AB0954 PEDIATRIC RHUPUS A RARE OVERLAP SYNDROME: CASE SERIES OF MEXICAN CHILDREN’S HOSPITAL FEDERICO GOMEZ
Authors: Velka Cortez, Merari Gomez Cortes, Edith Adriana Benitez Vazquez, Enrique Faugier Fuentes
Journal: Annals of the Rheumatic Diseases (2019)

<h3>Background</h3> Rhupus is an overlap syndrome of rheumatoid arthritis and systemic lupus erythematous (SLE) in adult patients. Its pediatric presentation is very rare, the presentation of both juvenile idiopathic arthritis (JIA) and SLE as a clinical manifestation of the same patient has aroused different theories being the most accepted a true overlap between SLE and JIA. To diagnosis a patient with Rhupus present with clinical symptoms of SLE with positive ANA, antiDNA or antiSm, associated with clinical symptoms of JIA <h3>Objectives</h3> Describe clinical and serological findings of pediatric patients diagnosed with Rhupus of Mexican Children's Hospital Federico Gomez <h3>Methods</h3> We present a case series of 4 patients diagnosed with Rhupus. We describe the demographic characteristics, clinical presentation, serological finding, the criteria for the diagnosis of SLE (ACR 1997) and JIA (ILAR 2001) and the treatment installed <h3>Results</h3> We describe four patients with Rhupus, the medium age at diagnosis was 12.5 years, and it was more common in female 3:1. Three presented joints manifestations, integrating an initial JIA diagnosis. The evolution to an overlap syndrome took an average time of 19 months. The main symptom at diagnosis was erosive arthritis, poliarticular, morning stiffness and decreased joint range of motion. Three patients met 6 classification ACR 1997 criteria for SLE. All of the patients met ILAR 2001 criteria for JIA diagnosis. The four patients were treated with multitarget therapy. Joint manifestations were treated with methotrexate, and kidney and neurological manifestations with MFM and IV CFM. One patient required etanercept therapy for persistent joint disease All of the patients presented with elevated acute phase reactants, positive ANA, antiDNA and antiCCP. Two patients had positive antiSm antibody and three presented with positive RF <h3>Conclusion</h3> The coexistence of two or more connective tissue disease in the same patient is a very uncommon finding. The overlap of SLE and RA has been estimated between 0.01-2%. There are no reliable data about the overlap of SLE and JIA. Patients with JIA and positive anti-nuclear antibodies should have a thorough follow up taking into account the possibility of developing a juvenile Rhupus, evaluating cutaneous, renal, neuropsychiatric or hematologic manifestations in each visit. Its manifestations can be very devastating, increasing the morbidity and mortality of children. Previous findings showed that positive antiCCP antibodies are very specific for Rhupus diagnosis (96-98%). The clinical response to conventional therapy as not as than seen in patients with JIA, requiring of a multitarget treatment and biological agents to achieve a respond. Severe organ specific manifestations such as lupic glomerulonephritis and neuropsychiatric disease, are common. It is very important to recognize the Rhupus in order to make a prompt diagnosis and intervention that assures a better outcome for patients <h3>References</h3> [1] Fernández A, et al. Lupus arthropathy: A case series of patients with rhupus.ClinRheumatol. 2006; 164-7 [2] Mondal R, et al. Childhood lupus: Experience from Eastern India. IndianJPediatr. 2010; 77: 889-91 <h3>Disclosure of Interests</h3> None declared