Lupus and Your Eyes


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by Cono Grasso, MD
Jamaica Hospital Medical Center

Presented at the S.L.E. Lupus Foundation's "Get into the Loop!" New York City Lupus Education Series on October 6, 2010.

Published in: Health & Medicine

Lupus and Your Eyes

  1. 1. Ophthalmic Manifestations of Systemic Lupus Erythematous Cono M. Grasso, M.D. Chairman, Department of Ophthalmology Jamaica Hospital Medical Center
  2. 2. Etiology and Pathogenesis <ul><li>Although the etiology is unknown there appears to be a genetic predisposition </li></ul><ul><li>Is a multisystem autoimmune disease and thus may affect almost any organ system </li></ul>
  3. 3. Clinical Features <ul><li>The incidence of SLE has been estimated as 2-9 cases per 100,000 per year </li></ul><ul><li>Has a chronic course marked by periodic exacerbations of varying severity </li></ul>
  4. 4. Clinical Findings <ul><li>Cutaneous disease (85%) </li></ul><ul><li>Mucosal lesions (40%) </li></ul><ul><li>Articular disease (80-85%) </li></ul><ul><li>Renal disease (50%) </li></ul><ul><li>Neurological disorders </li></ul><ul><li>Hematological disorders </li></ul><ul><ul><li>Often have an anemia seen in patient’s with chronic disease </li></ul></ul><ul><ul><li>Thrombocytopenia (33%) </li></ul></ul><ul><li>Immunological disorders </li></ul>
  5. 5. Treatment <ul><li>Because SLE is a multisystem disease with highly variable features treatment is directed towards the specific symptoms in any given patient. </li></ul>
  6. 6. Ocular Manifestations <ul><li>Involvement of the skin and eyelids with cutaneous disease </li></ul><ul><ul><li>These lesions develop as a red, inflamed patch with a scaling and crusty appearance. The center areas may appear lighter in color with a rim darker than the normal skin </li></ul></ul><ul><ul><li>Lesions usually involve the lid margins </li></ul></ul>
  7. 7. Ocular Manifestations <ul><li>Secondary Sjorgen’s Syndrome </li></ul><ul><ul><li>Seen in 20% of patients with SLE </li></ul></ul><ul><ul><li>Indistinguishable from the sicca component that is seen in other patients with rheumatic disorders </li></ul></ul>
  8. 8. Dry eye syndrome
  9. 9. Dry Eye Syndrome
  10. 10. Dry Eye Syndrome
  11. 11. Sjorgen’s Syndrome Diagnosis & Treatment <ul><li>Slit Lamp examination </li></ul><ul><li>Tear Break –up time testing </li></ul><ul><li>Schirmer (2) testing </li></ul><ul><li>Tear replacement therapy </li></ul><ul><li>Restasis </li></ul>
  12. 12. Ocular Manifestations <ul><li>Retinopathy </li></ul><ul><ul><li>The most common form of ocular involvement in SLE patients </li></ul></ul><ul><ul><li>Retinopathy mainly consists of nerve fiber layer infarcts (cotton-wool spots) with or without intraretinal hemorrhages </li></ul></ul><ul><ul><ul><li>Frequency of this finding varies with patient population studied </li></ul></ul></ul><ul><ul><ul><ul><li>3% of ambulatory patients </li></ul></ul></ul></ul><ul><ul><ul><ul><li>28-29% of hospitalized patients with active SLE </li></ul></ul></ul></ul>
  13. 13. Nerve Fiber Layer Infarcts
  14. 14. Lupus Retinopathy
  15. 15. Ocular Manifestations <ul><li>Retinopathy (continued) </li></ul><ul><ul><li>Occurs independently of hypertension </li></ul></ul><ul><ul><li>IVFA suggests mild background retinopathy with microaneurisms and telangiectatic vessels may be relatively common </li></ul></ul><ul><ul><li>Doppler ultrasound suggests altered microcirculatory hemodynamics in the ophthalmic artery and central retinal artery </li></ul></ul>
  16. 16. Ocular Manifestations <ul><li>Retinopathy (continued) </li></ul><ul><ul><li>Severity of the retinovascular changes correlates with the activity of the disease </li></ul></ul><ul><ul><li>May see vascular occlusive disease </li></ul></ul><ul><ul><ul><li>CRAO </li></ul></ul></ul><ul><ul><ul><li>CRVO </li></ul></ul></ul><ul><ul><ul><li>BRAO </li></ul></ul></ul><ul><ul><ul><li>BRVO </li></ul></ul></ul><ul><ul><ul><li>Diffuse retinal vaso-occlusive disease </li></ul></ul></ul><ul><ul><ul><ul><li>Poorest prognosis with 50% of affected eyes becoming blind </li></ul></ul></ul></ul>
  17. 17. Branch Vein Occlusion
  18. 18. Central Retinal Vein Occlusion
  19. 19. Branch Retinal Artery Occlusion
  20. 20. Central Retinal Artery Occlusion
  21. 21. Ocular Manifestations <ul><li>Retinopathy (continued) </li></ul><ul><ul><li>Choroidopathy is rather uncommon </li></ul></ul><ul><ul><li>Usually seen when patients have hypertension because if lupus nephropathy or systemic vasculitis </li></ul></ul>
  22. 22. Lupus Choroidopathy
  23. 23. Ocular Manifestations Treatment <ul><li>Retinopathy </li></ul><ul><ul><li>Treatment of often directed towards that of the underlying disease </li></ul></ul><ul><ul><ul><li>Systemic corticosteroids </li></ul></ul></ul><ul><ul><ul><li>Immunosuppressive agents </li></ul></ul></ul><ul><ul><li>In patients with diffuse retinovascular disease often panretinal photocoagulation is of benefit </li></ul></ul><ul><ul><ul><li>50% of eyes with severe vaso-occlusive disease become legally blind </li></ul></ul></ul><ul><ul><ul><li>In the more common mild retinopathy patients visual loss is unusual </li></ul></ul></ul>
  24. 24. Ocular Manifestations <ul><li>Neuro-ophthalmic involvement </li></ul><ul><ul><li>Any of the cranial nerve palsies </li></ul></ul><ul><ul><li>May get visual field defects </li></ul></ul><ul><ul><li>May get visual hallucinations </li></ul></ul><ul><ul><li>Lupus Optic neuropathy (1-2%) </li></ul></ul><ul><ul><ul><li>May be a retrobulbar neuritis </li></ul></ul></ul>
  25. 25. Ocular Manifestations <ul><li>Scleritis </li></ul><ul><ul><li>Less common in SLE than the other rheumatic disorders (4%) </li></ul></ul><ul><ul><li>Any type of scleritis may be seen but the anterior form is more common </li></ul></ul><ul><ul><ul><li>Diffuse </li></ul></ul></ul><ul><ul><ul><li>Nodular </li></ul></ul></ul><ul><ul><ul><li>Necrotizing </li></ul></ul></ul>
  26. 26. Scleritis
  27. 27. Scleritis
  28. 28. Scleritis
  29. 29. Scleritis
  30. 30. Ocular Manifestations Treatment <ul><li>Scleritis </li></ul><ul><ul><li>Usually directed towards controlling the patient’s SLE </li></ul></ul><ul><ul><li>Oral non-steroidal anti-inflammatory agents </li></ul></ul><ul><ul><li>Topical steroids </li></ul></ul><ul><ul><li>Low –dose oral steroids </li></ul></ul><ul><ul><li>In necrotizing scleritis may use oral cytotoxic agents </li></ul></ul>
  31. 31. Complications of treatment <ul><li>Cataracts </li></ul><ul><ul><li>(secondary to corticosteroid usage) </li></ul></ul><ul><li>Bulls-eye retinopathy </li></ul><ul><ul><li>(secondary to hydroxychoroquine or chloroquine) </li></ul></ul><ul><ul><li>Side effects are dose related </li></ul></ul><ul><ul><li>Less than 5% when on hydroxychoroquine less than 400mg per day </li></ul></ul><ul><ul><li>May be reversible if caught early </li></ul></ul>
  32. 32. Cataract
  33. 33. Cataract
  34. 34. Complications of Treatment <ul><li>Bulls –eye maculopathy (continued) </li></ul><ul><ul><li>Patients need base-line ophthalmic exam </li></ul></ul><ul><ul><li>Visual field testing </li></ul></ul><ul><ul><li>Follow-up examinations </li></ul></ul><ul><ul><ul><li>If dose is 400mg daily – every 6 months </li></ul></ul></ul><ul><ul><ul><li>If dose is 200 mg daily – once per year </li></ul></ul></ul>
  35. 35. Chloroquine retinopathy
  36. 36. Chloroquine retinopathy