OBJECTIVES: To report an unusual case of lipoma aborescens (LA) presented in a patient with treatment-responsive juvenile spondyloarthropathy (JSPA) and to summarize the clinical manifestations, therapy and prognosis of LA by literature review. METHODS: We report an atypical case of a 17-year-old patient with an initial presentation of juvenile spondyloarthropathy, whose inflammatory condition was improved successfully by traditional anti-rheumatic drugs and an anti-TNF alpha agent but developed progressive swelling of bilateral knees. Lipoma arborescens were diagnosed in each knee by synovial biopsy obtained by arthroscopic surgery. Fifty-one cases of LA have been reported and are reviewed in detail. RESULTS: Clinically, LA could present as monoarthritis or oligoarthritis. The lateral compartment of the knee is the most common site of involvement. Several cases were reported as a comorbidity of inflammatory diseases, but were not improved by anti-inflammatory therapy. Most patients were diagnosed by classic MRI and biopsy findings. The lesions can be managed by open or arthroscopic surgery, but a minority of the cases may have reoccurrence in the same or opposite joint. CONCLUSIONS: LA is a very rare lesion of the synovial and bursal tissue with an unknown etiology. It is considered to be a benign proliferation of the synovial fat associated with trauma, degenerative or inflammatory conditions. LA should be considered as a secondary or comorbid condition in inflammatory arthropathies if other joints respond well to intensive therapy and one or more do not.

The objective of the study to review an experiential cohort of patients receiving IACS and review the associated literature. Review of 121 IACS in 61 patients with JIA. At 3-month intervals, injected joints were evaluated for swelling and range of motion, and the patient and parent were questioned regarding associated pain and morning stiffness. Data were analyzed by log-rank analysis according to injected corticosteroid preparation and its dosage. Adverse events were also recorded. A thorough literature search was done for the literature review. Mean duration of response was 12.5 months (52% of joints in remission at 1 year, 20% after 2 years, and 7% after 3 years). Response was longer with at least 1 mg/kg of corticosteroid, with the longest responses seen with triamcinolone hexacetonide (THA)>triamcinolone acetonide>methylprednisolone. Adverse events were cutaneous atrophy at three injections sites (2.5%), and transient Cushingoid habitus and increased appetite in two patients (3%). Review of the literature generated similar responses to those included herein. Thus, there have been several recommendations for IACS to be a major JIA treatment, and surveys now demonstrate a high level of usage by pediatric rheumatologists. In conclusion the use of IACS in JIA substantiated. THA at a dose of 1-1.5 mg/kg is ideal.

OBJECTIVE: We previously demonstrated that levels of fibrin d-dimer correlate with disease activity and response to therapies in systemic juvenile idiopathic arthritis (sJIA). We hypothesized that persistence of D-dimer elevation in the patterns previously described, but over a longer followup period, would signal poor outcome. METHODS: We studied 31 children identified from 2 centers. Subjects were assigned a risk category based on their first obtained D-dimer concentration. Risk categories were based on results of our initial study, where normalization of D-dimer in patients no longer taking immunosuppressive therapy predicted good short-term outcome, and persistent D-dimer elevation while taking immunosuppressives predicted bad outcome (radiographic abnormalities, joint replacement surgery, or poor functional class) or a severe systemic manifestation. Outcome was determined at the last followup visit, a minimum of 2 years after measurement of the initial d-dimer level. RESULTS: The 31 children were a mean 16.4 years old at an average of 8.8 years after their initial diagnosis. Ten children had a severe outcome during this period; all 10 had a study baseline risk category of "high." Of the 14 subjects who had a high risk category at study baseline, none had a mild outcome. CONCLUSION: Our study indicated that a paradigm of risk of severe disease based upon persistent elevation of fibrin d-dimer on first measurements (greater than a mean of 29 months in our initial study and at least 24 months in the additional subjects) is promising to predict poor longer-term outcome in sJIA. A larger prospective study is warranted to substantiate the preliminary data and assess the relative comparative value to other biomarkers and clinical endpoints.