Characteristics Associated With Refractory Course, Blindness, and Treatment Strategy-Related Outcomes in Patients...

Area of Science:

• Autoimmune bullous diseases
• Ophthalmology
• Dermatology

Background:

• Mucous membrane pemphigoid (MMP) is a rare autoimmune blistering disease primarily affecting mucous membranes.
• Predictors of disease course and complications like blindness in MMP remain incompletely understood.

Purpose of the Study:

• To identify characteristics associated with a refractory disease course in MMP.
• To evaluate factors linked to blindness development in MMP patients.
• To assess the impact of different treatment strategies on MMP prognosis.

Main Methods:

• Retrospective cohort study of 121 MMP patients from 2007-2020.
• Multivariable logistic regression used to analyze associations with refractory disease and blindness.
• Data analysis conducted from January 2009 to June 2020.

Main Results:

• Refractory disease was associated with anti-LAD-1 IgA and anti-dermal-epidermal/epithelial junction (DEJ) IgG.
• Blindness correlated with older age (≥68 years), initial bilateral ocular involvement, and scarring ocular lesions.
• Intravenous immunoglobulin (IVIG) use was linked to both blindness and refractory disease.

Conclusions:

• Patients with MMP and anti-LAD-1 IgA or anti-DEJ IgG require close monitoring.
• While bilateral and scarring ocular lesions predict blindness, unilateral and non-scarring cases can also lead to severe vision loss.
• Understanding these associations can guide proactive management and monitoring strategies for MMP patients.