Background Adult onset of Still's disease (AOSD) is a systemic inflammatory disease characterized by fever, arthritis, salmon pink rash and elevated serum inflammatory markers. The classical fever of AOSD is intermittent with a quotidian or double-quotidian pattern. However, a significant subset of AOSD exhibits persistently high fever with little temperature fluctuation. It remains unclear as to whether the certain fever pattern is associated with specific clinical manifestations.
Objectives To investigate the association between fever pattern and clinical characteristics in patients with AOSD.
Methods A total of 70 patients with AOSD who were treated as inpatient at Seoul National University Hospital from 2004 through 2015 were enrolled. Patients were grouped based on the fever curves on days 2, 3 and 4 using hierarchical clustering. The clinical and laboratory characteristics of the groups were compared.
Results 70 patients were divided into 2 groups based on the fever curves. The group 1 with 14 patients had a higher mean temperature (38.1±0.4°C vs. 37.2±0.5°C, p<0.001) with wider daily variation (2.7±0.9°C vs. 1.9±0.7°C, p<0.001) as compared to 56 patients in the group 2.
Group 1 tended to be younger (38.4 ± 14.7 years vs. 46.1 ± 17.6 years, p=0.141) (Figure). Fever, arthritis, arthralgia and rash did not differ between them. However, group 1 tended to have more pericardial and lung involvement (42.9% v.s 23.2%, p=0.181). Group 1 had significantly lower platelet count (198,900 ± 68,000/μl vs. 312,900 ± 156,900/μl, p=0.0001), higher LDH (816.6 ± 376.4 IU/L vs. 477.5 ± 327.1 IU/L, p=0.002), higher mean ferritin level (27,004.1 ± 48,891.5 ug/mL vs. 6,852 ± 10,628.2 ug/mL, p=0.072) and d-dimer (9.5 ±7.9 ug/mL vs 3.3 ± 3.4 ug/mL) as compared to group 2, whereas white blood cell count, hemoglobin levels, CRP levels did note differ between the both groups. Group 1 tended to require longer time to a clinical remission (455.5 ± 850 days vs. 9.4 ± 115.7 days, p=0.137).
Conclusions The unbiased analysis of fever reveals the presence of at least 2 distinctive fever patterns in AOSD. Spiking fever with higher daily variation was more often associated with higher inflammatory markers and coagulopathy and required longer treatment. Thus, fever pattern might be a prognostic factor in AOSD and AOSD patients with spiking fever might need more intensified treatment.
Disclosure of Interest None declared