1. Grand Rounds Conference
Eric Downing MD
Sclerod...malacia Perforans
University of Louisville
Department of Ophthalmology and Visual Sciences

2. Subjective
CC/HPI: 68F referred by outside ophthalmologist for eval. Pt has been having irritation and light sensitivity OD for 7 years, but has gotten worse recently
Ptosis – progressive over several years, OD > OS. Holds lids up to see.

3. History
POH: none PMH: Rheumatoid Arthritis(~30 years), HLD, HTN Eye Meds: Art tears, Restasis OU BID Meds: Prednisone 5mg PO daily, MTX 25mg weekly, Enbrel 50mg weekly
FOH: Glaucoma
FH: Cancer, stroke

4. Objective OD OS VA: 20/300 20/25 Pupils: 4→3 4→3, no rAPD IOP: 20 13
EOM: full OU
Levator function > 10 mm is excellent; < 5 is poor

5. Objective
PLE: E/L/L WNL OU C/S Superior scleral thinning OD K few SPEs OU AC no cell or flare OU I/L PCIOL/1+NS Vit WNL DFE: ON/M/V/P: all WNL

6. Clinical photos

7. Assessment
68F with Rheumatoid Arthritis and scleral thinning OD without inflammation
Dx: Scleromalacia
Plan: referral back to rheumatologist, monitor for progression and development of glaucoma

8. Scleromalacia Perforans
AKA necrotizing scleritis without inflammation
Distinct from other forms of scleritis, which are characterized by redness, edema, and pain are not apparent here
Typically associated with RA, but has also been reported with Granulomatosis with Polyangitis, SLE, JRA, Relapsing Polychondritis, TB, Gout, Syphilis, HSV, and HZV

9. Anterior Scleritis Three forms
Diffuse: non-granulomatous reaction with anterior scleral edema with episcleral venous dilation
Nodular: one or more nodules, usually tender to palpation
Necrotizing: granulomatous reaction with a central area of fibrinoid necrosis
With inflammation
Without inflammation—Scleromalacia perforans

10. Pathophyisiology
Inflammatory process thought to be caused by a type III hypersensitivity (immune complex) and possibly subsequent type IV reaction
Causes immune complex vessel disposition in episcleral and scleral vasculature with thinning of the adjacent tissue

11. Epidemiology More common in women (3:2)
Most common in fourth to sixth decades
1/3 of diffuse/nodular scleritis and 2/3 of patients with necrotizing scleritis is associated with connective tissue or autoimmune disease
½ of cases are bilateral at some point

12. Medical Treatment Diffuse/Nodular: Severe nodular/necrotizing disease:
Oral NSAIDs
Topical steroids
Subconjunctival steroids
Severe nodular/necrotizing disease:
Oral steroids
MTX
Infliximab
Cyclosporine
Cyclophosphamide
Sjogren and Hashimoto thyroiditis; HIV

13. Surgical Treatment
For extreme corneal/scleral thinning or perforation, donor sclera or fascia lata, or periosteum can be used

14. Literature review of 30 manuscripts
No RTC, on retrospective reviews, case reports, and case series
All cases were scleritis refractory to immunosuppressive therapy
Infliximab/adalimumab is more effective than etanercept
Also effective at treating PUK
Rituximab
Good option for necrotizing scleritis and vasculitis-associated scleritis
60% of patients were able to d/c treatment after only one cycle
500mg better than1000mg dose

15. Actemra, IL-6 receptor antagonist
Retrospective review of 17 patients who had failed treatment with other immunosuppresive and biological therapies
50% of scleritis patients achieved quiescence
¾ achieved remained quiet through 32 months
¼ stopped due to side effects, such as neutropenia, nausea, edema, +/- GI upset
Tocilizumab is moderately effective

16. References BCSC Section 8: External Disease and Cornea, pp 204-212.
Akpek, EK. Necrotising Scleritis: Diagnosis and Therapy, PDF.
Watson PG, Hayreh SS. Scleritis and episcleritis. Br J Ophthalmol 1976; 60:
Sales AF, Vieira LA, Freitas D. Biologic therapy for refractory scleritis: a new treatment perspective. Int Ophthal Dec 2015; 35:6 pp