Increased ferritin response in adult Still's disease: specificity and relationship to outcome

Background: The disproportionate ferritin response encountered in some patients with adult Still's disease (ASD) may reflect a fundamental aspect in the pathophysiology of ASD. Methods: An observational case-control study of 22 ASD patients followed for 63 months. Baseline laboratory data were compared with age- and gender-matched controls with new-onset rheumatoid arthritis (RA). Serum levels of ferritin and C-reactive protein (CRP) and the ferritin/CRP ratio were related to clinical outcome in ASD through nonparametric statistical analyses. Results: Compared to RA patients, haemoglobin levels were lower (11.8 vs. 13.5 g/dL; p=0.009) and leucocyte counts (17.1 vs. 8.6 10(9)/mL; p < 0.001), erythrocyte sedimentation rate (ESR) (84 vs. 38 mm; p < 0.001), CRP (154 vs. 27 mg/L; p < 0.001), aspartate aminotransferase (ASAT) (52 vs. 23 U/l; p=0.004), serum ferritin (8750 vs. 62 mu g/L; p < 0.001) and ferritin/CRP ratios (9.7 vs. 1.7; p < 0.001) were higher in ASD patients at baseline. Six patients (27%) achieved sustained remission (monocyclic disease), while 16 patients (73%) developed chronic disease (progressive in 27%, relapsing/remitting in 46%). The levels of ESR and CRP or other baseline variables were not associated with outcome. However, baseline serum ferritin was significantly higher in ASD patients with chronic disease (p=0.04), while a cut-off of five times the normal upper level (NUL) was 100% sensitive and 60% specific for predicting chronic disease. Conclusion: An exaggerated ferritin response with levels > 5 times the NUL and high ferritin/CRP ratios is useful for distinguishing between ASD and RA patients. Ferritin levels > 5 times the NUL are also associated with a chronic disease course.