SCLERITIS 4th-6th decades Females>males Most common - Anterior, non-necrotizing, non infectious scleritis Worst prognosis - pos terior, necrotizing, infectious scleritis Systemic disease association Recurrent, Bilateral Deep episcleral vessels Differentiate between infectious vs non infectious ? TREATMENT
ETIOLOGY CONNECTIVE TISSUE DISORDERS: Rheumatoid arthritis (scleritis may be preceding joint disease ) SLE Relapsing polychondritis Sarcoidosis Vasculitis: GPA (Wegner's), PAN, CSS HLAB27 : Ankylosing spondylitis , Psoriatic arthritis, IBD ( Crohn’s), reiter’s INFECT IONS: Bacterial – TB, syphilis , l eprosy Virus – HZV, HSV Acanthamoeba Fungal POST SURGICAL: SICS Penetrating keratoplasty Squint surgery Trabeculectomy Pterygium surgery
PATHOLOGY Scleral biopsy should be done only in exceptional circumstances. Four patterns of inflammation, which can correlate with disease etiology. Zonal, necrotizing granulomatous - Systemic Diffuse, non necrotizing - Idiopathic Necrotizing with micro abscesses - Infection Nonzonal, non diffuse granulomatous
OCULAR MANIFESTATIONS Severe pain which is deep & boring in nature. Pain with EOM Tender on palpation Violaceous hue Scleral oedema Dilated, tortuous episcleral BV’s Scleral thinning leading to staphyloma Topical phenylephrine don’t blanch deep episcleral BV’s.
DOV ? Necrotizing, posterior scleritis, CME , PUK, induced astigmatism, ERD. Raised IOP ? Elevated episcleral venous pressure - Inflammatory cells block emissary veins. Ciliary body effusion – ACG. Uveitis – TM is clogged with inflammatory cells. Corticosteroids - secondary elevation. Cataract surgery : be careful with block and SRBS – risk of perforation.
Instillation of phenylephrine 1. Diffuse Anterior Scleritis Most common least severe anterior to insertion of recti. RA, SLE 2. Nodular Anterior Scleritis R/O infectious etiologies Necrotic change manifests as avascular area in centre of nodule. CLASSIFICATION
3. Necrotizing scleritis with inflammation Vaso occlusive (non perfusion) Granulomatous (vasculitis) Post surgical
4. Scleromalacia Perforans Necrotizing scleritis Without (overt) inflammation. lack of significant symptoms and signs eventually leading to staphyloma. Long-standing rheumatoid arthritis
4. Posterior Scleritis Sight-threatening Inflammation posterior to ora serrata Difficult to recognize because of lack of inflammatory signs Features : SRF (like VKH), ERD, Choroidal mass (like melanoma), Disc oedema, Choroidal folds.
SD OCT B san T SIGN Choroidal thickening FFA – subretinal leakage which increase in late phase.
INVESTIGATIONS CBC ESR, CRP RA factor, anti CCP : Rheumatoid arthritis C ANCA (Anti PR3), P ANCA (Anti MPO) : vasculitis (GPA, PAN) ANA : SLE HLAB27, X ray sacroiliac joint : seronegative arthropathies ACE, calcium : sarcoidosis Cartilage biopsy : relapsing polychondritis CxR , Mantoux : TB VDRL/RPR : syphilis
TREATMENT Topical therapy is usually ineffective except Difluprednate - strong steroid (new drug) Differentiate between infectious (antibiotics) vs non infectious (steroids)? 1. Oral NSAIDs First line of treatment with mild and moderately scleritis . Indomethacin 50 mg TID or sustained-release form 75 mg BD Add PPI or H2 antagonists, monitor RFT 2. Systemic corticosteroids Moderate to severe scleritis Oral prednisone 1 mg/kg/day Pulse IV MP 1 gm 0D X 3 days Chest x ray to R/O TB before starting steroids Add PPI or H2 antagonists (prevent gastric ulcer) Add calcium/VIT D (prevent osteoporosis, aseptic necrosis of femur head) Monitor GRBS (blood sugar elevation)
3. Immunosuppressives / immunomodulators (Off label) Necrotizing scleritis Unresponsive of / intolerant to / long-term therapy of prednisone Severe RA &GPA Oral methotrexate 7.5–25 mg weekly Azathioprine 1.5–2 mg/kg/day Cyclosporine Monitor LFT, RFT, CBC 4. Biologicals (Off label) Refractory scleritis Rituximab 5. Surgical Tectonic grafting Autologous periosteum
EPISCLERITIS SCLERITIS Etiology Idiopathic Connective tissue D/O ( RA ) Symptoms Irritation (mild pain) Severe pain , DOV Episcleral BV’s Superficial Deep Signs Pink Linear BV’s Movable Violaceous hue , Scleral oedema Criss cross BV’s Adherent Staphyloma, PUK, Inc IOP, Dec V/A PHENYLYEPHRINE (10%) Blanch Don’t blanch Classification Simple or nodular Anterior (necro/non necro) Posterior Treatment Topical Systemic Take Home Message