Adult-onset Still's Disease (AOSD), often though as the adult variant of systemic
juvenile idiopathic arthritis (JIA), has an incidence of 1-3 cases per 1 million. Cardinal manifestations include
fever,
arthritis,
skin rash,
sore throat, hepatosplenomegaly and
lymphadenopathy. Prolongation in diagnosing this disease results from its similarity to infectious, malignant and
rheumatic diseases and lack of
biomarkers.
Pulmonary arterial hypertension (PAH) is a rare pulmonary complication of AOSD, and we are aware of only six cases reported in literature to date. Here we present a patient with AOSD who has developed
pulmonary hypertension as a complication. We report a case of AOSD complicated by PAH treated successfully with
tocilizumab, a humanized
monoclonal antibody to human
interleukin (IL)-6 receptor. A Pubmed and Medline search for evidence of
pulmonary hypertension in AOSD and use of
IL-6 inhibition in management was performed. Data for this study was collected from the patient's chart records. No infectious or neoplastic cause of
her disease was identified and after extensive diagnostic workup, the patient was diagnosed with AOSD fulfilling Yamaguchi criteria. After initiation of
IL-6 therapy the patient was followed over time to monitor the hemodynamic changes in pulmonary vasculature. Following treatment with
Tocilizumab, the patient showed dramatic improvement in her clinical symptoms and remains in remission, through combination of
tocilizumab (8 mg/kg),
methotrexate and
prednisone. Improvement of systemic symptoms, right heart catheterization (RHC) findings and the
VECTRA-DA score served as a measure of treatment response.
Tocilizumab has been effective in demonstrating marked improvement in both the clinical and laboratory parameters.
Tocilizumab is an effective novel treatment for AOSD with PAH. This is the first documented report of successful use of
tocilizumab in AOSD patients presenting with PAH. Prospective comparative studies could help validate its efficacy and safety.