On the Current Care Situation and Treatment of Ocular Mucous Membrane Pemphigoid in Germany

TY - JOUR

T1 - On the Current Care Situation and Treatment of Ocular Mucous Membrane Pemphigoid in Germany

AU - Yaïci, Rémi

AU - Roth, Mathias

AU - Juergens, Lukas

AU - Nawaiseh, Sami Al

AU - Burkhard, Dick

AU - Besgen, Volker

AU - Fuest, Matthias

AU - Girbardt, Christian

AU - Hampel, Ulrike

AU - Heichel, Jens

AU - Heiligenhaus, Arnd

AU - Herwig-Carl, Martina C

AU - Kakkassery, Vinodh

AU - Kontopoulou, Kalliopi

AU - Löffler, Karin U

AU - Maier, Philip Christian

AU - Nölle, Bernhard

AU - Pach, Johanna

AU - Paul, Sebastian

AU - Pleyer, Uwe

AU - Pöllmann, Michael

AU - Saeger, Mark

AU - Schmidt, Enno

AU - Siebelmann, Sebastian

AU - Sokolenko, Ekatarina

AU - Strudel, Lisa

AU - Stübiger, Nicole

AU - Tarhan, Melih

AU - Theuersbacher, Johanna

AU - van Oterendorp, Christian

AU - Walker, Marten

AU - Wiecha, Carolin

AU - Wykrota, Agata Anna

AU - Geerling, Gerd

N1 - Thieme. All rights reserved.

PY - 2023/9

Y1 - 2023/9

N2 - BACKGROUND: Ocular involvement in mucous membrane 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.

AB - BACKGROUND: Ocular involvement in mucous membrane 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.

U2 - 10.1055/a-1720-1819

DO - 10.1055/a-1720-1819

M3 - SCORING: Journal article

C2 - 35609814

VL - 240

SP - 1077

EP - 1083

JO - KLIN MONATSBL AUGENH

JF - KLIN MONATSBL AUGENH

SN - 0023-2165

IS - 9

ER -