Ocular Cicatricial Pemphigoid With IgM-Positive Biopsy

Area of Science:

• Ophthalmology
• Immunodermatology
• Autoimmune Diseases

Background:

• Ocular cicatricial pemphigoid (OCP) is a chronic autoimmune blistering disease affecting the conjunctiva.
• The role and prevalence of specific immunoglobulin M (IgM) deposition along the basement membrane zone (BMZ) in OCP are not fully understood.

Purpose of the Study:

• To determine the prevalence of linear IgM deposition along the BMZ in patients diagnosed with OCP.
• To evaluate the clinical outcomes of OCP patients with IgM deposition who undergo immunomodulatory therapy (IMT).

Main Methods:

• Retrospective chart review of patients with conjunctival biopsy-proven OCP.
• Analysis of clinical data, including direct immunofluorescence or immunohistochemistry for linear IgM deposition along the BMZ.
• Assessment of response to IMT.

Main Results:

• Out of 817 patients, 93 (11.4%) showed linear IgM deposition along the BMZ in their conjunctival biopsies.
• Forty-six patients with sufficient follow-up were analyzed; 35 (76.1%) achieved durable remission with IMT.
• A majority (82.9%) of responders achieved remission with first-line antimetabolite therapy.

Conclusions:

• IgM positivity along the BMZ is present in a minority of OCP patients.
• OCP patients with IgM deposition demonstrate comparable treatment outcomes to the general OCP patient population.
• First-line antimetabolite therapy appears effective for achieving durable remission in this subgroup.