Germany: In patients with mucous membrane pemphigoid (MMP), dermatologists should be aware of the risk factors and treatment strategies for the development of blindness, researchers suggest in a recent study published in JAMA Dermatology.
"Patients with MMP with anti-LAD-1 IgA and anti-DEJ IgG reactivity are more susceptible to the refractory course; given this, they should be monitored closely," the researchers wrote. "Severe vision impairment may still occur in patients initially presenting with nonscarring and unilateral ocular disease."
Mucous membrane pemphigoid is a heterogeneous and rare subepithelial autoimmune bullous disease with principal mucosal involvement. Characteristics associated with the disease course and complications are yet to be described. Considering this, Khalaf Kridin from the University of Lübeck, Lübeck, Germany, and colleagues aimed to assess characteristics associated with refractory disease course and blindness in MMP patients and to determine the association of different treatment strategies with the prognostic outcome.
For this purpose, the team conducted a retrospective cohort study of consecutive patients with MMP diagnoses. They were followed for more than one year, from 2007 to 2020, in 2 tertiary referral centres. Data analysis was done between 2009 and 2020.
The multivariable logistic regression model was used to evaluate characteristics associated with refractory disease course and blindness.
The study led to the following findings:
The study encompassed 121 patients with MMP (mean age, 66.0 years; 64.5% were women), 46.3% followed a refractory course and 10.7% developed blindness. Anti–LAD-1 IgA (odds ratio [OR], 3.42) and anti–dermal-epidermal/epithelial junction (DEJ) IgG (by indirect immunofluorescence on human salt-split skin; OR, 2.92) were significantly associated with a refractory course.
Development of blindness was associated with older age (≥68 years; OR, 6.38), initial presentation with bilateral ocular involvement (OR, 7.92), and scarring ocular lesions (OR, 5.11).
30.8% and 15.4% of those experiencing blindness had no ocular scarring lesions and unilateral ocular involvement at the onset of their disease, respectively.
Patients progressing to blindness were more likely to be treated by three or more immunosuppressive/immunomodulatory drugs (OR, 4.07) and cyclophosphamide (OR, 7.64).
Patients developing blindness and refractory course were more frequently managed by intravenous immunoglobulin (OR, 7.64 and OR, 3.47, respectively).
"Our findings support that patients with MMP with anti– LAD-1 IgA and anti-DEJ IgG reactivity should be monitored carefully," the researchers wrote. "Initial bilateral ocular disease and scarring lesions were linked with blindness; patients initially presenting with nonscarring and unilateral ocular disease may still develop severe vision impairment."
Reference:
Kridin K, van Beek N, Bühler E, et al. Characteristics Associated With Refractory Course, Blindness, and Treatment Strategy–Related Outcomes in Patients With Mucous Membrane Pemphigoid. JAMA Dermatol. Published online January 11, 2023. doi:10.1001/jamadermatol.2022.5829