Reumatizam, Vol. 69 No. 2, 2022.
Professional paper
https://doi.org/10.33004/reumatizam-69-2-2
Clinical presentation and treatment of adult-onset Still disease – a single-centre experience
Josip Tečer
Stela Hrkač
Antica Mihaliček
Sara Tomašinec
Karla Lazibat
Majda Golob
Nikolina Ljubičić
Lea Šalamon
, Jadranka Morović-Vergles
Joško Mitrović
; 1 Division of Clinical Imm unology, Allergology and Rheumatology, Department of Internal Medicine, School of Medicine and Faculty of Pharmacy and Biochemistry, University of Zagreb, University Hospital Dubrava, Zagreb
*
* Corresponding author.
Abstract
Introduction: Adult-onset Still disease (AOSD) is an autoinflammatory disease which most commonly occurs in adults over the age of 16, either with new onset or following earlier diagnosis of Systemic juvenile idiopathic arthritis. Due to nonspecific symptoms and a lack of specific laboratory-immunologic markers, it represents a diagnostic and treatment challenge. The aim of this retrospective observational study was to show a single-center experience in the diagnosis and treatment of AOSD. Patients and methods: This retrospective observational study included patients from the University hospital Dubrava Rheumatology and Clinical Immunology Outpatient Clinic who were diagnosed with AOSD and fulfilled the Yamaguchi classification criteria. The study included 14 patients (10 female (71.43%) and 4 male (28.57%). Information was acquired from available medical documentation – data about age, clinical presentation, laboratory parameters, treatment and outcomes were analyzed using methods of descriptive statistics. Results: The mean age at diagnosis was 44.7 years (range 19–64 y/o). Most common signs and symptoms at presentation were fever (92.7%), rash (85.7%), arthralgia (85.7%) and sore throat (71.4%). Levels of CRP were elevated in all, ferritin in 91,7% and ESR in 90% of patients. Two patients developed macrophage activation syndrome (MAS). Patients were most commonly treated with glucocorticoids (76.9%), methotrexate (46.2%) and NSAIDs (non-steroidal anti-inflammatory drugs) (7.14%) which led to disease remission in 84.6% of patients in the first year following initial diagnosis. Conclusion: Reported symptoms and laboratory findings in our group of patients with AOSD are in accordance with other similar studies. However, age at diagnosis was somewhat higher in our group which underlines the importance of AOSD as a part of differential diagnosis even in older age. It is important to keep potential life threatening complications, especially MAS, in mind when treating AOSD patients. Most common treatment of choice, glucocorticoids and
methotrexate, led to initial disease remission in the majority of our patients.
Keywords
Hrčak ID:
309196
URI
Publication date:
22.10.2023.
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