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

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.

Uvod: Stillova bolest odrasle dobi (AOSD , engl. adult onset Still disease) autoinflamatorno je stanje koje se najčešće pojavljuje u odraslih iznad 16 godina, bilo kao novonastala bolest ili nakon ranije dijagnosticiranoga idiopatskog sistemskog juvenilnog artritisa. S obzirom na nespecifične simptome i nedostatak dijagnostičkog laboratorijsko-imunološkog markera, bolest predstavlja dijagnostički i terapijski izazov. Cilj ovog istraživanja bio je prikazati iskustvo jednog centra u dijagnosticiranju i liječenju AOSD -a. Ispitanici i metode: U ovo retrospektivno opservacijsko istraživanje uključeni su bolesnici Zavoda za kliničku imunologiju, alergologiju i reumatologiju KB Dubrava kojima je postavljena dijagnoza AOSD -a te su ispunjavali Yamaguchijeve kriterije (1992.). Ukupno je uključeno 14 bolesnika (10 žena i 4 muškarca). Podatci o dobi, kliničkoj slici, laboratorijskim nalazima, terapiji i ishodu liječenja prikupljeni su iz dostupne medicinske dokumentacije te su analizirani metodama deskriptivne statistike. Rezultati: Prosječna dob ispitanika pri postavljanju dijagnoze bila je 44,7 godina (raspon 19–64 god.). Najčešći simptomi pri dijagnozi bili su febrilitet u 92,9%, osip u 85,7%, artralgije u 85,7% te grlobolja u 71,4% ispitanika. Dvoje je bolesnika razvilo sindrom aktivacije makrofaga (MAS ). Povišene vrijednosti CRP-a nađene su u svih bolesnika, feritina u 91,7% te sedimentacije eritrocita u 90% bolesnika. Bolesnici su najčešće bili liječeni glukokortikoidima (76,9%), metotreksatom (46,2%) i nesteroidnim protuupalnim lijekovima (NSAID, engl. non-steroidal anti-inflammatory drugs) (7,14%) što je dovelo do remisije unutar prve godine od postavljanja dijagnoze u 84,6% ispitanika. Zaključak: Simptomatologija i laboratorijski nalazi u našoj skupini bolesnika s AOSD -om u skladu su s drugim sličnim istraživanjima. S druge strane, dob pri postavljanju dijagnoze u našoj grupi nešto je viša, što upućuje na važnost AOSD -a kao dijela diferencijalne dijagnoze i u starijoj dobi. Uvijek je potreban oprez zbog mogućeg razvoja potencijalno životno ugrožavajućih komplikacija, poput MAS -a. Najčešći modaliteti liječenja bili su glukokortikoidi i metotreksat te je u većine bolesnika došlo do inicijalne remisije bolesti.