Janus Kinase Inhibitors in the Treatment of Refractory Cicatrizing Conjunctivitis in Pemphigoid.

Abstract

Objective: To evaluate the efficacy of Janus kinase inhibitor (JAKinib) therapy in managing cicatrizing conjunctivitis associated with ocular cicatricial pemphigoid (OCP) and mucous membrane pemphigoid with ocular involvement (ocMMP).

Methods: Retrospective chart review of patients with cicatrizing conjunctivitis secondary to OCP or ocMMP who underwent treatment with JAKinibs at a tertiary academic medical center from August 2015 to November 2024 for minimum follow-up of six months. Collected data included demographics, Foster stage of cicatrization, and treatment course.

Results: Thirty-two patients met inclusion criteria: 23 (71.9%) with OCP and 9 (28.1%) with ocMMP. 96.9% of patients demonstrated clinical improvement within twelve months of treatment initiation. Best response achieved were as follows: 1 (3.1%) no response, 17 (53.1%) partial response, 14 (43.8%) complete clinical remission, and 12 (37.5%) steroid-free remission. The mean time to partial response, complete clinical remission, steroid-free remission was 3.1 ± 1.8 (range, 0.9-8.3), 7.8 ± 3.3 months (range, 2.3-14.7 months), and 10.3 ± 7.4 months, (range, 2.3-31.4 months), respectively. Relapse in disease activity occurred in 8/32 (25.0%) of patients. Side effects occurred in 8/32 (25.0%) of patients. Four patients (12.5%) discontinued therapy due to severe adverse events, including transient ischemic attack, pulmonary embolism, pyelonephritis, and cholecystitis. There was a significant association between lower Foster cicatrization stages and achieving remission (U = 630.0, p = 0.0036), with a rank-biserial correlation of 0.72.

Conclusions: JAK inhibitor therapy demonstrates efficacy in the management of recalcitrant cicatrizing conjunctivitis associated with pemphigoid. These findings highlight JAK inhibitors as a promising therapeutic option for refractory cases.