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Frontiers in Pediatrics ; 10:1012582, 2022.
Article in English | MEDLINE | ID: covidwho-2199087


Multisystem inflammatory syndrome in children (MIS-C) is a serious condition triggered by SARS-COV-2 infection, characterized by persistent fever, multiorgan dysfunction, and increased inflammatory markers. It requires hospitalization and prompt treatment, with nearly 60% of the cases needing intensive care and 2% fatality rate. A wide spectrum of clinical characteristics and therapeutic approaches has been reported in MIS-C. We describe a series of four patients with MIS-C, defined according to the current case definitions, with a self-limiting course and no need for immunomodulatory treatment ("self-limiting MIS-C"). Few data about self-limiting MIS-C are available to date and no information on medium- and long-term outcome of this subset of patients has been reported. Although limited in size, our experience provides new insights into the MIS-C syndrome, highlighting an underestimated aspect of the disease that may have significant therapeutic implications.

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.

Pediatric Rheumatology ; 18(SUPPL 2), 2020.
Article in English | EMBASE | ID: covidwho-1029730


Introduction: During SARS-COV-2 pandemic, different reports have been published regarding children who developed hyperinflammatory syndrome with certain or probable relationship with SARS-COV-2. These patients presented incomplete or atypical manifestations of Kawasaki disease (KD), particularly abdominal pain, myocarditis and macrophage activation syndrome features. Objectives: To report a case of SARS-COV-2-related Kawasaki-like disease with severe cardiac involvement. Methods: case report description. Results: A 10-year-old previously healthy girl presented progressively worsening abdominal pain, high grade fever for 3 days and vomiting. Lab tests showed WBC 11680/mmc, N 9370/mmc, Creactive protein (CRP) 329 mg/L, procalcitonin (PCT) 0,74 ug/L, PT-INR 1,35 and elevated D-dimer and fibrinogen levels (817 ug/L and 9,45 g/L respectively). Abdomen ultrasound revealed lymphadenopathies and hyperechogenic mesentery in the right lower quadrant, although the appendix was not visualized. She underwent laparoscopy showing moderate quantity of free fluid and appendectomy was performed. Thereafter she continued to complain of high-grade fever, abdominal pain and diarrhoea, despite broad-spectrum antibiotics. Blood, urine and stool cultures were negative. Bilateral non-exudative conjunctivitis was present. Moreover, the lab tests showed persistent marked elevation of CRP (370 mg/L), WBC 15590/mmc, N 14070/mmc, hypoalbuminemia (23 g/L), elevated ferritin and triglycerides (458 ug/L and 221 mg/dl). By taking into consideration the concomitant SARS-COV-2 pandemic, nasopharyngeal and rectal swabs were taken with negative results. Conversely, serological test showed anti-SARS-COV-2 IgG antibodies and absence of IgM. The family medical history showed that the mother had presented fever, cough, ageusia and anosmia one month before, preceded by a contact with a SARS-COV-2 positive case, while the patient was asymptomatic at that time. Suspecting a KD-like disease she was referred to our Paediatric Rheumatology Unit: cardiological assessment revealed negative Twaves in V4-V5-V6 on EKG while standard and advanced echocardiography showed mild mitral and tricuspid insufficiencies, mild dilatation of the left main coronary artery (LMCA, z score +2), normal global function (FEVS 2D 58%) but reduced longitudinal strain (GLS-16%). Lab tests confirmed myocardial injury with troponin (TnI) 100,1 ng/l and brain natriuretic peptide (P-BNP) 593 ng/L. A single infusion of intravenous immunoglobulin 2 g/kg associated with methylprednisolone (1 mg/kg/day) led to a rapid clinical improvement with apyrexia and resolution of abdominal pain and conjunctivitis. Blood test confirmed gradual normalization of inflammatory markers, ferritin, troponin and BNP and EKG showed positive T-waves. Shortly after the discharge, while she was on prednisone 0.5 mg/kg/day and acetylsalicylic acid 100 mg/day, she referred some episodes of heart pounding, lasting about ten minutes with spontaneous resolution. Three weeks after onset, cardiac MRI was normal, however, speckle tracking echocardiography showed persistent dilatation of LMCA and reduction of global longitudinal strain (GLS-14%). 24-hour EKG-Holter detected episodes of supraventricular tachycardia and several ventricular and supraventricular extrasystoles. Thus, oral atenolol therapy was started. Conclusion: In our patient SARS-COV-2 induced a possible postinfectious antibody or immune-complex mediated reaction that led to KD-like disease with acute surgical abdomen presentation and persistent myocardial damage and arrythmias. Speckle tracking echocardiography appears more reliable than MRI in early detection of myocardial damage in patients with preserved left ventricular ejection fraction.

Pediatric Rheumatology ; 18(SUPPL 2), 2020.
Article in English | EMBASE | ID: covidwho-1029414


Introduction: Macrophage activation syndrome (MAS) is a rare, potentially life-threatening complication of some rheumatologic diseases1. Objectives: We report the case of a child with systemic onset Juvenile Idiopathic Arthritis (sJIA) complicated by severe MAS and acute myocarditis, needing veno-arterial Extracorporeal Membrane Oxygenation (VA-ECMO), successfully rescued by high dose intravenous Anakinra (HDIV-ANA). Methods: Case report's description Results: A two-year-old boy presented with one month history of fever associated with limping gait, cervical lymphadenopathy and skin rash. Laboratory tests showed elevation of inflammatory markers and ferritin. By exclusion criteria, sJIA was diagnosed and steroid therapy started. After a soft tissue bacterial infection, fever relapsed and laboratory tests were consistent with MAS (day 1): Hb 8.5 g/dL, PLT 44000/mm3;FDP 1522 ug/L, CRP 100 mg/L, ferritin 2200 ug/L. High doses intravenous metilprednisolone and oral Cyclosporin A (CSA) were started. On day 2 he presented a Systemic Capillary Leak Syndrome and acute myocarditis. He was admitted into the pediatric intensive care unit (PICU) where intravenous immunoglobulin and subcutaneous Anakinra (ANA) were added. On day 4, due to an episode of cardiac arrest, VA-ECMO was started and we tried high dose intravenous ANA (HDIV-ANA, 8 mg/Kg/day q6h). This treatment brought immediate benefit: echocardiography showed progressive resolution of myocarditis so that VA-ECMO was definitely weaned off in six days. Laboratory test showed isolated neutropenia (PMNs 0-100/mm3). Suspecting a iatrogenic cause, HDIV-ANA was gradually reduced to the maintenance dose without benefit. On day 22, ANA was stopped and neutropenia resolved. Analysis of PRF1 gene revealed a mutation (c.[272C>T] p.[Ala91Val]) in heterozygosis. 49 days after admission he was discharged on oral prednisone and CSA. Neither neurological nor other organ consequences related to MAS were reported. A few months later, on tapering down of therapy, he relapsed. ANA was restarted with rapid improvement and no side effects, including neutropenia. Currently, after 12 months, the disease is in clinical remission on medication. Conclusion: MAS is a rare life-threatening complication of sJIA, triggered by infections in up to one-third of the patients 2. It is the result of a cytokine storm that lead to a dysregulated inflammatory activation of the immune system, with rapid progression to multiorgan failure. Treatment usually includes high dose corticosteroids and immunosuppressive agents. Recently, the use of selective cytokine inhibitors has been suggested. No standardized guidelines are available to date, but the use of ANA has been already reported, pointing out the need for a higher doses regimen in refractory cases. MAS in our patient appeared after a soft tissue infection which could have act as triggering factor in a patient with sJIA and genetic predisposing pattern. The choice of intravenous administration of ANA was partly due to the generalized edema and partly to the severe discoaugulopathy. Considering the higher doses needed for rapidly suppressing the cytokine storm and ANA pharmacokinetics, we split the daily dose into four administrations. No major adverse events were reported, except for a transient neutropenia, already reported 6. Based on our experience, HDIV-ANA is a safe and effective treatment for refractory life-threatening sJIA-related MAS. This therapeutic approach may be also considered in the current pandemic COVID-19 emergency where recent evidence showed IL1-driven MAS-like complication triggered by SARS-COV-2 virus as predictor of bad outcome7.