Still's Disease in a Pediatric Patient after Liver Transplantation

Abstract

Still's disease (SD) is a multisystemic inflammatory disease characterized by persistent arthritis and in many cases with fever of unknown origin. Diagnosis of SD is challenging because of nonspecific characteristics and especially in the case of a patient with solid organ transplantation and immunosuppressive therapy where multiple causes of fever are possible. There is no diagnostic test for SD, even though some useful diagnostic criteria or laboratory findings, such as serum ferritin levels, have been proposed, and useful imaging studies for the diagnosis or followup of SD have not been developed. We report the case of a 9-year-old child who presented with high grade fever associated with joint pain after a history of liver transplantation and immunosuppressive therapy. Laboratory tests showed increased acute phase reactants, elevated ferritin, and leukocytosis. An 18 F-fluorodeoxyglucose positron emission tomography (18F-FDG PET) was performed identifying abnormal hypermetabolic areas localized in spleen, transplanted liver, and bone marrow secondary to inflammatory process. All infectious, autoimmune, and malignant causes were ruled out. A diagnosis of SD was performed and a steroid-based regimen was initiated with adequate response and no evidence of recurrence. To our knowledge this is the first case of SD following a solid organ transplant.

Atypical clinical presentation of systemic juvenile idiopathic arthritis or Still's disease: a report of two cases

“…A progression towards a macrophage activation syndrome is to be feared because the mortality rate linked to this complication stands at 5-8% [ 2 ]. As a result, even though non-steroidal anti-inflammatory drugs or general corticosteroids are most often the preferred treatment during the first weeks of the disease, the progression is towards the increasingly frequent and early recourse to methotrexate and even to biological treatment using anti-interleukin 1 or 6 [ 6 ].…”