Dr. Sharath Kumar

Background: Many children with sJIA may have a delayed onset of arthritis and so fail to fulfil the ILAR criteria for sJIA. This study was undertaken to determine whether the Yamaguchi criteria (for adult onset Still's disease) is useful in classification of children with systemic juvenile idiopathic arthritis (sJIA) particularly in "pre-arthritic", pure systemic, phase of the illness. A secondary objective was to determine the time delay between disease onset and onset of arthritis in our sJIA cohort. Methods: Retrospective chart review all patients with a diagnosis of systemic juvenile arthritis in our department from Jan 1, 2004 to Jan 1, 2010. Results: Twenty boys and eleven girls formed the study cohort. Thirteen patients were diagnosed with "suspected" sJIA due to typical systemic features but an absence of arthritis. Overall, the Yamaguchi criteria was fulfilled in a higher number of patients in the study (n=23) as compared to the ILAR criteria (n=18). Among the 13 "suspected" sJIA patients, 12 fulfilled the Yamaguchi criteria. Overall, either ILAR criteria or Yamaguchi criteria was fulfilled in 30 patients (96.8% of patients). The degree of association between the two criteria was poor (Phi coefficient = -0.352, p=0.05). Eleven out of eighteen patients with arthritis gave a history of delay in onset of arthritis (range=15 days to more than a year; median=30 days). Thus a total of 24 patients (75%) had a delay in onset of arthritis at onset of disease. Conclusion: Patients with sJIA can have a significant period during their course (particularly at onset) when they do not have arthritis. The Yamaguchi criteria may be useful in this subset of patients in the "pre-arthritic" phase of the disease. Future criteria should incorporate the strengths of both, the Yamaguchi and the ILAR criteria.

Patients with systemic onset juvenile idiopathic arthritis (SoJIA) are rarely known to develop coronary artery dilatation. The American heart association (AHA) statement on evaluation of suspected Kawasaki disease (KD) would lead some SoJIA patients (particularly in the early stages of the disease) to be inaccurately classified as KD. In addition to the institution of inappropriate therapy with IVIG, misdiagnosis as KD can delay definitive treatment for these SoJIA patients who probably have a worse predicted outcome. We present a 6-year-old male patient with SoJIA who was initially classified as incomplete KD. The child developed life-threatening macrophage activation syndrome (MAS). Previous literature regarding coronary dilatation in SoJIA is also reviewed.

Posterior reversible encephalopathy syndrome (PRES)or Reversible posterior leukoencephalopathy syndrome (RPLS) is a neurological complication associated with various illnesses and medications(including rheumatological illnesses and their medications). Cyclosporine is the drug which is most commonly implicated in the causation of this condition. The authors report a 6 year old patient with systemic onset juvenile idiopathic arthritis (SoJIA) with macrophage activation syndrome who developed PRES during treatment with pulse methylprednsiolone therapy. In view of persitent SoJIA disease activity, the child was subsequently treated with Cyclosporine. The child made a complete neurological recovery. This case report highlights that, in accordance with recent literature, PRES is a complication of a unique set of conditions comprising of inflammation, hypertension, immunosupression and nephropathy in different degrees and does not necessarily preclude the use of drugs implicated as inciting agents. This has important implications in rheumatology where cyclosporine is a life saving medication used in macrophage activation syndrome.

Aim: To determine the clinical and laboratory characteristics of patients diagnosed with pediatric systemic lupus erythematosus (pSLE) in the general pediatrics department of a tertiary level hospital in southern India and compare them to data of case series from other parts of the country. Methods: Retrospective chart review by two independent reviewers. All patients diagnosed with SLE in the pediatrics department of the Amrita Institute of Medical Sciences hospital between 1 January 2004 and 31 September 2008, were included in the study. Results: Twenty children were diagnosed with pSLE in the above-mentioned period (female : male ratio 2.3 : 1.0). Fever (75% of patients) and arthralgia (65%) were the most common clinical presenting features. Many patients who were referred as 'Fever of unknown origin' (37.5%) or 'idiopathic thrombocytopenic purpura' (15%) fulfilled the diagnosis of SLE on detailed evaluation. Renal manifestations were present in 11 patients, seven of whom underwent a biopsy. Class IV lupus nephritis was the most common finding (4/7). A very high percentage of our patients had hypocomplementemia (85%). The statistical significance of the differences between our cohort and previously reported cohorts could not be determined. Conclusions: pSLE patients in our series, compared to previous literature from India had a much higher incidence of fever, thrombocytopenia and hypocomplementemia at presentation and much lower incidence of arthritis. It is unclear whether these differences represent unique characteristics of ethnically dissimilar subsets of the Indian population. A high index of suspicion should be maintained in order to make an early diagnosis of pSLE, since the most common presenting features in our cohort were arthralgia and fever.