Zur Versorgungssituation und konservativen Therapie des okulären vernarbenden Schleimhautpemphigoids in Deutschland

Translated title of the contribution: On the Current Care Situation and Treatment of Ocular Mucous Membrane Pemphigoid in Germany

Rémi Yaïci*, Mathias Roth, Lukas Juergens, Sami Al Nawaiseh, Dick Burkhard, Volker Besgen, Matthias Fuest, Christian Girbardt, Ulrike Hampel, Jens Heichel, Arnd Heiligenhaus, Martina C. Herwig-Carl, Vinodh Kakkassery, Kalliopi Kontopoulou, Karin U. Löffler, Philip Christian Maier, Bernhard Nölle, Johanna Pach, Sebastian Paul, Uwe PleyerMichael Pöllmann, Mark Saeger, Enno Schmidt, Sebastian Siebelmann, Ekatarina Sokolenko, Lisa Strudel, Nicole Stübiger, Melih Tarhan, Johanna Theuersbacher, Christian Van Oterendorp, Marten Walker, Carolin Wiecha, Agata Anna Wykrota, Gerd Geerling

*Corresponding author for this work
5 Citations (Scopus)

Abstract

Background Ocular involvement in mucousmembrane pemphigoid (MMP) is relatively rare, with a prevalence of 25 cases per million population, equating to approx. 2,100 patients throughout Germany. Diagnosis can be difficult - especially in cases of isolated ocular involvement - and treatment can be complex and lengthy. Immunosuppressants or immunomodulatory drugs are often used. Due to the complexity of diagnosis and treatment, MMP patients are usually referred to specialized centers. The aim of this project was to evaluate the current care situation of patients with ocular MMP in Germany. Methods A paper-based survey was designed and sent to all university eye clinics and other specialized centers in Germany in April 2020. The survey asked about the existence of a specialized outpatient service, the total annual number of patients with MMP, the annual number of newly diagnosed patients, any interdisciplinary collaboration for diagnostic or therapeutic purposes, as well as the local and systemic therapy used. Results Of a total of 44 clinics, 28 (64%) responded, reporting a total average of 27 ± 42 (0-200) patients and 3.6 ± 2.2 (0-10) new cases per year. This corresponds to a total of 741 patients. Only nine (32%) of the responding clinics offer specialized MMP clinics. 93% of the centers collaborate with the local dermatology department. 79% perform serological and histological diagnostics in-house. About half of the centers (n = 16) apply a standardized treatment regime. Systemic glucocorticoids (66.7%) are most commonly used, followed by mycophenolate mofetil and dapsone (57.1%), rituximab (33.3%), azathioprine and cyclophosphamide (28.6%), as well as methotrexate (19.0%). The least frequently used treatment is intravenous immunoglobulin (14.3%). Conclusion This survey of German ophthalmology departments obtained data from about one third of the estimated total cohort of all patients with MMP in Germany. These are presumed to be exclusively patients with at least one ocular involvement. The complex care of these patients is usually provided in collaboration with a dermatologist and with the use of systemic anti-inflammatory medication. Currently, an ophthalmological MMP register is being established to better record the epidemiology and care situation of this rare disease in Germany and to improve it in the long term.

Translated title of the contributionOn the Current Care Situation and Treatment of Ocular Mucous Membrane Pemphigoid in Germany
Original languageGerman
JournalKlinische Monatsblatter fur Augenheilkunde
Volume240
Issue number9
Pages (from-to)1077-1083
Number of pages7
ISSN0023-2165
DOIs
Publication statusPublished - 30.09.2021

Research Areas and Centers

  • Academic Focus: Center for Infection and Inflammation Research (ZIEL)
  • Centers: Center for Research on Inflammation of the Skin (CRIS)

DFG Research Classification Scheme

  • 204-05 Immunology
  • 205-19 Dermatology
  • 206-11 Ophthalmology

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