Paediatric inflammatory multisystem syndrome temporally associated with COVID-19 (PIMS-TS) is a novel condition that was first reported in April, 2020. We aimed to develop a national consensus management pathway for the UK to provide guidance for clinicians caring for children with PIMS-TS. A three-phase online Delphi process and virtual consensus meeting sought consensus over the investigation, management, and research priorities from multidisciplinary clinicians caring for children with PIMS-TS. We used 140 consensus statements to derive a consensus management pathway that describes the initial investigation of children with suspected PIMS-TS, including blood markers to help determine the severity of disease, an echocardiogram, and a viral and septic screen to exclude other infectious causes of illness. The importance of a multidisciplinary team in decision making for children with PIMS-TS is highlighted throughout the guidance, along with the recommended treatment options, including supportive care, intravenous immunoglobulin, methylprednisolone, and biological therapies. These include IL-1 antagonists (eg, anakinra), IL-6 receptor blockers (eg, tocilizumab), and anti-TNF agents (eg, infliximab) for children with Kawasaki disease-like phenotype and non-specific presentations. Use of a rapid online Delphi process has made it possible to generate a national consensus pathway in a timely and cost-efficient manner in the middle of a global pandemic. The consensus statements represent the views of UK clinicians and are applicable to children in the UK suspected of having PIMS-TS. Future evidence will inform updates to this guidance, which in the interim provides a solid framework to support clinicians caring for children with PIMS-TS. This process has directly informed new PIMS-TS specific treatment groups as part of the adaptive UK RECOVERY trial protocol, which is the first formal randomised controlled trial of therapies for PIMS-TS globally.

BackgroundGranulomatosis with polyangiitis (GPA), formerly known as Wegener’s granulomatosis (WG), belongs to the group of ANCA-associated necrotizing vasculitides. This study describes the clinical picture of the disease in a large cohort of GPA paediatric patients.Children with age at diagnosis ≤ 18 years, fulfilling the EULAR/PRINTO/PRES GPA/WG classification criteria were extracted from the PRINTO vasculitis database. The clinical signs/symptoms and laboratory features were analysed before or at the time of diagnosis and at least 3 months thereafter and compared with other paediatric and adult case series (>50 patients) derived from the literature.FindingsThe 56 children with GPA/WG were predominantly females (68%) and Caucasians (82%) with a median age at disease onset of 11.7 years, and a median delay in diagnosis of 4.2 months. The most frequent organ systems involved before/at the time of diagnosis were ears, nose, throat (91%), constitutional (malaise, fever, weight loss) (89%), respiratory (79%), mucosa and skin (64%), musculoskeletal (59%), and eye (35%), 67% were ANCA-PR3 positive, while haematuria/proteinuria was present in > 50% of the children. In adult series, the frequency of female involvement ranged from 29% to 50% with lower frequencies of constitutional (fever, weight loss), ears, nose, throat (oral/nasal ulceration, otitis/aural discharge), respiratory (tracheal/endobronchial stenosis/obstruction), laboratory involvement and higher frequency of conductive hearing loss than in this paediatric series.ConclusionsPaediatric patients compared to adults with GPA/WG have similar pattern of clinical manifestations but different frequencies of organ involvement.

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Patients.-Case notes from ninety-eight patients with Takayasu arteritis were reviewed retrospectively. Drug treatment, laboratory and imaging data were analysed, and disease activity further assessed using the Indian Takayasu arteritis (ITAS) and damage scores (TADs). Results.-Nine patients were treated with biologics, all had previously received high dose prednisolone and ! 1 immunosuppressant drug, and five patients had failed cyclophosphamide. Three patients received more than one agent and eight remain on biologics. The patients prescribed biologics had more extensive arterial disease than the rest of the cohort (5-9 arteries involved, TADs 3-11), with active disease prior to the initiation of biologics (ITAS 2-9 and CRP 12-206 mg/L). The mean duration of treatment was 2.6 years, and one patient suffered a significant adverse event. Eight patients were prescribed anti-TNFa therapy, three anti-IL-6R blockade. One patient developed new arterial stenoses while receiving anti-TNFa and subsequently responded to tocilizumab. Two patients received the IL-6R antagonist as a first-line biologic. Biologic therapy resulted in a significant fall in CRP (P < 0.01) and prednisolone dose (P < 0.01). Likewise, ITAS fell from 4.1 to 1.4 (P < 0.01), and no significant progression in arterial injury was observed, either by non-invasive imaging or TADs. Discussion.-Although anti-TNFa therapy is effective in refractory Takayasu's, upto 30% do not respond or relapse. While tocilizumab offers an alternative in these cases, suppression of constitutional symptoms and CRP complicates disease monitoring, and regular imaging is required. Conclusion.-In refractory Takayasu arteritis, TNFa and IL6R blockade proved an effective option. In light of their efficacy in cyclophosphamide non-responders, we propose to use biologics ahead of cyclophosphamide in these young patients.

BackgroundDiagnosis of Periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) is currently based on a set of criteria proposed in 1999 modified from Marshall’s criteria. Nevertheless no validated evidence based set of classification criteria for PFAPA has been established so far. The aim of this study was to identify candidate classification criteria PFAPA syndrome using international consensus formation through a Delphi questionnaire survey.MethodsA first open-ended questionnaire was sent to adult and pediatric clinicians/researchers, asking to identify the variables thought most likely to be helpful and relevant for the diagnosis of PFAPA. In a second survey, respondents were asked to select, from the list of variables coming from the first survey, the 10 features that they felt were most important, and to rank them in descending order from most important to least important.ResultsThe response rate to the first and second Delphi was respectively 109/124 (88%) and 141/162 (87%). The number of participants that completed the first and second Delphi was 69/124 (56%) and 110/162 (68%). From the first Delphi we obtained a list of 92 variables, of which 62 were selected in the second Delphi. Variables reaching the top five position of the rank were regular periodicity, aphthous stomatitis, response to corticosteroids, cervical adenitis, and well-being between flares.ConclusionOur process led to identification of features that were felt to be the most important as candidate classification criteria for PFAPA by a large sample of international rheumatologists. The performance of these items will be tested further in the next phase of the study, through analysis of real patient data.Electronic supplementary materialThe online version of this article (10.1186/s12969-018-0246-9) contains supplementary material, which is available to authorized users.