Adult-onset still's disease and budd-chiari syndrome: a case report

مقدمه : Adult onset still’s disease (AOSD) is a rare autoinflammatory disease displaying with a wide range of non-specific symptoms and budd-chiari syndrome (BCS) is an uncommon disorder characterized by obstruction of hepatic venous outflow.هدف : BCS occurs rarely in AOSD patients but can be a life-threatening disease. معرفی بیمار : We present the case of a young patient who presented with persistent fever, sore throat, elbow, hand fingers and knees arthralgia with abdominal pain. The patient's symptoms had started ۷ days before the referral. Imaging and laboratory data led to the diagnosis of BCS in the context of AOSD. The patient treated with corticosteroid in combination of warfarin with favorable outcome and complete improvement of signs and symptoms.بحث و نتیجه گیری: AOSD is a rare systemic autoinflammatory disease with a prevalence ranging from ۱ to ۳۴ cases per million people. ASOD affects young people with an average age of ۳۶ years at the time of diagnosis.It does not have specific clinical manifestation, but a wide range of symptoms is observed in these patients .Leukocytosis is commonly observed in these patients. No specific laboratory test is seen in these patients, but ferritin increases significantly. In addition to increasing ferritin and leukocytosis, erythrocyte sedimentation rate increases. Also, rheumatic diseases, infections and malignancies should be excluded before the final diagnosis of AOSD. This venous obstruction can be from the small hepatic vein to the inferior vena cava. An increase in liver enzymes is observed in ۹۲% of patients.Compared with other liver diseases in BCS patients, the prognosis is acceptable with the treatment of patients.Thrombosis can occur in AOSD patients. BCS is thrombosis of the hepatic veins or inferior vena cava. Spontaneous outcome in symptomatic patients is poor. BCS occurs rarely in AOSD patients but can be a life-threatening disease. Disease pathogenesis is unknown, but the similarity of AOSD and BCS in the dysregulation of innate immunity can be suggested as pathogenesis.

کلیدواژه ها:

Still's disease ، adult-onset ، hepatic vein thrombosis ، fever ، hepatitis ، skin rash ، exanthema ، rheumatology