الفهرس 
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Abstract
his was a retrospective study carried out at the Pediatric Allergy and Immunology Clinic, Children’s Hospital, Ain Shams University. We conducted a retrospective analysis of the medical records of the JRA patients enrolled in the clinic between the period from January 2005 to January 2011. The data enrolled included detailed demographic information, clinical presentation of the disease, and laboratory investigations. Establishment of database for the unit through a computerized program was our main objective to allow for careful follow up of the patients and retrieving data in the future.
This study revealed that the diagnosis of JRA during the period of the study has an ascending trend during 2007 and 2010. No specific gender predilection was observed in JRA subtypes whether polyarticular JRA, oligoarticular JRA or SOJRA. Age at onset of JRA and male to female proportion did not differ significantly between seronegative and seropositive polyarticular JRA. Family history of autoimmune diseases was found in 9 (10.6%) patients, while family history of JRA was reported in 5 (5.9%) patients.
Fever, maculopapular rash (salmon pink rash), generalized lymphadenopathy and hepatosplenomegally (HSM) were the most frequent manifestations noted in SOJRA at the initial presentation. Patients with polyarticular JRA had significantly more frequent small joint arthritis as well as wrist and elbow joint arthritis. Also, arthritis was among the initial presenting features in 37 (90.2%) cases of SOJRA.
Leucocytosis with neutrophilia at initial presentation was identified more frequently 11.6% in patients with SOJRA, as compared to patients with polyarticular JRA 1.8%, and those with oligoarticular JRA 0.3%. ANA was positive in five patients (four with polyarticular JRA and one with SOJRA). None of the ANA seropositive patients was reported to have uveitis. Rheumatoid factor was positive in 5 (16.1%) patients with polyarticular JRA.
Patients with polyarticular JRA had higher albeit insignificant JRA activity at presentation as assessed by DAS 28 score than those with oligoarticular JRA and SOJRA. However, patients with SOJRA had the highest frequency of relapses during the studied period. JRA related articular damage as assessed by juvenile rheumatoid arthritis damage index- articular damage (JADI-A) was significantly higher among patients with seropositive polyarticular JRA, while JRA related extra-articular damage as assessed by juvenile rheumatoid arthritis damage index- extraarticular damage (JADI-E) was significantly higher among patients with SOJRA. MAS was reported in only one patient with SOJRA in activity with non-adherence to MTX treatment and was presented by HSM, anaemia, thrombocytopenia and DAS28 score was 4.7 Among the studied patients, 58 (68.2%) patients had grade 3 malnutrition (< 60th percentile for weight), while the weight for age > 95th percentile was reported in 6 (7.1%) patients, where SOJRA, polyarticular JRA and oligoarticular JRA were evenly represented. Also, osteoporosis with a Z score < -2 was documented in 5 (31%) patients.
Cardiac abnormalities are uncommonly reported among the studied group, where mitral regurge was reported in only two patients, while thickened interventricular septum was reported in one patient with polyarticular seronegative JRA. Pericarditis was reported in only one patient with SOJRA.
NSAIDs showed a good clinical response and remission in 12 (14.1%) patients with a mean (SD) DAS28 score after therapy was 2.5 (0.5). Corticosteroids were used in 72 (84.7%) patients where 8 (11.1%) patients passed into full remission with a mean (SD) DAS28 score of 2.2 (0.2). MTX was used in 53(62.4%) patients, where 24 (45.3%) patients passed into full remission with a mean (SD) DAS28 score of 2.5 (0.1). Leflunomide was used in 5 (5.8%) patients with good clinical response and a mean (SD) DAS28 score was 2.2 (0.14). Hydroxychloroquine was used in 9 (10.5%) patients. It produced an additive good clinical effect in two (22.2%) patients with SOJRA with DAS28 score mean (SD) of 2.7 (0.5). In conclusion, paper medical records have major limitations for precise assessment and analysis of JRA data including missed data either being lost or not assessed by the clinician in charge. This demonstrates the essential need for electronic filing system for the medical records of these patients to help better assessment of JRA presentations, its course and response to treatment and hence its outcome. Both DAS28 score, JADI-A and JADI-EA were beneficial in assessment of JRA status and long term morbidity respectively, however, in absence of computerized system, DAS28 score calculation is difficult to be done in the clinical setting.
Use of electronic filing system in clinical practice for recording the medical data of the patients will ensure precise data recording, and hence better management and follow up of JRA patients. Periodic assessment of JRA status and related damage is mandatory to be able to tailor the medications used for every individual case of JRA.