Adult Still's disease: part I. Manifestations and complications in sixty-five cases in France

Abstract

Design: a retrospective multicenter study conducted in France identified 65 cases of adult Still's disease. Data were recorded on a standardized questionnaire validated by the Inflammatory Joint Disease and Immunorheumatology Committee of the Société Française de Rhumatologie.

Objectives: (1) To compare clinical and laboratory findings in our patients with those reported in earlier studies, particularly two large series from Canada and Japan, respectively; (2) to describe the systemic and visceral complications associated with adult Still's disease.

Results: as compared with the two above-mentioned series, our study group included more patients who had experienced onset of their disease after the age of 35 years and fewer patients with involvement of the liver, spleen, or lymph nodes. Rates of occurrence of arthritis, myalgia, sore throat, pleuritis, pericarditis, and abdominal pain were significantly higher in the Canadian series than in the other two series. Arthritis was absent in one fourth of our patients. Life-threatening complications included "Still's hepatopathy", disseminated intravascular coagulation (with hemophagocytosis in some cases), and "Still's myocarditis".

Conclusion: differences in the expression of adult Still's disease were found between patients from Canada, France, and Japan. Adult Still's disease can be responsible for life-threatening complications.