The tubular transport of urate was studied in 47 uremic patients and in 20 normal subjects using probenecid and pyrazinamide tests. There was a marked increase in urate excretion per nephron as the renal function deteriorated. Presecretory reabsorption of urate per nephron, which was almost complete in normal subjects, showed a diminution with increasing severity of chronic renal failure. Until the creatinine clearance had decreased to less than 10 ml/min, the secreted urate per nephron remained almost constant, while in the end stage of renal failure it was markedly decreased. With the progression of renal disease, the postsecretory reabsorption of urate per nephron diminished. In patients with a creatinine clearance less than 10 ml/min, it was 4 times lower than in normal subjects. These findings indicate that urate secretion does not contribute to the increase of urate excretion per nephron at any level of renal failure, whereas the impairment of both reabsorptive components accounts for the augmented urate excretion per nephron in uremic patients.

Secondary thrombotic microangiopathies are associated with several underlying conditions, with most of them being resolved after the treatment of background disease. Thrombotic thrombocytopenic purpura (TTP) is a rare microangiopathy presenting with anemia, thrombocytopenia, and neurological deficits, occurring most often in various autoimmune diseases due to inhibition of ADAMTS13 by autoantibodies, as well as in pregnant women with or without an autoimmune substrate. In this article, we report two newly diagnosed TTP cases, who have not been published so far. The first is a 27-year-old woman with a history of polyarticular rheumatoid factor negative juvenile idiopathic arthritis, who presented with thrombocytopenia, anemia, schistocytes on blood smear, headache, and active arthritis. Originally she was treated successfully with plasma exchange, intravenous prednisone, and vincristine, and a few months after the TTP episode, she was commenced on rituximab, resulting in remission of primary disease and no relapse of TTP. The second case refers to a 29-year-old pregnant woman complaining of dizziness and fatigue with microangiopathic hemolytic anemia. She was treated with plasma exchanges, intravenous prednisolone, and INN human normal immunoglobulin with full remission of the TTP episode. Six and half years later, she was diagnosed with multiple sclerosis and was commenced on interferon beta-1 alpha, with no recurrent episode of TTP. These cases broaden the spectrum of autoimmune disorders manifested or complicated clinically by TTP. Furthermore, biological agents such as rituximab appear to be an effective treatment option for refractory cases of TTP related to systemic rheumatic disease, indicating an alternative therapeutic solution in persistent cases of this disorder.

The deficient T-cell proliferation in RA peripheral blood mononuclear cells is related to the relative proportions of IL-2:IL-10 rather than the absolute amounts secreted.

Background:Enthesitis-Related Arthritis (ERA) is a subtype of Juvenile Idiopathic Arthritis (JIA) subtype with an estimated prevalence ranging from 8% to 37.4%. The improvement of the disease course and outcome has been related with the introduction of biologic Disease-Modifying Antirheumatic Drugs (bDMARDs) and the uninterrupted monitoring following the transition of young patients to adult rheumatology settings.Objectives:To capture the contemporary ERA profile in Northern Greek patients by analyzing the characteristics and treatment outcome in the era of bDMARDs.Methods:This retrospective cohort study included patients who had been monitored on a 3-month schedule for ≥12 months, from 2000 to 2017. The periodic metric assessment included the disease status and burden by applying contemporary tools in respect to activity, clinical remission (CR) and damage (cJADAS, JSpADA, Wallace criteria for CR and JADI, respectively).Results:Forty-three patients, mainly male (60%) with a mean age at disease onset of 10.75 (SD:2.75) years were enrolled. The predominant joints were the hip, ankle and sacroiliac (56%, 49% and 46%, respectively). Median lag time from diagnosis to bDMARDs initiation was 8.5 months. Patients with sacroiliitis were more likely to receive bDMARDs (hazard ratio [HR]:3.26, 95% confidence interval [CI]: 1.35, 7.88). Thirty six patients (84%) achieved clinical remission (CR) on medication (CRONM), within a median time of 11 months and correlated with compliance (HR:3.62, 95%CI: 1.34, 9.76). Twenty patients (47%) experienced a flare following CR, mainly as a single episode (75%). The median flare-free survival following remission on and off medication (CROFFM) was 42 and 34 months, respectively. At the last evaluation, both median baseline cJADAS (8), and JSpADA (2) dropped to 0, while 13 patients (30%) were in CROFFM, 17 (40%) in CRONM, and 13 (30%) had persistent disease activity. The median percentage of CR per patient was 54% and no patient had JADI >0.Conclusion:Early administration of bDMARDs and compliance to monitoring and treatment improved the long-term outcome in ERA. Axial involvement emerged as a negative prognostic factor with an increased need for bDMARDs and diminished rates of CR.Disclosure of Interests:Dimitrios Deligeorgakis: None declared, Maria Trachana: None declared, Polyxeni Pratsidou-Gertsi: None declared, Despoina Dimopoulou: None declared, Anna Bettina Haidich: None declared, Alexandros Garyfallos Grant/research support from: MSD, Aenorasis SA, Speakers bureau: MSD, Novartis, gsk

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