal disease, intracranial hypertension
The course of SLE may be unpredictable, with periods of exacerbation and remission. The skin, kidneys, lungs, spleen, joints, mucous membranes, central nervous system and heart are the organs principally affected.2-4 However, complications from SLE can involve almost any organ system, including ocular tissues.

SLE may affect the eyes and/or visual system in up to one-third of patients. Ocular manifestations of SLE are mediated directly or indirectly by antibody formation and the creation of immune complexes. Complications may be sight threatening, and virtually every component of the eye and visual pathway may be affected.

Keratoconjunctivitis sicca is the most common finding in the eye, but other ophthalmic sites of involvement include the cornea, conjunctiva, episclera, sclera, uveal tract, retinal vasculature, optic nerve and orbit.9 In fact, ocular manifestations may be the presenting sign of SLE and can be a useful indicator of underlying systemic disease activity.9

Stay tuned for part two of this series in the March 2013 issue, where we will discuss the diagnostic workup, treatment and management of SLE.

1. Male D, Brostoff J, Roth DB, Roitt I. Immunology. 8th ed. Philadelphia: Elsevier Saunders; 2012.
2. Diseases of Immunity. In: Kumar V, Cotran RS, Robbins S (eds.). Robbins Basic Pathology. 7th ed. Philadelphia: Saunders; 2003:103-64.
3. Siegel RM, Lipsky PE. Autoimmunity. In: Firestein GS, Budd RC, Harris Ed, et al. (eds.). Kelley’s Textbook of Rheumatology. 8th ed. Philadelphia: Saunders Elsevier; 2009.
4. Blotzer JW. Systemic lupus erythematosus I: historical aspects. Md State Med J. 1983 Jun; 32(6):439-41.
5. Helmick CG, Felson DT, Lawrence RC, et al. Estimates of the prevalence of arthritis and other rheumatic conditions in the United States. Part I. Arthritis Rheum. 2008 Jan;58(1):15-25.
6. National Institute of Arthritis and Musculoskeletal and Skin Diseases. Handout on health: systemic lupus erythematosus. Available at: www.niams.nih.gov/Health_Info/Lupus/. Accessed December 27, 2012.
7. Arevalo JF, Lowder CY, Muci-Mendoza R. Ocular manifestations of systemic lupus erythematosus. Curr Opin Ophthalmol. 2002 Dec;13(6):404-10.
8. Sivaraj RR, Durrani OM, Denniston AK, et al. Ocular manifestations of systemic lupus erythematosus. Rheumatology (Oxford). 2007 Dec;46(12):1757-62.
9. Read RW. Clinical mini-review: systemic lupus erythematosus and the eye. Ocul Immunol Inflamm. 2004 Jun;12(2):87-99.