ABSTRACT
ABSTRACT Ocular cicatricial pemphigoid (OCP) is a chronic, immune-mediated, bullous, cicatricial disease within the spectrum of mucocutaneous membranous pemphigoids (MMP). Although the diagnosis is often ophthalmological, due to the autoimmune nature of the pathology, it requires a joint approach with rheumatologists and immunologists. The objective of this narrative review was to explore the evidence available in the literature from 2000 to 2020 with respect to clinical manifestations, diagnosis, and treatment. The clinical presentation varies widely, from mild cases with slow progression of years of progression, to severe cases with a torpid and rapidly progressive evolution to fibrosis, refractory to multiple treatments. A com plete evaluation of the patient will help guide the diagnosis. The gold standard for diagnosis is conjunctival biopsy with direct immunofluorescence, although on occasions it can be reached if the symptoms are characteristic. Treatment is local and systemic according to its severity and evolution. The evidence on topical and systemic therapeutics is obtained mainly from uncontrolled observational and experimental studies. Immunomodulatory therapy has made it possible to preserve vision and, in many cases, prevent sequelae. The evolu tion is linked to the early diagnosis and immunosuppressive treatment, so it is essential to be aware of this disease, the diagnostic methods, as well as the immunomodulating and immunosuppressive therapies available.
RESUMEN El penfigoide ocular cicatrizal (POC) es una enfermedad crónica, inmunomediada ampollar, mucosinequiante, comprendida dentro del espectro de penfigoides membranosos mucocutáneos (PMM). El diagnóstico es, con frecuencia, oftalmológico, pero debido al carácter autoinmune de la patología, requiere el abordaje en conjunto con reumatólogos e inmunólogos. El objetivo de esta revisión narrativa fue explorar la evidencia disponible en la literatura, desde el año 2000 hasta el 2020, en lo que respecta a sus manifestaciones clínicas, diagnóstico y tratamiento. La presentación clínica varía ampliamente, desde casos leves con progresión lenta de años de evolución hasta casos severos con evolución tórpida y rápidamente progresiva a la fibrosis, refractarios a múltiples tratamientos. Una evaluación completa del paciente ayudará a guiar el diagnóstico. El estándar de oro diagnóstico es la biopsia conjuntival con inmunofluorescencia directa, si bien en ocasiones puede diagnosticarse por la clínica característica. El tratamiento es local y sistêmico de acuerdo con su severidad y evolución. En los últimos 20 anos, la evidencia sobre los tratamientos tópicos y sistêmicos corresponde en su mayoría a estudios observacionales y experimentales no controlados. Los métodos de tratamiento inmunomoduladores han permitido preservar la visión y, en muchos casos, prevenir secuelas. La evolución está ligada al diagnóstico temprano y a los tratamientos disponibles, por lo que es fundamental el conocimiento de esta patología, los métodos diagnósticos y los tratamientos inmunomoduladores e inmunosupresores.
Subject(s)
Male , Female , Middle Aged , Dry Eye Syndromes , Pemphigoid, Benign Mucous Membrane , Conjunctival Diseases , Eye DiseasesABSTRACT
@#Ocular cicatricial pemphigoid(OCP)is a special manifestation of mucosal pemphigoid(mucous membrane pemphigoid, MMP), and the pathogenesis is not clear at present. It can be caused by variety factors such as antigen-antibody reaction, inflammation cell infiltration, the action of various cytokines, elevated calcium ion levels and susceptibility genes. In the early period of diseases, conjunctival present chronic progressive fibrotic inflammation, later the corneal opacity and the neovascularization will eventually lose vision. Therefore, it is particularly important to carry out clinical standardized treatment for OCP patients in a timely manner. To use medicine to control the inflammation and delay the progression of the disease, for example, dapsone, intravenous immunoglobulin(IVIG), rituximab(RTX), tumor necrosis factor antagonists and adrenocorticoids. Surgical treatment can be considered appropriately when OCP patients are complicated by severe trichiasis, corneal disease and cataract.
ABSTRACT
El penfigoide cicatrizal ocular (PCO) es una enfermedad ampollar autoinmune que produce daño conjuntival grave. Se conoce poco acerca de la respuesta del PCO al tratamiento inmunosupresor. Describimos un grupo de 76 pacientes con PCO, 62 mujeres y 14 hombres. La edad media al diagnóstico fue de 67 ± 14 años, con un retraso de 7.5 ± 10 años. Sesenta se siguieron en nuestro servicio por 19 ± 21 meses. De 51 en quienes se describe la gravedad de la enfermedad al inicio del tratamiento, fue leve en 19 pacientes, moderada en 19, grave en cinco y muy grave en ocho. Las drogas mayormente prescriptas fueron dapsona en 35 pacientes, de los que 23 la discontinuaron por efectos adversos, y metotrexate en 42, de los que nueve lo suspendieron. Otros recibieron azatioprina, ciclofosfamida y ciclosporina. A 17 se les indicaron corticoides orales, además del inmunosupresor. Cuatro combinaron dos drogas para controlar la enfermedad. Tres pacientes refractarios recibieron gammaglobulina EV con buena respuesta. De 48 evaluados, 39 mostraron mejoría, ocho no tuvieron cambios y uno progresó. En nuestra experiencia, metotrexate y azatioprina son efectivos, con baja toxicidad. Dapsona es útil en casos leves, con efectos adversos frecuentes. La gammaglobulina EV fue efectiva en casos refractarios.
Ocular cicatricial pemphigoid (OCP) is a blistering autoimmune disease that can produce severe conjunctival damage. Its response to immunosuppressive treatment is poorly known. We describe a group of 76 patients, 62 women and 14 men. Mean age at diagnosis was 67±14 years old, with a delay to diagnosis of 7.5±10 years. Sixty patients continued their follow up in our services for 19±21 months. Nineteen out of 51 had mild disease, 19 moderate, 5 severe and 8 very severe at onset of treatment. The more frequently prescribed drugs were dapsone, in 35 (23 discontinued it because of adverse effects), and methotrexate in 42 patients, nine of them stopped it. Other patients received azathioprine, cyclophosphamide and ciclosporine. Seventeen received oral steroids in addition to immunosuppresive drugs. Four patients combined two immunosupressive drugs to control their disease. In three refractory cases IV immunoglobulin (Ig) was administered with good response. From 48 evaluated patients, 39 improved with treatment, eight remained stable and one progressed. In our experience, methotrexate and azathioprine were effective drugs, with low toxicity. Dapsone was useful in mild cases, with frequent adverse effects. IVIg was effective for refractory cases.
Subject(s)
Adult , Aged , Aged, 80 and over , Female , Humans , Male , Middle Aged , Autoimmune Diseases/drug therapy , Immunosuppressive Agents/therapeutic use , Pemphigoid, Benign Mucous Membrane/drug therapy , Azathioprine/therapeutic use , Delayed Diagnosis , Dapsone/therapeutic use , Follow-Up Studies , Immunoglobulins, Intravenous/therapeutic use , Immunosuppressive Agents/adverse effects , Methotrexate/therapeutic use , Pemphigoid, Benign Mucous Membrane/pathology , Severity of Illness Index , Treatment OutcomeABSTRACT
Objective To observe the outcome of patients with ocular cicatricial pemphigoid (OCP) after amniotic membrane grafting or penetrating keratoplasty.Design Retrospective,noncomparative case series.Participants 3 consecutive OCP patients (4 eyes),were included.Methods For 3 patients (4 eyes) in this study,preoperative visual acuity was from HM/5 cm to HM/10 cm.The symblepharon (gradeⅢ) of 2 patients (3 eyes) were detached and amniotic membrane was transplanted,and bandage contact lens were used till 2 months after operations.The other patient (1 eye) was undergone penetrating keratoplasty with glycerol-cryopreserved cornea because of corneal ulcer and perforation.Main Outcome Measures Visual acuity,recovering of conjunctiva and cornea.Results Am- niotic membrane dissolved about 1 month after operation in 2 patients (3 eyes).Symblepharon changed from gradeⅢto gradeⅡ,visual acuity increased to 0.04-0.05,central corneal epithelium was regenerated significantly,and a little new vessel appeared at corneal lim- bus in 2 patients (2 eyes).The graft deliquesced in the patient with penetrating keratoplasty at 20 days after operation and undergone penetrating keratoplasty again after 1 month.In this patient,the graft molten induced the ocular contents run-off and became eyeball at- rophy eventually.Conclusion Our small sample study shows that the symblepharon detachment and amniotic membrane transplantation is effective for improving visual acuity in OCP with ocular surface dysfunction.However,because of various factors,penetrating kerato- plasty is ineffective for OCP with perforating corneal ulcer.
ABSTRACT
Ocular cicatricial pemphigoid isa chronic, progressive disease characterized by cicatricial shrinkage of the conjunctiva, entropion, trichiasis, xerosis, and finally reduced vision from corneal opacification. It is a relatively rare, autoimmune disease. In the early stages, it can be easily overlooked as simple chronic conjunctivitis, so it can be untreated. We report a case of ocular cicatricial pemphigoid that resulted in significant morbidity. We were able to control the progression of cicatrization with aggressive immunosuppressive therapy.