Grand Rounds Scleromalacia Amir R. Hajrasouliha, M.D. University of Louisville Department of Ophthalmology and Visual Sciences Friday, January 17, 2014

Patient Presentation HPI: 79 y/o white male present for annual work up. He has a h/o dry AMD with no new complaint. He has no pain or discomfort. HPI: 79 y/o white male present for annual work up. He has a h/o dry AMD with no new complaint. He has no pain or discomfort.

Patient Presentation POH: POH: AMD dry AMD dry PMH: PMH: Rheumatoid arthritis Rheumatoid arthritis H/o bladder cancer H/o bladder cancer Meds: Meds: AREDS AREDS Plaquenil 400mg for 16 years (2,304g total) Plaquenil 400mg for 16 years (2,304g total) Allergies: Allergies: NKDA NKDA SH: SH: No tobacco, no ETOH No tobacco, no ETOH ROS: ROS: Negative (No joint pain and swelling now) Negative (No joint pain and swelling now)

Exam VA cc P T TP VA cc P T TP EOM: Full OU CVF: FULL OU 20/40 3  2mm (-) RAPD OU 20/25

ODOS Extwnlwnl L/Lwnlwnl Conjsup and tempwnl scleral thinning scleral thinning Kclearclear ACformedformed Iriswnlwnl Lens2+ NS2+NS Anterior exam

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Assessment 79 year old male with rheumatoid arthritis and scleral thinning without inflammation OD 79 year old male with rheumatoid arthritis and scleral thinning without inflammation OD Differential Diagnosis Differential Diagnosis scleromalacia scleromalacia Plan Referral to rheumatologist Referral to rheumatologist

Scleromalacia Also known as necrotizing scleritis without inflammation. Also known as necrotizing scleritis without inflammation. It is clinically distinct from other forms of anterior scleritis in which typical signs (redness, edema) and symptoms (pain) of inflammation are not apparent. It is clinically distinct from other forms of anterior scleritis in which typical signs (redness, edema) and symptoms (pain) of inflammation are not apparent.

Scleromalacia Typically occurs in patient with long standing rheumatoid arthritis. Typically occurs in patient with long standing rheumatoid arthritis. It has been also reported to have association with Wegener's granulomatosis, SLE, JRA, PAN, Relapsing Polychondritis, psoriasis, gout, TB, syphilis, HSV, HZV. It has been also reported to have association with Wegener's granulomatosis, SLE, JRA, PAN, Relapsing Polychondritis, psoriasis, gout, TB, syphilis, HSV, HZV.

Scleromalacia A bulging staphyloma develops if intraocular pressure is elevated; A bulging staphyloma develops if intraocular pressure is elevated; Spontaneous perforation Spontaneous perforation is rare, although these eye is rare, although these eye may rupture with minimal Trauma.

Case report A clinical case of scleromalacia perforans in a 56-year-old woman with 20 years of seropositive rheumatoid arthritis. She developed rapidly progressed to scleromalacia perforans OS and became perforated. It was surgically enucleated, and the patient was maintained with steroidal therapy. A clinical case of scleromalacia perforans in a 56-year-old woman with 20 years of seropositive rheumatoid arthritis. She developed rapidly progressed to scleromalacia perforans OS and became perforated. It was surgically enucleated, and the patient was maintained with steroidal therapy. 2 months later she developed new-onset scleromalacia OD. She was first evaluated by a rheumatologist and treated with 200 mg/dose of infliximab, which was administered monthly for the following four months. The biological treatment was accompanied by methotrexate and prednisone. With this therapy, the ocular lesion dramatically improved, and complete remission of rheumatoid arthritis and scleritis was archived four months later. In conclusion, tumur necrosis factor (TNF) blockers are effective therapeutic agents in ocular complications of rheumatoid arthritis. 2 months later she developed new-onset scleromalacia OD. She was first evaluated by a rheumatologist and treated with 200 mg/dose of infliximab, which was administered monthly for the following four months. The biological treatment was accompanied by methotrexate and prednisone. With this therapy, the ocular lesion dramatically improved, and complete remission of rheumatoid arthritis and scleritis was archived four months later. In conclusion, tumur necrosis factor (TNF) blockers are effective therapeutic agents in ocular complications of rheumatoid arthritis. Reumatismo Jul-Sep;61(3):212-5.Infliximab treatment in a case of rheumatoid scleromalacia perforans.

MEDICAL TREATMENT In patients with simple diffuse or nodular scleritis systemic non-steroidal anti- inflammatory drug therapy is almost invariably effective In patients with simple diffuse or nodular scleritis systemic non-steroidal anti- inflammatory drug therapy is almost invariably effective For unresponsive cases and posterior scleritis, the mainstay of treatment is systemic steroids in a dose of 1 mg/kg/day. As soon as the patient responds, the dose should be tapered once 20 mg/day is reached, alternate day therapy can be started. Topical steroids can be applied for symptom relief. For unresponsive cases and posterior scleritis, the mainstay of treatment is systemic steroids in a dose of 1 mg/kg/day. As soon as the patient responds, the dose should be tapered once 20 mg/day is reached, alternate day therapy can be started. Topical steroids can be applied for symptom relief. Immunosuppressive therapy is mandatory for definitively diagnosed systemic vasculitic disease and/or progressive destructive ocular lesions. If the necrotizing scleritis is not severe, not rapidly progressing, the first choice of therapy is methotrexate 7.5 mg once a week as a starting dose Immunosuppressive therapy is mandatory for definitively diagnosed systemic vasculitic disease and/or progressive destructive ocular lesions. If the necrotizing scleritis is not severe, not rapidly progressing, the first choice of therapy is methotrexate 7.5 mg once a week as a starting dose

Scleromalacia Extreme corneal thinning or perforation requires reinforcement. Donor sclera, fascia lata, Extreme corneal thinning or perforation requires reinforcement. Donor sclera, fascia lata, periosteum or artificial materials can be used. To maintain its integrity the material must be covered by conjunctiva. periosteum or artificial materials can be used. To maintain its integrity the material must be covered by conjunctiva.

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