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Scleritis Diagnosis & Management

Scleritis Diagnosis & Management. Armando L. Oliver, MD Ocular Immunology & Uveitis Specialist Vitreoretinal Surgeon Boston University Department of Ophthalmology. Scleritis. Ophthalmic disease characterized by inflammation of both the episcleral and scleral tissue.

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Scleritis Diagnosis & Management

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  1. ScleritisDiagnosis & Management Armando L. Oliver, MD Ocular Immunology & Uveitis Specialist Vitreoretinal Surgeon Boston University Department of Ophthalmology

  2. Scleritis Ophthalmic disease characterized by inflammation of both the episcleral and scleral tissue. Approximately 50% of cases are bilateral and 60% have an associated complication involving other ocular structures or alterations in visual function. Approximately 50% of cases are associated with a systemic disease, (~ 40% Rheumatic, 10 % Infectious).

  3. CC: Redness Superficial Radial Vessels Bleaches with PE 2.5% Pt. OK with palpation Responds to topical steroids CC: Pain, often severe! Deep Scleral Plexus “Mesh” Deep persistent violaceous hue Tender to palpation! Topical steroids almost useless! Episcleritis vs. Scleritis

  4. Use good illumination! Open the palpebral fissure widely! Scleritis… Physical Examination

  5. Subtypes Watson & Hayreh, Br J Ophthal. 1976;60:163-191. • Classified scleritis in 3 categories: • Anterior • Necrotizing • Posterior

  6. Anterior Scleritis Two Sub-types: Nodular Diffuse

  7. Associated with poor life prognosis in the “older literature”. Common in patients with RA Necrotizing Scleritis Scleral Necrosis Scleromalacia Perforans

  8. Important! Do not confuse scleromalacia perforans with old inactive scleral thinning! Active Quiet

  9. Posterior Scleritis Patients typically present with proptosis, retrobulbar pain, gaze restriction and a visual field loss (from serous RD).

  10. Posterior Scleritis T-Sign

  11. Anterior Scleritis Keratitis Uveitis Glaucoma Scleral Ectasia Globe Perforation Posterior Scleritis Vitritis CME Serous RD OcularComplications & Associations

  12. Corneal Complications Sclerokeratitis IK- Think Infectious! PUK- Think Vasculitis!

  13. Scleritis and Systemic Disease Akpek EK, et al. Ophthalmology 2004; 111: 501-506

  14. Rheumatoid Arthritis • Additive, Symmetric, Deforming, Inflammatory, Polyarthritis • Joint Stiffness (Swelling & Pain), lasting more than 30 min., worse in AM.

  15. Rheumatoid Arthritis • Rheumatoid factor may serve as a confirmatory test, however it is of limited value as a screening tool.

  16. Systemic Vasculitis • Autoimmune disorders characterized by inflammation of the vessel walls which results in vaso-occlusion and ischemia. • Ex.: Wegener’s Granulomatosis, Polyarteritis Nodosa, Takayasu’s Arteritis, etc… • Wegener’s is the most commonly associated vasculitis. • Not a surprise, it often involves URT!

  17. Systemic Granulomatous Vasculitis URT & Kidneys ROS: Sinusitis, nose bridge deformity, nose bleeds, chest discomfort, cough,... Screening: Chemistry, ANCA, CXR, U/A Sedimentation Rate is not a useful screening test! Wegener’s Granulomatosis

  18. Systemic Lupus Erythematosus • Multi-Systemic Autoimmune Disorder • Type 3 immune complex mediated vaso-occlusion, Not a Vasculitis! • Type 2 Immune Complex mediated Hematologic & Immunologic Phenomena

  19. Malar Rash, Discoid Rash, Photosensitivity. Oral Ulcers Arthritis (Non-Erosive, >2 Peripheral Joints) Serositis (Pleuritis, Pericarditis), Renal Disorder (ie. Nephritis), SLE: Diagnostic Criteria

  20. Neurologic Disorder Psychosis, Seizures Hematologic Disorder Hemolytic Anemia, leukopenia, lymphopenia, thrombocytopenia) Immunologic Disorder + LE Prep, Anti-SS, False + VDRL. ANA test positive. SLE: Diagnostic Criteria

  21. SLE • Most diagnostic criteria are elicited on the past medical history & review of systems. • Most other anomalies (Ie. Nephritis, Serositis,…) would be picked-up on a Vasculitis Work-up. • Most have a SLE diagnosis upon presentation. • N=243, 100% dx. upon presentation. Akpek, EK, et al. Ophthalmology 2004; 111:501-506. • ANA, anti-SS and other immunologic test are generally not useful as screening tests, these serve more as confirmatory exams if other diagnostic criteria are met.

  22. Seronegative Spondyloarthropaties • Ankylosing Spondylitis • Lower Back Pain, > 30 minutes, worse in AM. • Reiter’s Syndrome • Episodic, non-deforming, asymmetric, oligoarthropathy. • May have post infectious etiology. • Psoriatic Arthritis • Inflammatory Bowel Disease associated. • Undifferentiated Spondyloarthropathy.

  23. Check HLA-B27! Remember… The diagnosis is made on a clinical ground. Not all AS is HLA-B27 positive. Seronegative Spondyloarthropathy

  24. Inflammatory Bowel Disease • Chron’s Disease & Ulcerative Cholitis • Diarrhea, Steatorrhea, Abdominal pain, etc… • Usually not screened for if asymptomatic! • HLA-B27 (+) patients typically have AAU. • HLA-B27 (-) patients more often have scleritis.

  25. Rare autoimmune disorder characterize by inflammation of the cartilaginous structures. Patients typically have history of red ears and red nose. Relapsing Polychondritis

  26. May become a life threatening disorder because it may involve the LRT cartilaginous structures. The diagnosis is typically made by a Dermatologist via a direct tissue biopsy. There are no blood tests to aid in the diagnosis. Typically responds well to Dapsone. Relapsing Polychondritis

  27. Do’s BUN & Creatinine U/A CXR ANCA RPR & FTA-Abs Lyme Antibody Don’ts ANA Anti-SS, DS, etc... Rheumatoid Factor Sed. Rate CH 50 Maybe… HLA-B27 What to order?

  28. Clinical Pearl... “If you have not spoken to the patient for at least half hour, you might be missing a diagnosis.”

  29. Treatment... Infectious etiology… If in doubt… Treat!

  30. Treatment Medications required for control of inflammation* • 30.4 % Will require NSAIDs • 31.9 % Will require oral Prednisone • 26.1 % Will require immunosuppressive drugs • 5.8 % Other medications (Antibiotics, Antivirals) *Jabs DA, et al., AJO 2000;130:469-476

  31. Treatment: NSAIDS • Agents of choice are typically characterized more by their anti-inflammatory potency rather than their analgesic potency. • Poor choices are agents which may be good analgesics but poor anti-inflammatory agents. • For example: Flurbiprophen 100mg tid vs. (Ibuprophen 1,600 mg q 4 hr => GI distress)

  32. Treatment: Usual Regimens • Flurbiprophen: 100 mg tid. • Indomethacin: 50 mg tid. • Generally a 1 week trial should be allowed. • If significant improvement is noted, the same dose should be kept to complete 4 weeks. • The dose should be slowly tapered only if the patient becomes absolutely quiet.

  33. Treatment...Usual Regimens & Algorithms. • Once the NSAIDs are being tapered, one should look-out for possible threshold dosages for reactivation. • If such a dosage is found the patient likely has chronic scleritis and is likely to require a long term suppressive dose which may be slightly above the threshold. • If the patient reactivates during a taper, one may consider doubling the dosage at which the patient reactivated and after the patient is ABSOLUTELY QUIET, the taper may be resumed.

  34. Indications for Systemic Steroids. • Failure to improve significantly after a week of treatment with NSAIDs • Failure to reach absolute quiescence after a month of a maximum NSAID dosage. • Posterior Scleritis • Necrotizing Scleritis • Sclerokeratitis with PUK. • Associated Systemic Illness requiring steroids. • NSAID Allergy

  35. Start 1mg/kg po qd. Do not exceed 60 mg/day! Always prescribe along with Calcium supplementation! Prednisone

  36. Weight & arterial blood pressure on every visit… Monthly basic chemistry. Bi-annual serum lipids. Prednisone…

  37. Yearly DEXA Scan… Remember… Osteopenia and Osteoporosis are treatable conditions... Prednisone...

  38. So I started the patient on “Pred. 60”…Now What? Continue Pred 60 until: A) Patient is absolutely quiet... - Then proceed to a standard taper... B) Completes a month of Pred 60. - Start a steroid sparing agent…

  39. The “Standard” Prednisone Taper Always on a weekly basis: First by ten: 60, 50, 40… Then by 5: 35, 30, 25, 20… Then by 2.5: 17.5, 15, 12.5, 10, 7.5, 5, 2.5, stop • F/U on a monthly basis or sooner if a threshold is reached!

  40. Prednisone… looking for thresholds... • Adequate chronic dosages of prednisone should be less or equal to 10mg/day. • If a threshold is found and it is above 10mg/day of prednisone the patient will require a steroid sparing agent.

  41. Prednisone… looking for thresholds... • If a threshold is found the prednisone dose is doubled (not to exceed 60mg), and the “standard taper” is resumed along with a steroid sparing agent if indicated. • If the threshold is less than 10mg, the “standard taper” is modified so that the prednisone is tapered by 1 mg intervals up to a dose slightly above the threshold.

  42. Indications for Steroid Sparing Agents • Failure to absolutely control the inflammation after a month of “Pred 60”. • Failure to control chronic scleritis with a dose of 10mg of prednisone or less. • The development of systemic complications from chronic steroid use. • The presence of a systemic disease that precludes the long term use of prednisone in the setting of chronic scleritis. • The presence of a life threatening disease known to likely be refractory to treatment with steroids alone.

  43. Indications for Steroid Sparing Agents “There is a subset of patients with positive c-ANCA, but no evidence for systemic vasculitis, who have difficult to control scleritis, and typically require systemic immunosuppressive drug therapy” Akpek EK, et al. Ophthalmology 2004; 111: 501-506

  44. Very Strong: Akylating agents: cyclophosphamide, chlorambucil. Moderate to strong: CellCept, Imuran. Mild: Cyclosporine, Methotrexate Probably as effective as prednisone itself. Steroid Sparing Agents

  45. Steroid Sparing Agents • For Example: • Akylating agent: • A patient with Wegener’s • a patient who has active necrotizing scleritis despite a month on Pred 60. • CellCept: • A patient with diffuse anterior scleritis reactivated at 40mg of prednisone/day during a “standard taper”. • Methotrexate: • A patient with rheumatoid arthritis who flared-up at 12.5 mg during a “standard taper”.

  46. A word on Cyclophosphamide... • Pulsed IV cyclophosphamide may not provide adequate immunesuppression for the treatment of ophthalmic disorders. Patients should not be deemed as having “cyclophosphamide resistant eye disease” until oral daily cyclophosphamide has been tried and the WBC count has been brought to a level between 4000-3000.

  47. Biologics Very expensive! Limited coverage by insurance companies. Their use may become more frequent in the future...

  48. That’s all folks!

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