1	Necrotizing Scleritis in Wegener’s Granulomatosis by Peter Maris, Jr.
2	Patient History 59 y/o Hispanic male with hx. biopsy-proven Wegener’s granulomatosis c/o redness and boring pain in both eyes x 2 months, OS worse than OD. Pt. also reports minimally decreased vision in OS over past several weeks.
3	Past Medical History Wegener’s diagnosed in ’98 when pt. presented with cough, hemoptysis and myalgias. Lung biopsy positive. hx. recurrent sinus disease. End-stage renal disease (on hemodialysis 3x/week). s/p several treatments with cytoxan and solumedrol – last cytoxan tx. 7 months prior. past cytoxan treatments complicated by leukopenia and opportunistic infections (VZV and CMV).
4	"Medications:" Medications: Coumadin, Oscal, Nephrovite, Fosamax, Indomethacin – 75mg BID, Artificial Tears – 0/2 POHx.: No hx. surgery or trauma; wears bifocals
5	Clinical Exam Va(w/gl.): OD 20/25 OS 20/50 ph> 20/30 Pupils: reactive OU, no APD Motility: full OU External: no proptosis, no resistance to retropulsion; pain on mild palpation OU
6	Slit Lamp Exam l/a – WNL OU c/s – injection of superficial and deep episcleral vessels OU; edematous sclera and episclera OU; bluish hue to sclera superiorly, particularly OS. K- limbal stromal haze OU AC – deep and quiet OU I/L – WNL and clear OU
7	"Slit lamp photographs from the..." Slit lamp photographs from the first presentation
8	Additional Exam Tonometry: 13 mm Hg OU Topical phenylephrine (10%): no blanching of scleral vessels OU Dilated Fundus Exam: no disc swelling, no choroidal folds or exudative retinal detachment, no retinal hemorrhages Fluorescein Angiogram: unremakable
9	Fluorescein Angiogram
10	Treatment Overnight hospitalization for: Solumedrol – 1 gram IV x 1 Cytoxan (Cyclophosphamide) – 500 milligrams x 1
11	Slit lamp photographs of both eyes 10 days after first presentation
12	Scleritis – differential diagnosis (common) Wegener’s granulomatosis rheumatoid arthritis relapsing polychondritis systemic lupus erythematosis Reiter’s syndrome polyarteritis nodosa ankylosing spondylitis herpes zoster ophthalmicus syphilis gout
13	Scleritis – differential diagnosis (less common) tuberculosis Lyme disease sarcoidosis parasitic infections other bacteria (Pseudomonas species)
14	Wegener’s Granulomatosis - triad necrotizing granulomatous vasculitis of the upper and lower respiratory tracts variable degrees of small-vessel vasculitis focal necrotizing glomerulonephritis
15	Ophthalmic Manifestations Bullen CK et al. Ocular Complications of Wegener’s Granulomatosis. Ophthalmology, 1983. 140 pts. with biopsy-proven Wegener’s were examined at Mayo Clinic, btw. 1966 –1982 40 pts. (28.6%) had ophthalmic complications substantial mortality/ ocular morbidity: 4 patients died 3 eyes were enucleated
16	Sites of Involvement No. of Patients Percentage(%) Orbital 18 45 Scleral/Episcleral 15 38 Corneal 11 28 Conjunctival 6 15 Eyelid 8 20 Nasolacrimal 10 25 Optic Nerve 9 25 Retinal 7 18 Uveal 4 10
17	Treatment - Scleritis Non-necrotizing anterior scleritis: oral non-steroidal anti-inflammatory drugs more severe: oral corticosteroids necrotizing scelritis: immunosuprssive agents w/wo systemic steroids
18	Recommendations of an Expert Panel Jabs DA et al. Guidelines for the Use of Immunosuppressive Drugs in Patients with Ocular Inflammatory Disorders. American Journal of Ophthalmology 2000.: Cyclophosphamide – starting at 2mg/kg is the preferred drug for necrotizing scleritis in setting of systemic vasculitis. concomitant steroid therapy should be initially started as well, which can then be tapered over first 2-4 months of cytotoxic therapy.
19	Side Effects of Cyclophosphamide bone marrow suppression (granulocytopenia and lymphopenia) risk of opportunistic infections (P. carinii) hemorrhagic cystitis teratogenicity (contraindicated in pregnancy) alopecia
20	In Summary Scleritis, particularly necrotizing scleritis, can both jeapordize the integrity of the eye and be representative of a life-threatening systemic vasculitis. Appropriate aggressive treatment, often with immunosuppressives, is important for preserving the eye and preventing nonocular morbidity.