Still’s disease (SD) is a heterogeneous autoinflammatory disorder for which several phenotypes have been described. We conducted a retrospective study to re-evaluate the dichotomous view of the disease, to compare the juvenile and adult forms, and to look for prognostic factors. We collected data from ten French centers, seeking patients with a diagnosis of adult-onset SD (AOSD) or systemic juvenile idiopathic arthritis (sJIA). We identified 238 patients, 152 (64%) of whom had AOSD while 86 (36%) had sJIA. The median age at SD onset was 26.6 years. In patients with identifiable patterns, the course of SD was systemic in 159 patients (74%), chronic in 55 (26%). Sore throat and myalgia were more frequent in patients with AOSD. Abnormal liver tests, serum ferritin and C-reactive protein levels were higher in AOSD group. Fever and skin rash were predictive of complete remission or recovery and high lactate dehydrogenase level was a poor prognosis factor. Symptoms such as splenomegaly, skin rash, high polymorphonuclear neutrophils count and macrophage activation syndrome were predictive of a systemic phenotype. Overall, there were no major differences between sJIA and AOSD. Our results are consistent with the “biphasic” model of an autoinflammatory disease that can progress to chronic arthritis if not treated early.
Cystic echinococcosis is a widespread zoonosis caused by the proliferation of the larval stage of Echinoccocus granulosus, which leads to the formation of cysts. In 75% of cases, there is a single cyst, which mostly affects the liver and the lungs. Disseminated forms are exceptional, and their treatment is complex and non-standardized. There are currently four therapeutic strategies. The indication for each strategy depends on several factors, including the number and stage of the cysts. We reported the case of a 24-year-old woman, suffering from a multivisceral disseminated hydatidosis, with a large number of cysts in the lung, the liver, the spleen and the peritoneum. Initially, she presented with cutaneous jaundice, associated with biological inflammatory syndrome, cholestasis and cytolysis. This was a sign of a frequent complication of cystic echinococcosis: the rupture of a cyst in the bile duct. She was treated with two antiparasitic drugs and then underwent a surgery to remove a large number of cysts. During surgery, an omentectomy, splenectomy and hepatic lobectomy were necessary. We described a rare case of disseminated cystic echinococcosis, for which there is currently no standardized therapeutic strategy.