ANCA-associated vasculitis in childhood: Recent advances

Abstract Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides are rare systemic diseases that usually occur in adulthood. They comprise granulomatosis with polyangiitis (GPA, Wegener’s), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA, Churg-Strauss syndrome). Their clinical presentation is often heterogeneous, with frequent involvement of the respiratory tract, the kidney, the skin and the joints. ANCA-associated vasculitis is rare in childhood but North-American and European cohort studies performed during the last decade have clarified their phenotype, patterns of renal involvement and their prognostic implications, and outcome. Herein, we review the main clinical and therapeutic aspects of childhood-onset ANCA-associated vasculitis, and provide preliminary data on demographic characteristics and organ manifestations of an Italian multicentre cohort

Identification of the Clinical Features Distinguishing Psoriatic Arthritis and Fibromyalgia

To identify the clinical features that can help to distinguish between psoriatic arthritis (PsA) and fibromyalgia (FM)

PTPN22 R620W polymorphism in the ANCA-associated vasculitides

Objectives. PTPN22 is involved in T-cell activation and its R620W single-nucleotide polymorphism ( SNP) has been shown to predispose to different autoimmune diseases. The aims of this study were to investigate the role of the PTPN22 R620W SNP in conferring susceptibility to the ANCA-associated vasculitides (AAVs), and to explore potential associations between the PTPN22 genotype and the disease manifestations. Methods. PTPN22 R620W SNP was genotyped in a cohort of 344 AAV patients [143 with granulomatosis with polyangiitis (Wegener's) (GPA), 102 with microscopic polyangiitis (MPA) and 99 with Churg-Strauss syndrome (CSS)] and in 945 healthy controls. Results. The frequency of the minor allele (620W) was significantly higher in GPA patients than in controls [P = 0.005, chi(2) = 7.858, odds ratio (OR) = 1.91], while no statistically significant association was found with MPA or CSS. Among GPA patients, the 620W allele was particularly enriched in ANCA-positive patients as compared with controls (P = 0.00012, chi(2) = 14.73, OR = 2.31); a particularly marked association was also found with ENT involvement (P = 0.0071, chi(2) = 7.258, OR = 1.98), lung involvement (P = 0.0060, chi(2) = 7.541, OR = 2.07) and skin manifestations of all kinds (P = 0.000047, chi(2) = 16.567, OR = 3.73). Conclusion. The PTPN22 620W allele confers susceptibility to the development of GPA (but not of MPA or CSS), and particularly of its ANCA-positive subset

The theory and practice of public choice. Introduction to the special issue in honour of Francesco Forte

The editorial introduces to the special issue based on the international conference on ‘Empirical and Experimental Advances in Public Choice’ held in October 2018 in Reggio Calabria, Italy, in honour of Francesco Forte

Tocilizumab for polymyalgia rheumatica: Report of two cases and review of the literature.

Background: Glucocorticoids (GC) are the mainstay of treatment of polymyalgia rheumatica (PMR). However GC-related adverse events occur frequently, particularly in patients with relapsing disease. Several studies have demonstrated that IL-6 is a key player in the pathogenesis of PMR. Objectives: To report 2 patients with PMR treated with the anti-IL-6 receptor monoclonal antibody tocilizumab (TCZ) and to review the published evidence on the efficacy and safety of TCZ in patients with PMR. Methods: We treated 2 GC-naive patients with newly diagnosed pure PMR with monthly TCZ infusions (8 mg/kg body weight) for 6 months. Disease activity and drug tolerability were assessed clinically, by laboratory tests, and bilateral shoulder ultrasonography before starting the treatment and subsequently every month during TCZ therapy. We performed a systematic literature search (PubMed until July 2012) using the terms "tocilizumab," "anti-IL-6-receptor," "polymyalgia rheumatica," "giant cell arteritis", and "large-vessel vasculitis" to identify published reports of patients with PMR treated with TCZ. Results: One of our patients responded well to TCZ, while the other patient required GC therapy after the 2nd TCZ infusion because of lack of appreciable clinical response. Both patients tolerated TCZ well. The review of the literature revealed 4 reports with a total of 9 patients who received TCZ for PMR. In 7 of these 9 patients, PMR was associated with giant cell arteritis. Including our patients, 5 patients received TCZ alone and 6 TCZ plus GC. A good response to TCZ treatment was observed in all patients reported in the literature without any major adverse events. Conclusions: TCZ both as monotherapy and in association with GC appears to be mostly effective and safe to treat patients with PMR. However, larger controlled studies are required to confirm these favorable data

Drug-induced retroperitoneal fibrosis: a case/non-case study in the French PharmacoVigilance Database

International audienceObjectives: The potential role of drugs in the onset of retroperitoneal fibrosis (RPF) is poorly understood. The aim of this study was to identify drugs that may cause RPF.Methods: The authors used case/non-case method in the French PharmacoVigilance Database (FPVD).Results: Among the 722992 reports recorded, 73 cases of RPF were identified. 67% were men and the median age was 60 years (range 26-87). In these 73 cases, 176 drugs were 'suspect.' Derivatives of ergot alkaloids (DEA) presented the most significant association with RPF. To a lesser extent, significant associations were found with many drugs used in cardiology, e.g. beta-blockers, platelet antiaggregant, statins, and antihypertensive drugs, drugs used in neuropsychiatry, e.g. hypnotics, antiepileptic drugs, anxiolytics, antipsychotics, and antidepressants, and with other pharmacological classes, e.g. TNF-alpha antagonists.Conclusion: This study confirmed an association between RPF and derivatives of ergot alkaloids. These data represent a pharmacovigilance signal despite the limits of non/non-case method (underreporting, confounding factors, etc.). Indeed, a significant signal was found with drugs less known (TNF-α antagonists) or not known (some hypnotics, antiepileptic drugs, antipsychotics, anxiolytics, and antidepressants) to induce such an adverse drug reaction (ADR). Finally, these data could contribute to realize prospective studies to confirm these signals