Umut Kalyoncu1, Dilek Solmaz2, Hakan Emmungil3, Ayten Yazici4, Timucin Kasifoglu5, Gezmiş Kimyon6, Ayşe Balkarli7, Cemal Bes8, Mustafa Ozmen9, Fatma Alibaz-Oner10, Sükran Erten11, Yonca Cagatay12, Gözde Yıldırım Cetin13, Sedat Yilmaz14, Fatih Yildiz15, Omer Nuri Pamuk16, Orhan Kucuksahin17, Levent Kilic18, Veli Yazisiz19, Omer Karadag20, Süleyman Serdar Koca21, Mutlu Hayran22, Servet Akar23, Kenan Aksu24, Nurullah Akkoc25, Gokhan Keser26, Emel Gonullu27, Bunyamin Kisacik28, Ahmet Mesut Onat29, Mehmet Soy30, Nevsun Inanc31, Haner Direskeneli32, Mehmet Sayarlioglu33, Eren Erken34, Murat Turgay35, Ayse Cefle36, Ihsan Ertenli37, Salih Pay38. 1. Hacettepe University Faculty of Medicine, Department of Internal Medicine, Division of Rheumatology, Ankara, Turkey. Electronic address: umut.kalyoncu@yahoo.com. 2. Dokuz Eylul University Faculty of Medicine, Department of Internal Medicine, Division of Rheumatology, Izmir, Turkey. Electronic address: drdileksolmaz@yahoo.com. 3. Ege University Faculty of Medicine, Department of Internal Medicine, Division of Rheumatology, Izmir, Turkey. Electronic address: emmungil@mynet.com. 4. Kocaeli University Faculty of Medicine, Department of Internal Medicine, Division of Rheumatology, Kocaeli, Turkey. Electronic address: burakdefy@hotmail.com. 5. Eskisehir Osmangazi University Faculty of Medicine, Department of Internal Medicine, Division of Rheumatology, Eskisehir, Turkey. Electronic address: tkas@mtet.com. 6. Gaziantep University Faculty of Medicine, Department of Internal Medicine, Division of Rheumatology, Gaziantep, Turkey. Electronic address: gkimyon@gmail.com. 7. Pamukkale University Faculty of Medicine, Department of Internal Medicine, Division of Rheumatology, Denizli, Turkey. Electronic address: drayseayan@yahoo.com.tr. 8. Abant Izzet Baysal University Faculty of Medicine, Department of Internal Medicine, Division of Rheumatology, Bolu, Turkey. Electronic address: cemalbes@hotmail.com. 9. Izmir Ataturk Training and Research Hospital Department of Internal Medicine, Division of Rheumatology, Izmir, Turkey. Electronic address: drmozmen@yahoo.com. 10. Marmara University Faculty of Medicine, Department of Internal Medicine, Division of Rheumatology, Istanbul, Turkey. Electronic address: falibaz@gmail.com. 11. Yildirim Beyazit University, Ankara Ataturk Education and Research Hospital, Department of Internal Medicine, Division of Rheumatology, Ankara, Turkey. Electronic address: sukranerten@yahoo.com. 12. T.C Istanbul Bilim University, Department of Internal Medicine, Division of Rheumatology, Istanbul, Turkey. Electronic address: yoncacagatay@yahoo.com. 13. Sutcu Imam University, Department of Internal Medicine, Division of Rheumatology, Kahramanmaras, Turkey. Electronic address: gozdeyildirimcetin@yahoo.com. 14. Gulhane School of Medicine Division of Rheumatology, Ankara, Turkey. Electronic address: drsy75@gmail.com. 15. Cukurova University Department of Internal Medicine, Division of Rheumatology, Adana, Turkey. Electronic address: drfatih75@gmail.com. 16. Trakya University Faculty of Medicine, Department of Internal Medicine, Division of Rheumatology, Edirne, Turkey. Electronic address: omernpamuk@yahoo.com. 17. Ankara University Faculty of Medicine, Department of Internal Medicine, Division of Rheumatology, Ankara, Turkey. Electronic address: orhankcs@gmail.com. 18. Hacettepe University Faculty of Medicine, Department of Internal Medicine, Division of Rheumatology, Ankara, Turkey. Electronic address: drleventkilic@yahoo.com. 19. Akdeniz University Faculty of Medicine, Department of Internal Medicine, Division of Rheumatology, Antalya, Turkey. Electronic address: drveliyazisiz60@hotmail.com. 20. Hacettepe University Faculty of Medicine, Department of Internal Medicine, Division of Rheumatology, Ankara, Turkey. Electronic address: omerk@hacettepe.edu.tr. 21. Fırat University Faculty of Medicine, Department of Internal Medicine, Division of Rheumatology, Elazıg, Turkey. Electronic address: kocassk@yahoo.com. 22. Hacettepe University Faculty of Medicine, Department of Preventive Oncology, Ankara, Turkey. Electronic address: mhayran@hacettepe.edu.tr. 23. Dokuz Eylul University Faculty of Medicine, Department of Internal Medicine, Division of Rheumatology, Izmir, Turkey. Electronic address: servet.akar@gmail.com. 24. Ege University Faculty of Medicine, Department of Internal Medicine, Division of Rheumatology, Izmir, Turkey. Electronic address: kenan.aksu@ege.edu.tr. 25. Dokuz Eylul University Faculty of Medicine, Department of Internal Medicine, Division of Rheumatology, Izmir, Turkey. Electronic address: nurullah.akkoc@gmail.com. 26. Ege University Faculty of Medicine, Department of Internal Medicine, Division of Rheumatology, Izmir, Turkey. Electronic address: agkkeser@gmail.com. 27. Eskisehir Osmangazi University Faculty of Medicine, Department of Internal Medicine, Division of Rheumatology, Eskisehir, Turkey. Electronic address: emelorge@yahoo.com. 28. Gaziantep University Faculty of Medicine, Department of Internal Medicine, Division of Rheumatology, Gaziantep, Turkey. Electronic address: bunyamin.kisacik@yahoo.com. 29. Gaziantep University Faculty of Medicine, Department of Internal Medicine, Division of Rheumatology, Gaziantep, Turkey. Electronic address: ahmetmesutonat@yahoo.com. 30. Abant Izzet Baysal University Faculty of Medicine, Department of Internal Medicine, Division of Rheumatology, Bolu, Turkey. Electronic address: mhmtsoy@gmail.com. 31. Marmara University Faculty of Medicine, Department of Internal Medicine, Division of Rheumatology, Istanbul, Turkey. Electronic address: inanc.nevsun@gmail.com. 32. Marmara University Faculty of Medicine, Department of Internal Medicine, Division of Rheumatology, Istanbul, Turkey. Electronic address: hanerdireskeneli@gmail.com. 33. Sutcu Imam University, Department of Internal Medicine, Division of Rheumatology, Kahramanmaras, Turkey. Electronic address: sayarli@hotmail.com. 34. Cukurova University Department of Internal Medicine, Division of Rheumatology, Adana, Turkey. Electronic address: eerken@cu.edu.tr. 35. Ankara University Faculty of Medicine, Department of Internal Medicine, Division of Rheumatology, Ankara, Turkey. Electronic address: tmturgay@hotmail.com. 36. Kocaeli University Faculty of Medicine, Department of Internal Medicine, Division of Rheumatology, Kocaeli, Turkey. Electronic address: acefle@hotmail.com. 37. Hacettepe University Faculty of Medicine, Department of Internal Medicine, Division of Rheumatology, Ankara, Turkey. Electronic address: iertenli@hacettepe.edu.tr. 38. Gulhane School of Medicine Division of Rheumatology, Ankara, Turkey. Electronic address: drsalihpay@gmail.com.
Abstract
BACKGROUND: Adult-onset Still's disease (AOSD) is a rare condition, and treatment choices are frequently dependent on expert opinions. The objectives of the present study were to assess treatment modalities, disease course, and the factors influencing the outcome of patients with AOSD. METHODS: A multicenter study was used to reach sufficient patient numbers. The diagnosis of AOSD was based on the Yamaguchi criteria. The data collected included patient age, gender, age at the time of diagnosis, delay time for the diagnosis, typical AOSD rash, arthralgia, arthritis, myalgia, sore throat, lymphadenopathy, hepatomegaly, splenomegaly, pleuritis, pericarditis, and other rare findings. The laboratory findings of the patients were also recorded. The drugs initiated after the establishment of a diagnosis and the induction of remission with the first treatment was recorded. Disease patterns and related factors were also investigated. A multivariate analysis was performed to assess the factors related to remission. RESULTS: The initial data of 356 patients (210 females; 59%) from 19 centers were evaluated. The median age at onset was 32 (16-88) years, and the median follow-up time was 22 months (0-180). Fever (95.8%), arthralgia (94.9%), typical AOSD rash (66.9%), arthritis (64.6%), sore throat (63.5%), and myalgia (52.8%) were the most frequent clinical features. It was found that 254 of the 306 patients (83.0%) displayed remission with the initial treatment, including corticosteroids plus methotrexate with or without other disease-modifying antirheumatic drugs. The multivariate analysis revealed that the male sex, delayed diagnosis of more than 6 months, failure to achieve remission with initial treatment, and arthritis involving wrist/elbow joints were related to the chronic disease course. CONCLUSION: Induction of remission with initial treatment was achieved in the majority of AOSD patients. Failure to achieve remission with initial treatment as well as a delayed diagnosis implicated a chronic disease course in AOSD.
BACKGROUND: Adult-onset Still's disease (AOSD) is a rare condition, and treatment choices are frequently dependent on expert opinions. The objectives of the present study were to assess treatment modalities, disease course, and the factors influencing the outcome of patients with AOSD. METHODS: A multicenter study was used to reach sufficient patient numbers. The diagnosis of AOSD was based on the Yamaguchi criteria. The data collected included patient age, gender, age at the time of diagnosis, delay time for the diagnosis, typical AOSD rash, arthralgia, arthritis, myalgia, sore throat, lymphadenopathy, hepatomegaly, splenomegaly, pleuritis, pericarditis, and other rare findings. The laboratory findings of the patients were also recorded. The drugs initiated after the establishment of a diagnosis and the induction of remission with the first treatment was recorded. Disease patterns and related factors were also investigated. A multivariate analysis was performed to assess the factors related to remission. RESULTS: The initial data of 356 patients (210 females; 59%) from 19 centers were evaluated. The median age at onset was 32 (16-88) years, and the median follow-up time was 22 months (0-180). Fever (95.8%), arthralgia (94.9%), typical AOSD rash (66.9%), arthritis (64.6%), sore throat (63.5%), and myalgia (52.8%) were the most frequent clinical features. It was found that 254 of the 306 patients (83.0%) displayed remission with the initial treatment, including corticosteroids plus methotrexate with or without other disease-modifying antirheumatic drugs. The multivariate analysis revealed that the male sex, delayed diagnosis of more than 6 months, failure to achieve remission with initial treatment, and arthritis involving wrist/elbow joints were related to the chronic disease course. CONCLUSION: Induction of remission with initial treatment was achieved in the majority of AOSD patients. Failure to achieve remission with initial treatment as well as a delayed diagnosis implicated a chronic disease course in AOSD.