DISEASE ENDOTYPES PREDICT SEVERITY OF MUCOUS MEMBRAND PEMPHIGOID.

Mucous membrane pemphigoid (MMP) is an autoimmune disease with variable clinical presentation and multiple autoantigens. To determine if disease endotypes could be identified based on the pattern of serum reactivity, the clinical and diagnostic information of 70 MMP patients was collected, and reactivity to dermal or epidermal antigens, using indirect immunofluorescence (IIF), and specific reactivity to BP180, BP230, collagen VII and laminin 332 was evaluated. Most patients had lesions at multiple mucosae with the most prevalent being the oropharyngeal (mouth, gingiva, pharynx, 98.6%), followed by ocular (38.6%), nasal (32.9%), genital or anal (31.4%), laryngeal (20%), and esophageal (2.9%) sites, and skin (45.7%). Autoantigen profiling identified BP180 (71%) as the most common autoantigen, followed by laminin 332 (21.7%), collagen VII (13%), and BP230 IgG (11.6%). Reactivity to dermal antigens predicted a more severe disease characterized by a higher number of total sites involved, especially high-risk sites, and a decreased response to rituximab (RTX). In most cases, identification of dermal IIF reactivity is an accurate predictor of disease course; however, confirmation of laminin 332 reactivity is important with dermal IIF positivity due to an increased risk of solid tumors. Additionally, the ocular mucosae should be monitored in patients with IgA on DIF.