{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,29]],"date-time":"2026-10-29T02:52:24Z","timestamp":1793242344806,"version":"4.5.0"},"reference-count":11,"publisher":"American Medical Association (AMA)","issue":"2","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JAMA Dermatol"],"published-print":{"date-parts":[[2023,2,1]]},"abstract":"<jats:sec id=\"ab-dbr220024-4\"><jats:title>Importance<\/jats:title><jats:p>Mucous membrane pemphigoid (MMP) is a rare and heterogeneous subepithelial autoimmune bullous disease with predominant mucosal involvement. Characteristics associated with the disease course and complications are yet to be delineated.<\/jats:p><\/jats:sec><jats:sec id=\"ab-dbr220024-5\"><jats:title>Objectives<\/jats:title><jats:p>To evaluate characteristics associated with refractory disease course and blindness among patients with MMP and to estimate the association of different treatment strategies with the prognostic outcome.<\/jats:p><\/jats:sec><jats:sec id=\"ab-dbr220024-6\"><jats:title>Design, Setting, and Participants<\/jats:title><jats:p>A retrospective cohort study of consecutive patients diagnosed with MMP and followed up for more than 1 year from 2007 to 2020 in 2 tertiary referral centers. Data were analyzed from January 1, 2009, to June 30, 2020.<\/jats:p><\/jats:sec><jats:sec id=\"ab-dbr220024-7\"><jats:title>Main Outcomes and Measures<\/jats:title><jats:p>Characteristics associated with refractory disease course and blindness were evaluated using multivariable logistic regression model.<\/jats:p><\/jats:sec><jats:sec id=\"ab-dbr220024-8\"><jats:title>Results<\/jats:title><jats:p>The study encompassed 121 patients with MMP (mean [SD] age, 66.0 [14.0] years; 78 (64.5%) were women), of whom 56 (46.3%) followed a refractory course and 13 (10.7%) developed blindness. Anti\u2013LAD-1 IgA (odds ratio [OR], 3.42; 95% CI, 1.11-10.52; <jats:italic>P<\/jats:italic>\u2009=\u2009.03) and anti\u2013dermal-epidermal\/epithelial junction (DEJ) IgG (by indirect immunofluorescence on human salt-split skin; OR, 2.92; 95% CI, 1.26-6.78; <jats:italic>P<\/jats:italic>\u2009=\u2009.01) were significantly associated with refractory course. Development of blindness was associated with older age (\u226568 years; OR, 6.38; 95% CI, 1.35-30.16; <jats:italic>P<\/jats:italic>\u2009=\u2009.009), initial presentation with bilateral ocular involvement (OR, 7.92; 95% CI, 2.04-30.68; <jats:italic>P<\/jats:italic>\u2009=\u2009.001), and scarring ocular lesions (OR, 5.11; 95% CI, 1.47-17.79; <jats:italic>P<\/jats:italic>\u2009=\u2009.006). However, 4 (30.8%) and 2 (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 3 or more immunosuppressive\/immunomodulatory drugs (OR, 4.07; 95% CI, 1.17-14.14; <jats:italic>P<\/jats:italic>\u2009=\u2009.02) and by cyclophosphamide (OR, 7.64; 95% CI, 2.24-26.09; <jats:italic>P<\/jats:italic>\u2009&amp;amp;lt;\u2009.001). Patients developing blindness and refractory course were more frequently managed by intravenous immunoglobulin (OR, 7.64; 95% CI, 2.24-26.09; <jats:italic>P<\/jats:italic>\u2009&amp;amp;lt;\u2009.001 and OR, 3.47; 95% CI, 1.42-8.45; <jats:italic>P<\/jats:italic>\u2009=\u2009.005, respectively).<\/jats:p><\/jats:sec><jats:sec id=\"ab-dbr220024-9\"><jats:title>Conclusions and Relevance<\/jats:title><jats:p>Findings of this cohort study support that patients with MMP with anti\u2013LAD-1 IgA and anti-DEJ IgG reactivity should be carefully monitored. While initial bilateral ocular disease and scarring ocular lesions were associated with blindness, patients initially presenting with unilateral and nonscarring ocular disease may still develop severe vision impairment.<\/jats:p><\/jats:sec>","DOI":"10.1001\/jamadermatol.2022.5829","type":"journal-article","created":{"date-parts":[[2023,1,11]],"date-time":"2023-01-11T16:04:00Z","timestamp":1673453040000},"page":"198","source":"Crossref","is-referenced-by-count":10,"title":["Characteristics Associated With Refractory Course, Blindness, and Treatment Strategy\u2013Related Outcomes in Patients With Mucous Membrane Pemphigoid"],"prefix":"10.1001","volume":"159","author":[{"given":"Khalaf","family":"Kridin","sequence":"first","affiliation":[{"name":"L\u00fcbeck Institute of Experimental Dermatology (LIED), University of L\u00fcbeck, L\u00fcbeck, Germany"},{"name":"Azrieli Faculty of Medicine, Bar-Ilan University, Safed, Israel"},{"name":"Unit of Dermatology and Skin Research Laboratory, Baruch Padeh Medical Center, Poriya, Israel"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nina","family":"van Beek","sequence":"additional","affiliation":[{"name":"Department of Dermatology, University of L\u00fcbeck, L\u00fcbeck, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elena","family":"B\u00fchler","sequence":"additional","affiliation":[{"name":"Department of Dermatology, University of L\u00fcbeck, L\u00fcbeck, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anne S.","family":"Kochan","sequence":"additional","affiliation":[{"name":"Department of Dermatology, Carl Gustav Carus University Hospital, TU Dresden, Dresden, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mahdy","family":"Ranjbar","sequence":"additional","affiliation":[{"name":"Department of Ophthalmology, University of L\u00fcbeck, L\u00fcbeck, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stefan","family":"Beissert","sequence":"additional","affiliation":[{"name":"Department of Dermatology, Carl Gustav Carus University Hospital, TU Dresden, Dresden, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Detlef","family":"Zillikens","sequence":"additional","affiliation":[{"name":"Department of Dermatology, University of L\u00fcbeck, L\u00fcbeck, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Claudia","family":"G\u00fcnther","sequence":"additional","affiliation":[{"name":"Department of Dermatology, Carl Gustav Carus University Hospital, TU Dresden, Dresden, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Enno","family":"Schmidt","sequence":"additional","affiliation":[{"name":"L\u00fcbeck Institute of Experimental Dermatology (LIED), University of L\u00fcbeck, L\u00fcbeck, Germany"},{"name":"Department of Dermatology, University of 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