Experience with Cidofovir as an adjunctive therapy in a patient of adenovirus-induced macrophage activation syndrome in systemic arthritis

Abstract

A 5-year-old female child, with known systemic juvenile idiopathic arthritis diagnosed at 18 months of age (on low dose Prednisolone + Methotrexate + Leflunomide + Tocilizumab), presented with fever for 1 day, vomiting, drowsiness followed by seizures. On admission to PICU, she was drowsy, tachycardic, tachypneic, with rashes, and hepatosplenomegaly. Lab findings showed thrombocytopenia, leucopenia, low ESR, normal CRP, elevated liver enzymes, high ferritin, LDH, and triglycerides suggestive of macrophage activation syndrome (MAS). Chest X-ray showed left basal pneumonia and DNA PCR of throat swab revealed adenovirus. She was diagnosed as adenovirus-triggered MAS and was initiated on pulse methylprednisolone (6 mg/kg). Because of suboptimal response after 2 doses, manifested by increasing drowsiness, further fall in platelets and rising ferritin, methylprednisolone dosage was increased to 30 mg/kg/day with the addition of oral cyclosporine (4 mg/kg/day). In view of worsening of the chest X-ray and increasing oxygen requirement, Cidofovir infusion (1 mg/kg thrice weekly) was also started simultaneously considering increased activity of the adenoviral infection concurrent to immunosuppression. Within 48 h, the child showed signs of recovery with improved consciousness, lower oxygen requirements, and improving lab parameters. She was discharged after 3 weeks of IV Cidofovir, on oral prednisolone and cyclosporine. To the best of our knowledge, this is the first reported use of Cidofovir in adenovirus-induced MAS.

Macrophage Activation Syndrome in Viral Sepsis

“…Human adenovirus infection is seldom associated with HLH. The majority of reported cases pertain to children, while only a few cases have involved adults [58,[86][87][88]. A recent study investigated the risk factors associated with HLH development in children with severe adenovirus pneumonia and found that patients with longer duration of fever (over 12.5 days) and elevated triglyceride levels (over 3.02 mmol/L) were at higher risk of developing HLH [89].…”

Management of Hepatitis A-induced Macrophage Activation Syndrome With Anakinra in Systemic Juvenile Idiopathic Arthritis: A Case Report

“…MAS identifies hemophagocytic syndrome in children with systemic juvenile idiopathic arthritis (sJIA) and other chronic rheumatic diseases [ 1 ]. MAS may develop spontaneously, as a complication of active disease, or can be induced by an infection, changes in drug therapy, or a toxic effect of medication [ 2 ]. Most cases (58% to 88.9%) have possible triggering factors prior to the onset of MAS [ 1 ].…”