[Clinical course and prognostic parameters in adult-onset Still's syndrome. Own experience and review of the literature]. / Verlauf und prognostische Parameter bei Still-Syndrom des Erwachsenen. Eigene Erfahrungen und Literaturübersicht.

ABSTRACT

PATIENTS AND METHODS: Ten patients with adult onset of Still's disease (AOSD) were examined one to nine years after the established diagnosis. Clinical symptoms, laboratory parameters and the outcome of the cases are presented and compared to international literature and to Yamagushi's in 1992 proposed diagnostic criteria. Nine patients were reexamined in our out-patient clinic. The chart of one additional patient, who died 10 month after the initial symptoms was also available for data analysis. Retrospectively, it was investigated whether any parameters were predictive for a chronic or severe form of the disease. RESULTS: One patient died 10 month after the diagnosis was established due to a secondary haemophagozytic syndrome. One patient developed a chronic form of the disease, whereas 2 patients had a chronic-remitting form. Six patients presented a self-limiting, shorter than 12 month lasting course of AOSD with a restitutio ad integrum. All patients fulfilled the diagnostic criteria of Yamagushi et al. Three of 10 patients developed a chronic form of AOSD, compared to up to 70% of the patients reported by others. The patient who died was significantly older (46 years) than the average age (24,9 years) of all patients. Interestingly, he did not present Still's rash or lymphadenopathy, but rather developed a secondary hemophagocytic syndrome with an excessive hyperferritinaemia. CONCLUSION: Predicting parameters for a chronic course of the disease could not be found. Each patient's diagnosis retrospectively could be confirmed using the Yamagushi's diagnostic criteria. Thus, these criteria appear helpful in the difficult diagnostic process of this disease.