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Publications

Journal Article Epublish 1 citation

PubMed: 39941651 DOI: 10.3390/jcm14030981

Patients with Adult-Onset Still's Disease in Germany: A Retrospective Analysis of Clinical Characteristics and Treatment Practices Ahead of the Release of the German Recommendations.

J Clin Med 14(3)

Authors: Verena Schoenau, Sarah Wendel, Koray Tascilar, Joerg Henes, Eugen Feist, Niklas Thomas Baerlecken, Florian Popp, Matthias Schmidt-Haendle, Bernhard Hellmich, Ina Kötter, Ioana Andreica, Jürgen Rech

Affiliations

  • Department of Internal Medicine 3-Rheumatology and Immunology, Friedrich-Alexander University (FAU) Erlangen-Nürnberg and Universitätsklinikum Erlangen, 91054 Erlangen, Germany.
  • Deutsches Zentrum Immuntherapie, Friedrich-Alexander University (FAU) Erlangen-Nürnberg and Universitätsklinikum Erlangen, 91054 Erlangen, Germany.
  • Department of Rheumatology and Clinical Immunology, Medical Center-University of Freiburg, Faculty of Medicine, University of Freiburg, 79106 Freiburg im Breisgau, Germany.
  • Centre for Interdisciplinary Clinical Immunology, Rheumatology and Auto-inflammatory Diseases, Department of Internal Medicine II (Oncology, Haematology, Immunology and Rheumatology), Universitatsklinikum Tübingen, 72074 Tübingen, Germany.
  • Helios Clinic Vogelsang-Gommern, Department for Rheumatology and Clinical Immunology & Experimental Rheumatology, Otto-von-Guericke-University, 39106 Magdeburg, Germany.
  • Private Practice Rheumatology, 50996 Cologne, Germany.
  • MVZ for Rheumatology Dr. M. Welcker, 82152 Planegg, Germany.
  • Rheumapraxis Bayreuth, 95444 Bayreuth, Germany.
  • Medius Kliniken, Department of Internal Medicine, Rheumatology, Pulmonology, Nephrology and Diabetology, University of Tübingen, 73230 Kirchheim-Teck, Germany.
  • University Hospital Hamburg-Eppendorf, 20246 Hamburg, Germany.
  • Clinic for Rheumatology and Immunology, 24576 Bad Bramstedt, Germany.
  • Rheumatology, Ruhr-Universität Bochum, 44801 Bochum, Germany.
  • Rheumazentrum Ruhrgebiet, 44649 Herne, Germany.
  • Center for Rare Diseases Erlangen (ZSEER), Friedrich-Alexander University (FAU) Erlangen-Nürnberg and Universitätsklinikum Erlangen, 91054 Erlangen, Germany.

Abstract

Adult-onset Still's disease (AOSD) is an autoinflammatory disorder that can be challenging to diagnose and manage. The aim of this study was to analyze retrospective data to provide insights into the clinical presentation, disease activity, and treatment patterns and outcomes of AOSD during routine clinical care prior to the release of new AOSD guidelines. This retrospective database analysis evaluated adult patients (≥18 years) with a diagnosis of AOSD who engaged in a clinical visit between 1 January 2010 and 31 December 2020. The evaluated outcomes included demographic characteristics, symptoms, disease activity, and treatment. Our study included 120 patients (67 [55.8%] of whom were female) diagnosed with AOSD according to the Yamaguchi criteria at ten German rheumatology centers. The median (quartile [Q] 1, Q3) age was 51 (36, 62) years, and the median (Q1, Q3) time from diagnosis was 9 (4, 11) years. Approximately half (66 [55.0%]) had a polycyclic disease course. The most frequent symptoms at initial diagnosis were arthralgia (105 [87.5%]) and fever (86 [71.7%]), and these symptoms continued for a substantial proportion of patients at the current visit (35 [29.2%] and 22 [18.3%], respectively). High neutrophil and ferritin levels were also common. The mean Still Activity Score, a measure of disease activity, improved from 4.66 at initial diagnosis to 1.97 at the most recent visit. The treatments most frequently used at some point in the disease course were glucocorticoids (118 [98.3%]), interleukin (IL)-1 inhibitors (89 [74.2%]), and methotrexate (85 [70.8%]). The most common current treatments were IL-1 inhibitors (55 [45.8%]), followed by methotrexate (29 [24.2%}) and glucocorticoids (28 [23.3%]). Our cohort of patients with AOSD seen at German rheumatology clinics showed strong improvements in symptoms and disease activity from initial diagnosis, but a high symptom burden remained for some patients. Future studies may be able to build on our data to document the impact of new guidelines on treatment patterns.

MeSH

Glucocorticoids, Rheumatology, Retrospective Studies, Signs And Symptoms, Diagnosis, Therapeutics, Still'S Disease, Adult-Onset, Methotrexate