Das okuläre vernarbende Pemphigoid – aktuelle Aspekte zu Klinik und Therapie einer problematischen Erkrankung

Zusammenfassung

Das okuläre Schleimhautpemphigoid (OCP = ocular cicatricial pemphigoid) gehört zu einer Familie von seltenen, chronisch-fortschreitenden Autoimmunerkrankungen, die vorwiegend die Schleimhäute befallen. Es ist eine immunpathologisch heterogene Gruppe mit unterschiedlichen Phänotypen, deren gemeinsames klinisches Korrelat eine subepidermale Blasenbildung durch Störung der Adhäsion zwischen Epidermis und Dermis ist. Entscheidend ist, dass es aufgrund einer chronischen Entzündungsreaktion mit konsekutiver Fibrose zum Funktionsverlust der betroffenen Organe kommt. Die chronisch vernarbende Konjunktivitis stellt eine häufige Manifestation des Schleimhautpemphigoids dar. Die Deutung der subtilen Symptomatik und zügige Einleitung einer stadiengerechten Therapie ist dabei von höchster Wichtigkeit. Ziel ist die Vermeidung visusbedrohender Komplikationen durch eine korneale Beteiligung und u. U. lebensbedrohender Komplikationen durch ösophageale oder tracheale Strikturen im Rahmen einer extraokulären Beteiligung. Bislang liegen keine prospektiven, randomisierten Studien vor, die eine Therapieempfehlung über dem Evidenzgrad III ableiten ließen. Aufgrund der Autoimmunpathogenese und klinischer Erfahrung wird die systemische Immunsuppression, vor allem bei fortgeschrittenen Stadien, als einzige wirkungsvolle Behandlungsmöglichkeit angesehen.

Ziel dieser Übersichtsarbeit ist es, einen Leitfaden für die stadiengerechte Therapie zu bieten. Des Weiteren soll ein Ausblick auf zukünftige Alternativen, insbesondere für schwere und therapieresistente Verlaufsformen gegeben werden.

Abstract

The ocular cicatricial pemphigoid (OCP) belongs to a family of chronically progressive autoimmune disorders, predominantly affecting mucous membranes (mucous membrane pemphigoids). It is an immunopathologically heterogeneous group of disorders with variable phenotypes that share the unique feature of a subepidermal blistering, through disruption of the adhesion between epidermis and dermis. A key feature is the chronically active inflammation with consecutive fibrosis, leading to a partial or complete loss of function of the affected organ. The ocular disease as a chronic cicatrising conjunctivitis is a common manifestation of the mucous membrane pemphigoid. The identification of the subtle pathology and the prompt initiation of an appropriate therapy are of pivotal importance. One purpose is to prevent further vision loss due to extensive corneal scarring and life-threatening systemic complications, such as the formation of oesophageal or tracheal strictures. So far there are no prospective, randomised studies, regarding the therapy guidelines with an evidence level more than III. The autoimmune nature of the disease implies that systemic immunosuppression is the only effective treatment option, most notably in extended stages.

The aim of our study is to give a guideline for a stage adjusted therapy with conventional immunosuppressants and to give a perspective for alternative therapies, especially for recalcitrant disease.

Schlüsselwörter

Keratopathie - okuläres Pemphigoid - Symblephara - Therapie

Key words

keratopathy - ocular cicatricial pemphigoid - symblephara - therapy

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