Janus kinase inhibitors in the treatment of refractory cicatrizing conjunctivitis in pemphigoid

Objective

To evaluate the efficacy of Janus kinase inhibitor (JAKi) 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 JAKi 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 remission, and 12 (37.5 %) steroid-free complete remission. The mean time to partial response, complete remission, steroid-free complete 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.