Scleritis Diagnosis, Systemic Associations & Management

1. ScleritisDiagnosis, Systemic Associations & Management Juan G. Santiago, MD Ophthalmology Fellow Beetham Eye Institute Joslin Diabetes Center

2. Chief Complaint “Pain, redness and swelling in the right eye”

3. History of Present Illness • HBB is a 58 y/o male patient with hx of HTN, Multiple Sclerosis (dx 10 years ago), medical hx of syphilis, illicit drugs abuse, migraines, multiple episodes of uveitis that presents with a complaint of right eye pain and blurred vision that had worsened during a period of 2 weeks. • 5 months before presentation: Right eye anterior uveitis was dx, with (+)RPR titers. Patient received 14 days of Penicillin G IV with improvement of condition.

4. History of Present Illness • One month before, patient was found with recurrent right eye anterior uveitis and was send to ER to r/o syphilitic uveitis and new workup. Patient was going to be admitted but he refused, workup was not performed. Patient received home IV therapy with Ceftriaxone 2gm daily, PF oph and atropine oph. Patient refers improvement of symptoms with treatment, reason for which he did not look further medical care.

5. History of Present Illness • Now patient refers right eye pain, blurred vision associated with eye redness, headaches and lid swelling that had worsened for 2 weeks. Patient refers (+) lacrimation, (-) purulent secretions, (+) photophobia, (-) flashlights, (-) curtain effect, (-) floaters. Patient denies recent trauma, viral illness, insect bite or any associated systemic symptoms.

6. Past Medical History • Medications • Antihypertensives (Amlodipine, HCTZ, Lisinopril, Metoprolol) • Copaxone for MS • Habits • Alcohol – For 30 yrs, one "Don Q" bottle almost every night. Quitted 10 years ago. • Tobacco – Active smoker. One pack daily/40 yrs. • Drugs – Marijuana / Cocaine / Denies IVDA • Sex – Active sexual relationships with multiple male partners

7. Past Medical History • Allergies • Amphotericin B • Dramamine • Family History – DM, HTN and Heart diseases. • Childhood illness – Chickenpox • Operations – Left femur fracture repair

8. ROS General - No fever Head – Headaches Ear – No earache Eyes – Blurred vision, redness, lacrimation, pain Nose – Denies bleeding, sinusitis Throat – No sore throat, no pain on swallowing Neck – No lumps Respiratory – No cough, no SOB, no blood in sputum Cardiovascular – No chest pain GI – Denies nausea, vomits or change in bowel habits GU – Incontinence, no hematuria Lymph – No nodes MSK – Joint pain at afternoons, no stiffness Endocrine – no heat or cold intolerance, no loss of libido PV – no discoloration of fingers Neuro - Depression

9. Exam

10. Exam

11. B-scan Right Eye • Scleral thickening • T sign • Sub-tenon's effusion at the junction of the optic nerve and globe **Not actual B-scan from our patient**

12. Imaging (6 months before)

13. Imaging (6 months before)

14. Imaging (@ presentation)

15. Imaging (@ presentation)

16. Imaging (@ presentation)

17. Imaging (@ presentation)

18. Imaging (@ presentation)

19. Imaging (@ presentation) ORBIT CT: (Official report) • Right periorbital and retro orbital inflammatory changes with marked thickening and enhancement of the right globe. There is a medial suprachoroidal collection, slightly dense than vitreous, r/o hemorrhagic or infectious component. Soft tissue stranding of the intraconal fat seen. There is enhancement of the entire optic nerve. • Unremarkable left eye.

20. Imaging (@ presentation)

21. Imaging (@ presentation)

22. Workup • CBC • WBC 14.3 • Segs 66.9 • Lymphs 20.9 • Monos 10.4 • RBC 4.8 • HGB 13.1 • HCT 39.9 • PLT 291 • ESR >120 • C-RP 145.3 • U/A  Negative • Chem • Glu 118 • Bun 24.1 • Crea 1.20 • Na 134 • K 3.5 • Cl 93 • CO2 29

23. Workup • Chem • Ca 10.0 • Mg 2.5 • Alb 3.3 • Toxicology • Cocaine – Neg • Opiates – Neg • Cannabis – 64.63 • CSF • VDRL – Nonreactive • Cryptoc – Neg • Fluid App – Clear • Glu 105 • Prot 41.2 • Cultures - Neg • Serum Prot Electroph • WNL

24. Workup • Serology • HIV Elisa – Neg • RF – Neg • Hepatitis B – Neg • Hepatitis C – Neg • ANA – Neg • RPR – Rx 1:1 (Neg) • MHA-TP – Reactive • ACE – 54 WNL • ANCA – (+) Atypical • Toxo – Neg • HSV I – Neg • HSV II – Pos • VZV – Pos • Blood culture – Neg • Mycobacteria – Neg

25. Differential Diagnosis • Idiopathic scleritis (50%) • Infectious disease (10%) • Herpes zoster ophthalmicus • Herpes simplex keratitis • Syphilis • Lyme disease • Rheumatic disease (40%) • Rheumatoid arthritis • Systemic vasculitis • Wegener’s granulomatosis • Systemic lupus erythematosus • Relapsing polychondritis • Inflammatory bowel disease • Spondyloarthropathy

26. Scleritis • Ophthalmic disease characterized by inflammation of both the episcleral and scleral tissue. • Approximately 50% of cases are bilateral. • 60% have an associated complication involving other ocular structures. • 16% had a decrease in visual acuity.

27. Episcleritis vs. Scleritis 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!

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

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

30. Anterior Scleritis Two Sub-types: Nodular Diffuse

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

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

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

34. Posterior Scleritis T-Sign

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

36. Corneal Complications Sclerokeratitis Interstitial Keratitis: Think Infectious! Peripheral Ulcerative Keratitis: Think Vasculitis!

37. ~78% of the patients with scleritis and an associated medical condition had the associated disease present before the diagnosis of scleritis • ~14% of patients an associated disease was diagnosed as a result of the initial evaluation • ~8% of patients developed an associated disease during follow up Akpek EK, et al. Ophthalmology 2004; 111: 501-506

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

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

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

41. 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, GCA, Behcet’s disease • Wegener’s is the most commonly associated vasculitis • 50% of the vasculitis diagnoses • Not a surprise, it often involves URT!

42. Only 59% of the patients had a diagnosis of vasculitis before the diagnosis of scleritis, whereas 84% of patients with other rheumatic diseases had a diagnosis before presenting with scleritis. • Because of the potential for life-threatening complications of systemic vasculitis, all patients with scleritis should be evaluated carefully for it’s presence.

43. Wegener’s Granulomatosis • Systemic GranulomatousVasculitis • 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!

44. 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

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

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

47. 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=10, 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.

48. 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.

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

50. 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.