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Background: COVID-19 is a major challenge worldwide. Although the risk for a severe disease course is low among children with COVID-19, symptoms may be exacerbated by underlying disease and/or immunosuppressive medication. We analysed clinical data from COVID-19 cases in among pediatric patients with juvenile idiopathic arthritis (JIA) in Germany reported to the BIKER registry. Objectives: This is an analysis of clinical data for 56 COVID-19 cases reported to the German BIKER registry from 29 German pediatric rheumatology centers and clinics from February 2020 to January 2021. Methods: The major task of the German BIKER (Biologics in Paediatric Rheumatology) Registry is surveillance of biologics used in pediatric rheumatology patients. Following the start of the COVID-19 pandemic in Germany, a survey was established to proactively interview all participating centers regarding the occurrence, presentation and outcome of SARS-CoV-2-infected children with rheumatic diseases. Initially, the interviews were conducted in weekly intervals, later bi-weekly. A standardized Adverse Event of Special Interest form was developed requesting biographic data, pre-treatment, current medication, data on clinical presentation, course, treatment and outcome of COVID-19 pediatric rheumatology patients. Results: In all, 56 patients with JIA and SARS-CoV-2 infection were reported (Table 1). Of these patients, 71% were 12 or more years old. At the time of infection, 41% of the patients received conventional DMARDs and 52% received biologics (Table 1). Forty-four patients (79%) received either a conventional DMARD or a biologic. Most patients had a polyarticular course of their JIA (57%). In 49 of the 56 cases (88%) COVID-19 was detected directly by PCR (n=46), by antigen test only (n=1) or an undisclosed method (n= 2). Six patients had detectable SARS-CoV2 antibodies and reported to have had typical symptoms. One patient tested negative but developed typical symptoms at approximately the same time a positive SARS-CoV-2 test was returned for a family member. Symptoms were reported in 43 of the 56 patients (77%): fever n=15, rhinitis n=14, cough n=12, headache n=10, loss of sense of taste and/or smell n=9, pharyngitis n=8, fatigue n=5, musculoskeletal pain n=5, GI symptoms n=2 (abdominal pain n=1, diarrhoea n=1), dizziness n=3, encephalitis/seizure/respiratory failure/death n=1. Thirteen patients (23%) were asymptomatic. A 3-year-old female patient initially diagnosed with systemic JIA developed intracranial oedema and respiratory failure. Her SARS-CoV2 PCR test was positive and pulmonary imaging displayed typical changes in lung texture. Before her SARS-CoV-2 infection, the patient was treated with methotrexate and low-dose steroids. Unfortunately, she died three days following hospital admission. Genetic testing revealed an inborn immunodeficiency. Except for this one patient, all other cases were treated as outpatients and no deaths were reported. Conclusion: Apart from one patient with an inborn immunodeficiency who died from her COVID-19 infection, no case of hospitalization or severe COVID-19 was reported in our cohort of JIA patients. At the time of COVID-19 diagnosis, nearly 80% of patients in our cohort had been treated with conventional DMARD and/ or biologics. This seemed not to have a negative effect on severity or outcome of SARS-CoV2 infection.
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Background: Although children and adolescents are less likely to develop COVID-19 and generally show milder disease courses, it is unclear what impact the SARS-CoV2 infection has on children and adolescents with rheumatic and musculoskeletal disease (RMD). Due to their underlying disease as well as therapeutic immunosuppression these patients may be at higher risk of being more severely affected by SARS-CoV2. Furthermore, SARS-CoV2 infection might trigger a flare of the underlying disease. Objectives: To evaluate clinical characteristics and disease course of COVID-19 in children and adolescents with RMD and to analyze possible effects of SARSCoV2 infection on the underlying disease under different therapeutic regimens. Methods: Data from juvenile patients with RMD recorded via the SARS-CoV2 questionnaire within the National Pediatric Rheumatology Database and the registry for hospitalized children and adolescents with COVID-19 of the German Society for Pediatric Infectious Diseases were analyzed. In addition to age, sex and diagnosis, information was collected about the date and method of a positive SARS-CoV2 testing, reason for testing, on clinical manifestations, disease course, treatment and outcome of COVID-19, on drug therapy at the time of virus detection, on disease activity (NRS 0 -10, 0 = best) of the underlying disease at the last visit before and after the SARS-CoV2 infection. Results: From April 17th 2020 until January 25th 2021, data of 67 patients with RMD and confirmed SARS-CoV2 infection were collected. Mean age was 13.5 ± 3.9 years with equal sex distribution. The majority of patients were diagnosed with juvenile idiopathic arthritis (JIA, 64%), 12 (18%) patients had an autoinflammatory disease (FMF, CAPS, PFAPA, TRAPS) and 5 (7%) a connective tissue disease. Fifty-two patients (78%) were treated with a disease modifying antirheumatic drug (DMARD), 39% with a biological DMARD and 9% systemic glucocorticoids at the time of SARS-CoV-2 infection. Nineteen patients (28%) were tested for SARS-CoV-2 because of typical symptoms, the majority (67%) because of contact to an infected person. PCR was used most often (in 60 %). 52 patients (78%) developed symptoms of COVID-19, 15 patients remained asymptomatic. The most common symptom of COVID-19 was rhinitis (42%) and fever (38%), followed by fatigue (34%), taste/smell disorder (33%), sore throat (27%) and cough (23%). Disease severity was graded as mild in 44 of 52 (85%) symptomatic patients, only two patients were hospitalized, one of whom required intensive care and died of cardiorespiratory failure 3 days after symptom onset. In 22 of 26 (85%) SARS-CoV2-positive patients, no relevant increase in disease activity (difference in NRS ≤ 1 before/after infection) of the underlying disease was observed 31 days after symptom onset (median, IQR 17-52 days). One patient, who had paused tocilizumab for 2 doses, experienced a flare of his seronegative polyarthritis 2 months after asymptomatic SARS-CoV-2 infection. Conclusion: In our cohort, the clinical picture of COVID-19 in children and adolescents with RMD was similar to that of healthy peers. The majority of patients showed mild disease course with good outcome under various medications, however, one patient with a severe course of COVID-19 died. In addition, SARSCoV2 infection does not appear to have a relevant impact on the underlying disease activity, whereas discontinuation of therapy might pose a risk of flare.
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BACKGROUND: Reliable data on the course and treatment of pediatric COVID-19 ("corona virus disease 2019") in immunosuppressed patients with rheumatic diseases are missing. AIM: Delineation of individual strategies of the members of the Society for Pediatric Rheumatology (GKJR) in cases of COVID-19. METHODS: In May 2020 all GKJR members were invited to take part in an online survey. Opinion data regarding an approach using disease-modifying anti-rheumatic drugs (DMARD) in cases of COVID-19 as well as the readiness to use new therapeutic agents in patients in different stages of the disease were collected. RESULTS: A total of 71 respondents (27.3% of all contacted pediatric rheumatologists) took part in the survey. Of these 28.2% had treated patients with COVID-19. Over 95% of the respondents did not support a preventive adaptation of the anti-rheumatic treatment during the SARS-CoV2 pandemic. In the case of outpatients under immunosuppression with proven COVID-19 more than 50% of the respondents would refrain from administering intravenous high-dose steroids, cyclophosphamide, anti-CD20 antibodies as well as BAFF, CTLA4 and TNF-alpha blockades. Conversely, >70% of the respondents would continue the treatment with nonsteroidal anti-inflammatory drugs, hydroxychloroquine (HCQ), oral steroids, mycophenolate, IL1 blockade and immunoglobulins (Ig). In the case of inpatients 74.6% of respondents would consider targeted COVID-19 treatment. In stable patients with oxygen treatment (stage I) HCQ (18.3%), azithromycin (16.9%) and Ig (9.9%) were most frequently used. In cases of early signs (stage II) or a manifest cytokine storm (stage III) anakinra (40.8% for stage II and 46.5% for stage III), tocilizumab (26.8% and 40.8%, respectively), steroids (25.4% and 33.8%, respectively) and remdesivir (29.6% and 38.0%, respectively) were most frequently used. The need for a personalized approach based on the current clinical situation was emphasized by many respondents. CONCLUSION: The currently low prevalence of COVID-19 in Germany limits the general clinical experience. Therefore, the presented results have to be interpreted with caution and mostly as hypothetical treatment considerations. It is to be expected that there will always be a limited amount of evidence on pediatric COVID-19; therefore, a continuous and critical exchange of expert opinions on the treatment strategies is important.