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Pediatric Rheumatology ; 20(SUPPL 1), 2022.
Article in English | EMBASE | ID: covidwho-1677521


Introduction: During COVID-19 pandemic, acute acral chilblain-like lesions (ACBLL), reminiscent of lupus pernio, were observed during both first and second COVID-19 peak among patients with highly suspected (but mostly unconfirmed) infection with SARS-CoV-2.The aetiology of this phenomenon has not been elucidated yet and pathogenetic mechanism remains unknown. Several studies have investigated cytokine and chemokine profile in patients with COVID-19 but an accurate characterization of ACBLL patients is lacking. Objectives: We aimed to describe the clinical, laboratory and immunological features of children presenting with ACBLL referred to our Institute during the COVID-19 pandemic spread. Methods: We prospectively collected data of children referred to our Institute from April 1st 2020 to February 28th 2021. We investigate the presence of SARS-CoV2 infection through RT-PCR from nasopharingeal swabs and three different serologic kit. All patients underwent a laboratory work-up including coagulation, viral serology and autoantibodies panel. Finally, we analysed peripheral blood IFN signature, a panel of inflammatory biomarkers in serum/plasma by a flow cytometry bead array (CXCL10, CXCL9, IL-6, IL-1β,TNFα) and the presence of SARS-CoV2 T specific lymphocytes. Results: We examined 36 children during the first peak, and 11 children during the second COVID-19 peak (F: 28 median age 12 y), at a median delay of 26 days after symptoms onset (2-73 days). Fifteen patients (31%) presented non-specific systemic symptoms preceding ACBLL onset. Nine patients (19%) reported a possible contagion from a close contact. All patients presented stereotypical features resembling classical chilblains with acral erythematousedematous violaceous plaques and nodules localized on the toes (n= 35, 74%), the fingers (n=5, 10%) or on both sites (n=7, 15%). SARSCoV- 2 RNA detection resulted negative except for 2 patients. Furthermore, ten patients observed during the first wave showed a recurrence during the second (F:6), which developed 1-4 weeks after the second COVID-19 peak the clinical features were comparable to those of the previous episode. Five of them (50%) reported nonspecific systemic symptoms before onset and/or close contact with SARS-CoV2 positive subject. Repeated SARS-CoV-2 specific IgG/IgA tests were negative for all patients except for three cases (two of them with positive swabs). Neither common virus serology nor coagulation studies revealed significative results. Two patients presented positive ANA and anti β2 glycoprotein, respectively. A positive IFN signature was detected in 12/ 33 patients (36%).Among the 35 patients tested, the cytokine array showed high levels of IP10 (n= 35, range 12.4-739 pg/ml, n.v. 0.0-0.2 pg/ml) and a mild increase of IL-6 (n=21, range 2.4-401 pg/ml, n.v. 0.5-2.2pg/ml), without alterations of CXCL9, IL-1β and TNFa. The detection of SARS-CoV2 specific lymphocytes showed the presence of SARS-CoV2 specific lymphocytes in 9/17 (52%) patients tested (validated with positive and negative controls), only one of them with a positive serological test. Conclusion: Albeit the pathogenetic mechanism of ACBLL remains to be elucidated, our preliminary results showed a significant increase in serum IP10 levels, not frankly associated with a peripheral blood IFN signature, which is instead a characteristic of pernio-related chilblains. We also proved the presence of a T-specific memory against in 50% of the tested patients, despite the negativity of coltures and serological tests, strengthening the link between SARS-CoV2 infection and this peculiar clinical manifestation.

Pediatric Rheumatology ; 18(SUPPL 3), 2020.
Article in English | EMBASE | ID: covidwho-1094038


Introduction: Italy was affected by the SARS-CoV-2 epidemic after its outbreak in China. With a 4-weeks delay after the peak in adults, we observed an abnormal number of patients with characteristics of a multi-inflammatory disease and similarities with Kawasaki Disease (KD). Others reported similar cases, defined PIMS-TS or MIS-C.1,2 Objectives: To better characterize clinical features and treatment response of PIMS-TS and to explore its relationship with KD. Methods: We conducted an observational, retrospective, multicenter study. On April 24th-2020 the Rheumatology Study Group of the Italian Pediatric Society launched a national online survey, to enroll patients diagnosed with KD or with a multisystem inflammatory disease between February 1st 2020 and May 31st. The population was then divided into two different groups: 1) Classical and incomplete KD, named Kawasaki Disease Group (KDG);2) KD-like multi-inflammatory syndrome, named KawaCOVID (KCG). An expert panel of pediatric rheumatologists re-analyzed every single patient to ensure appropriate classification. Data were collected with an online database. Results: 149 cases were studied, 96 with KDG and 53 with KCG. The two population significantly differed for clinical characteristics (see table 1). Lymphopenia, higher CRP levels, elevated Ferritin and Troponin-T characterized KCG such as lower WBC and platelets (all p values<0,05). KDG received more frequently immunoglobulins (IVIG) and acetylsalicylic acid (ASA) (81,3% vs 66%;p=0.04 and 71,9% vs 43,4%;p=0.001 respectively) as KCG more often received glucocorticoids (56,6% vs 14,6%;p<0.0001). SARS-CoV-2 assay more often resulted positive in KCG than in KDG (75,5% vs 20%;p<0.0001). Short-term follow data on KCG showed minor complications while on KDG a majority of patients had persistence of CAA. Comparing KDG with a KD-Historical Italian cohort (598 patients), no statistical difference was found in terms of clinical manifestations and laboratory data between the two groups Conclusion: Our study would suggest that SARS-CoV-2 infection might determine two distinct inflammatory diseases in children: KD, possibly triggered by SARS-CoV-2, and PIMS-TS. Older age at onset and clinical peculiarities, like the occurrence of myocarditis, characterize this multiinflammatory syndrome. Our patients had an optimal response to treatments and a good outcome, with few complications and no deaths.