Study Of Initial Clinical Manifestations in children with Systemic Juvenile Idiopathic Arthritis – Still Disease

Abstract

Background & Aim: The research aims to study the initial clinical manifestations in children diagnosed with SJIA and determine the incidence of each of them, which contributes to the early diagnosis, which depends mainly on the clinical manifestations, and similar studies had never done in university pediatric hospital.   

Materials and Methods: A Retrospective Cross-Sectional Study, the study included children with SJIA who were admitted to the Children's Hospital of Damascus University in the period from 1/1/2003 to 31/12/2019 (through 17 years) and was completed between 1/6/2020 to 1/6/2021.

Results: The research included 100 patients (49 females, 51 males) age range 1-12.5 years (mean age 5.7 years), the majority of whom were aged 1-5 years (64%). All patients had a fever (100%) . 81% of patients had arthritis, and the most common joint symptoms were arthralgia (56%), joint swelling (52%) and limited joint movement (39%). The affected joints were: the knee (61%), the ankle (41%),  the elbow (18%), the hip (17%), the feet (15%), the carpal (13%), the wrist (25) %), interphalangeal joints (5%), shoulder (4%) and TMJ (2%). 44% had a rash (72.7% non-itchy rash, 27.3% itchy rash,18.1% constant rash) and it was more frequent on the trunk (35%), face (25%) and extremities (16%). 68% of patients had lymphadenopathy, 38% had hepatomegaly (mean 3.8 cm) and 50% had an splenomegaly (mean 2.7 cm). serositis ( cardiac symptoms 4%, pulmonary symptoms 2% )Other systemic symptoms included: pallor 94%, muscle pain 64%, pharyngitis 40%, gastrointestinal symptoms 38%, macrophage activation symptoms 18%, neurological symptoms 14%,  

Conclusions: Systemic juvenile idiopathic arthritis presents with symptoms of fever and arthritis in addition to : rash, lymphadenopathy , hepatomegaly , splenomegaly and serositis . which may be absent at the beginning of the disease ,and that is inconsistent with the  current  global standards on which the diagnosis depends  (two big standards must be present) ,that may suggest reconsider about the current standards , or add some laboratory changes to diagnostic standards .