Subject(s)
Humans , Male , Female , Pemphigoid, Benign Mucous Membrane/diagnosis , Rheumatologists , Ophthalmologists , RheumatologyABSTRACT
PURPOSE: To assess the prevalence of autoimmune diseases (ADs) associated with ocular cicatricial pemphigoid (OCP) and analyze clinical, laboratory, and treatment associations between these entities. METHODS: A multicentre cross-sectional study of patients with an OCP diagnosis. The population was divided into two groups according to their association with other ADs or not. Clinical, laboratory and treatment variables were described and compared between groups. A multivariable logistic regression analysis was performed to identify variables that could suggest the association between OCP and ADs. RESULTS: Eighty-eight patients were recruited, with a mean age at diagnosis of 64.3 years (SD 11.9). Biopsy was performed in 86.8% of the patients. There was a median delay of 2 years from the onset of symptoms to diagnosis. Extraocular involvement was evidenced in 11.5%. The group associated with ADs included 24 patients (27.3%). The most prevalent diagnosis was Sjögren´s syndrome. Hypergammaglobulinemia was associated with ADs and OCP, adjusted for age, sex, smoking, skin and mucosal involvement, and erythrocyte sedimentation rate (OR 8.7; 95%CI 1.6-46.8; p = 0.012). CONCLUSIONS: Due to OCP's autoimmune nature, it could coexist with other ADs. This study observed that more than a quarter of the population presented with this association, and hypergammaglobulinemia could suggest it.
Subject(s)
Autoimmune Diseases , Pemphigoid, Benign Mucous Membrane , Sjogren's Syndrome , Humans , Middle Aged , Pemphigoid, Benign Mucous Membrane/complications , Pemphigoid, Benign Mucous Membrane/diagnosis , Cross-Sectional Studies , Hypergammaglobulinemia , Autoimmune Diseases/complications , Autoimmune Diseases/diagnosis , Autoimmune Diseases/epidemiologyABSTRACT
Objetivo: Analizar los datos clínicos/epidemiológicos, el manejo y los tratamientos instaurados a pacientes con penfigoide ocular cicatricial en un Servicio de Reumatología. Material y métodos: Revisión de historias clínicas de pacientes con penfigoide ocular cicatricial derivados por oftalmología entre 2008 y 2019. Resultados: En nuestra serie de 27 pacientes, el 67% fue diagnosticado y derivado en los últimos 2 años. La mayoría en estadio 1 de Foster. El 18,5% presentó síndrome de Sjogren asociado, conllevando una evolución desfavorable. El 88,8% recibió metotrexato, el 74% lo utilizó en monoterapia y el 66% continuó el tratamiento hasta el final. El 18,5% debió combinar/rotar terapias, los fármacos utilizados fueron micofenolato, azatioprina, ciclofosfamida, sirolimus, rituximab. El 29% utilizó esteroides. Solo en 2 ojos progresó el estadio. No se registraron eventos adversos serios y el 37% presentó eventos adversos leves. Conclusión: El inicio temprano de la terapia inmunosupresora es fundamental, siendo el metotrexato una buena alternativa como tratamiento de inicio según nuestra experiencia, debiendo escalar el tratamiento de acuerdo a la evolución.(AU)
Objective: To analyse the clinical / epidemiological data, the management and the treatments established in patients with ocular cicatricial pemphigoid in a Rheumatology Service. Material and methods: Review of clinical histories of patients with ocular cicatricial pemphigoid referred by ophthalmology between 2008 and 2019. Results: In our series of 27 patients, 67% were diagnosed and referred in the last 2 years. Most in Foster stage 1. Of the patients, 18.5% presented associated Sjogren's syndrome, with poor progression: 88.8% received Methotrexate, 74% used it as monotherapy and 66% continued with said treatment up to the end. Eighteen point five percent had to combine or rotate therapies, the drugs used were mycophenolate, azathioprine, cyclophosphamide, sirolimus, etanercept, rituximab: 29% used steroids. Only in 2 eyes was stage progression observed. There were no serious adverse events, and 37% had mild adverse events. Conclusion: Starting immunosuppressive therapy early is essential, methotrexate being a good initial alternative in our experience, and treatment must be escalated in line with disease progression.(AU)
Subject(s)
Humans , Male , Female , Pemphigoid, Benign Mucous Membrane , Methotrexate/therapeutic use , Sjogren's Syndrome , Drug Therapy, Combination , Immunosuppression Therapy , Pemphigoid, Benign Mucous Membrane/diagnosis , Pemphigoid, Benign Mucous Membrane/drug therapy , Rheumatology , Immunosuppressive Agents/adverse effectsABSTRACT
OBJECTIVE: To analyse the clinical/epidemiological data, the management and the treatments established in patients with Ocular Cicatricial Pemphigoid (OCP) in a Rheumatology Service. MATERIAL AND METHODS: Review of clinical histories of patients with OCP referred by ophthalmology between 2008 and 2019. RESULTS: In our series of 27 patients, 67% were diagnosed and referred in the last 2 years. Most in Foster stage 1. Of the patients, 18.5% presented associated Sjogren's syndrome, with poor progression: 88.8% received Methotrexate, 74% used it as monotherapy and 66% continued with said treatment up to the end. Eighteen point five percent had to combine or rotate therapies, the drugs used were mycophenolate, azathioprine, cyclophosphamide, sirolimus, etanercept, rituximab: 29% used steroids. Only in 2 eyes was stage progression observed. There were no serious adverse events, and 37% had mild adverse events. CONCLUSION: Starting immunosuppressive therapy early is essential, methotrexate being a good initial alternative in our experience, and treatment must be escalated in line with disease progression.
Subject(s)
Methotrexate , Pemphigoid, Benign Mucous Membrane , Azathioprine , Cyclophosphamide/adverse effects , Humans , Immunosuppressive Agents/adverse effects , Methotrexate/adverse effects , Pemphigoid, Benign Mucous Membrane/diagnosis , Pemphigoid, Benign Mucous Membrane/drug therapyABSTRACT
OBJECTIVE: To analyse the clinical / epidemiological data, the management and the treatments established in patients with ocular cicatricial pemphigoid in a Rheumatology Service. MATERIAL AND METHODS: Review of clinical histories of patients with ocular cicatricial pemphigoid referred by ophthalmology between 2008 and 2019. RESULTS: In our series of 27 patients, 67% were diagnosed and referred in the last 2 years. Most in Foster stage 1. Of the patients, 18.5% presented associated Sjogren's syndrome, with poor progression: 88.8% received Methotrexate, 74% used it as monotherapy and 66% continued with said treatment up to the end. Eighteen point five percent had to combine or rotate therapies, the drugs used were mycophenolate, azathioprine, cyclophosphamide, sirolimus, etanercept, rituximab: 29% used steroids. Only in 2 eyes was stage progression observed. There were no serious adverse events, and 37% had mild adverse events. CONCLUSION: Starting immunosuppressive therapy early is essential, methotrexate being a good initial alternative in our experience, and treatment must be escalated in line with disease progression.
