Background/Purpose:   Peripheral Ulcerative Keratitis (PUK) is a severe inflammation of the outer portions of the cornea that may be associated with systemic conditions. The progression of marginal corneal thinning may lead to perforation of the cornea and to rapid and permanent vision loss in the involved eye. Treatment of PUK is based on corticosteroids and conventional systemic immunosuppressive drugs. Our aim was to evaluate the response to biologic therapy in cases with severe and refractory PUK.

Methods:   Multicenter study of 27 (35 affected eyes) patients. All of them presented inadequate response to conventional therapy with corticosteroids and at least 1 systemic traditional immunosuppressive drug. The main outcome measures were visual acuity, signs of inflammation (scleritis and episcleritis), progression to corneal thinning, central keratolysis and ocular perforation.

Results:   We studied 27 patients/35 affected eyes (7 men/20 women) with a mean age of 57.2±16.3 years. PUK was primary (n=1) and in the 26 remaining cases, the underlying diseases were Rheumatoid Arthritis (RA) (n=19), Psoriasic Arthritis (2), RA+Felty syndrome+common variable immunodeficiency (1), Behçet Disease (1), Type I diabetes mellitus (1), granulomatous polyangiitis (1) and microscopic polyangiitis (1). They received the following topical therapy: corticosteroids (n=18), antibiotics (17), lubricants (18), autologous serum (11), topical cyclosporin (11) and topical tacrolimus 0.03% (1). Besides oral corticosteroids and before the biologic therapy they had received iv methylprednisolone (n=8), methotrexate (16), oral doxycycline (9), azathioprine (3) and ascorbic acid (2). Moreover, 10 patients required surgery: amniotic membrane (n=7), penetrating keratoplasty (4), conjunctival resection (3), tissue adhesives (2), conjunctival flap (1) and lamellar keratoplasty (1). Anti-TNFα drugs were the most common biologic agents (n=19): Adalimumab (ADA) (10; 37%), Infliximab (IFX) (; 29.6%) and etanercept (n=1; 3.7%). In the remaining 8 cases the biologic agents were rituximab (n=7; 25.9%) and tocilizumab (n=1; 3.7%). The main outcome measures are summarized in the Table. After a mean follow-up of 23.7±20 months, all objective outcomes had improved with a reduction of the median prednisone dose from 33.7 [IQR 17.5-52.5] mg at baseline to 0 [0-2.5] mg (p=0.028). The main observed adverse effects were supraventricular tachycardia (n=1) and pulmonary Tuberculosis (n=1).

Conclusion:  In our series, biological therapy, especially IFX and ADA, is effective and relatively safe in patients with PUK refractory to standard systemic treatment. TABLE

* p <0.05 compared with basal data# Data are expressed as % of the active eyes

ACR Meeting Abstracts - https://acrabstracts.org/abstract/biologic-therapy-in-severe-peripheral-ulcerative-keratitis-puk-multicenter-study-of-27-patients/