TY - JOUR
T1 - Zur Versorgungssituation und konservativen Therapie des okulären vernarbenden Schleimhautpemphigoids in Deutschland
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 - Funding Information:
Martina Herwig-Carl: Research grant: EPIC-XS (project no. 823839, funded by the Horizon 2020 programme of the EU), German Research Foundation (HE5775/5-1); lectures and consultancy work: GlaxoSmithKline. I declare that the other authors have not declared any economic or personal connections as defined above in the past 3 years.
Funding Information:
Martina Herwig-Carl: Research grant: EPIC-XS (project no. 823839, funded by the Horizon 2020 programme of the EU), German Research Foundation (HE5775/5-1); lectures and consultancy work: GlaxoSmith-Kline. I declare that the other authors have not declared any economic or personal connections as defined above in the past 3 years.
Publisher Copyright:
© 2021 Georg Thieme Verlag. All rights reserved.
PY - 2021/9/30
Y1 - 2021/9/30
N2 - 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.
AB - 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.
UR - http://www.scopus.com/inward/record.url?scp=85131297824&partnerID=8YFLogxK
U2 - 10.1055/a-1720-1819
DO - 10.1055/a-1720-1819
M3 - Zeitschriftenaufsätze
C2 - 35609814
AN - SCOPUS:85131297824
SN - 0023-2165
VL - 240
SP - 1077
EP - 1083
JO - Klinische Monatsblatter fur Augenheilkunde
JF - Klinische Monatsblatter fur Augenheilkunde
IS - 9
ER -
