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Recurrent pericarditis (RP) is a rare cause of morbidity in children. Non-steroidal anti-inflammatory drugs (NSAIDs), glucocorticoids and colchicine are the standard of care in adults. Recently, anakinra has been proven to be effective in patients with steroid-dependence and colchicine resistance.

Introduction: Systemic juvenile idiopathic arthritis (sJIA) accounts for 10-20% of all patients with JIA. The demonstration of a key role of IL-1 and IL-6 in the pathogenesis of the disease, led to consider sJIA an autoinflammatory disease: this explain the successfully use of IL-1 and IL-6 inhibitors. While the efficacy and safety of anakinra in sJIA is widely documented, there are no reports on large series of patients treated with canakinumab outside of the setting of clinical trials. Objectives: The aim of this study was to evaluate clinical response rate and disease course of canakinumab in Italian cohort of patients with sJIA. Methods: This is a retrospective multicenter study. Demographic features, previous medical history and therapies was evaluated for each patients. Clinical features, laboratory parameters and adverse events were collected at baseline and after 6 months from starting canakinumab. Clinically inactive disease (CID) was defined according to Wallace criteria. Results: We enrolled 82 (50 F) patients with sJIA treated with canakinumab from 2006 to 2020; 75 of them reached a follow up of 6 months. At baseline 49 patients (59.8%), of which 36 in active disease (AD) and 13 in CID, were previously treated with anakinra, while 33 patients in AD (40.2%) were naïve. At 6 months of follow-up 51/75 patients (68%) met criteria of CID off-glucocorticoids, including all 13 patients in CID at baseline, 19 patients in AD previously treated with anakinra and 19 patients naïve. Twenty-four patients (32%) maintained AD. To evaluated if the response to canakinumab might be related to the baseline features we excluded 13 patients in CID at baseline; we divided the 62 patients in responders (38/62, 61.3%) and non-responders (24/62, 38.7%). There were no significant differences between the two groups regarding demographic, clinical and laboratory parameters, except for a higher number of active joints (p=0.021) and for a greater use of disease-modifying antirheumatic drugs (DMARDs) (p

The Lupus Foundation of America (LFA) convened an international working group to obtain a consensus definition of disease flare in lupus. With help from the Paediatric Rheumatology International Trials Organization (PRINTO), two web-based Delphi surveys of physicians were conducted. Subsequently, the LFA held a second consensus conference followed by a third Delphi survey to reach a community-wide agreement for flare definition. Sixty-nine of the 120 (57.5%) polled physicians responded to the first survey. Fifty-nine of the responses were available to draft 12 preliminary statements, which were circulated in the second survey. Eighty-seven of 118 (74%) physicians completed the second survey, with an agreement of 70% for 9/12 (75%) statements. During the second conference, three alternative flare definitions were consolidated and sent back to the international community. One hundred and sixteen of 146 (79.5%) responded, with agreement by 71/116 (61%) for the following definition: “A flare is a measurable increase in disease activity in one or more organ systems involving new or worse clinical signs and symptoms and/or laboratory measurements. It must be considered clinically significant by the assessor and usually there would be at least consideration of a change or an increase in treatment.” The LFA proposes this definition for lupus flare on the basis of its high face validity.

vgf is a neurotrophin response-specific, developmentally regulated gene that codes for a neurosecretory polypeptide. Its transcription in neuronal cells is selectively activated by the neurotrophins nerve growth factor (NGF), brain-derived neurotrophic factor, and neurotrophin 3, which induce survival and differentiation, and not by epidermal growth factor. We studied a short region of the rat vgf promoter which is essential for its regulated expression. A cyclic AMP response element (CRE) within this region is necessary for NGF induction of vgf transcription. Two sites upstream of CRE, an E box and a CCAAT sequence, bind nuclear protein complexes and are involved in transcriptional control. The E box has a dual role. It acts as an inhibitor in NIH 3T3 fibroblasts, together with a second E box located downstream, and as a stimulator in the NGF-responsive cell line PC12. By expression screening, we have isolated the cDNA for a basic helix-loop-helix transcription factor, a homolog of the HTF4/HEB E protein, that specifically binds the vgf promoter E box. The E protein was present in various cell lines, including PC12 cells, and was a component of a multiprotein nuclear complex that binds the promoter in vitro. The E box and CRE cooperate in binding to this complex, which may be an important determinant for neural cell-specific expression.

Die vorliegenden Forschungen zeigen, das die Arteriolen von Leber, Pankreas, Magen, Dunndarm und Dickdarm, Nieren und Hirn, ahnlich wie Venen, Endokard des Vorhofs und des rechten Ventrikels sowie Lungenarterien nicht mit sensitiven und KCN-reizbaren Endigungen ausgestattet sind.