ABSTRACT
Background: The study of the features of the course and mutual influence of the new coronavirus disease COVID-19 and various rheumatic diseases (RD) in children can still give us new lessons, warnings and fears. Objectives: To update the analysis in a retrospective study the course of covid-19 in children with RD based on the results of two years of the pandemic. To analyze the impact of COVID-19 on the course of RD in children. Methods: Retrospective analysis based on data from single center. The study included patients with RD and confirmed COVID-19 for 2 years (2020-2021). Results: Were registered 320 cases of COVID-19 in children with RD. 158 (49%) patients were asymptomatically infected, 162 (51%) had clinical symptoms. A detailed description of the groups is presented in Table 1. Clinical symptoms were fever (67%), anosmia (47%), rhinitis (34%), cough (19%), arthralgia/myalgia (16%), dyspepsia (5%), rash (2.5%), pneumonia (3%). In the majority of cases (98%), COVID-19 proceeded in mild to moderate severity. Hospitalization due to COVID-19 was required just in 5 cases. 2 children were admitted to the intensive care unit. First, an 11-year-old girl with sJIA with the history of recurrent episodes of MAS resolved by regular administration of canakinumab. 2nd, a 12-year-old girl with Sjogren's syndrome, who received Rituximab and 1 month later she developed the COVID-19 with MIS-like clinical picture, pneumonia with 25%CT lessions. Both cases have a favorable outcome. The clinical characteristics of the patients are presented in Table 1. COVID-19 didǹt affect the course of RD in the 86% of pts. However, 15% developed a fare of the RD with average of 3 months after COVID-19. In this group for 13 pts (girls mostly F/M-9/4, mean age 15 years [9;16]), COVID-19 triggered the new onset of RD (non-systemic JIA-4, Uveitis-1, non-bacterial osteomyelitis-3, systemic JIA-2, Scleroderma-2, Sjögren's syndrome-1. 12 from 13 these children had clinical symptoms on COVID-19. Whether this is Long-COVID syndrome or an independent RD is not known. Conclusion: Our study suggests that the new coronavirus infection in most cases in children with RD, had mild or asymptomatic course, regardless of therapy with immunosuppressive drugs and bDMARD, except of 1 observation with the previous therapy of Rituximab. Worsening of RD after coronavirus infection developed in 15% of cases, regardless of its clinical manifestations. In 13 patients, the RD were started just after COVID-19. The explosive increasing of the incidence of a new strain of COVID-19 for a past month may change the current results and conclusions.
ABSTRACT
Background: Primary Sjögren's syndrome (pSS) is a chronic and progressive multisystem autoimmune disease which rarely onset in children and adolescents. Diagnostic delay in large part of patients are common due to the non-specifc and variable symptoms and the slow progression of disease. Objectives: To analyse demographic data, specifc extraglandular, salivary and ocular manifestations, laboratory parameters and therapy of pSS with juvenile onset. Methods: Retrospective study of all patients (pts) with pSS in single center. Results: pSS was verifed in 15 pts (6.7% were boys), which amounted to 23.8% of all pts with SS in our pediatric rheumatologic department. The median age of pSS onset was 8.0 y.o. [IQR 7.0;10.2]. The median of disease duration at the time of pSS verifcation was 2.75 years [2.2;5.6]. All patients had systemic manifestations at onset: constitutional abnormalities-33.3%, nonerosive polyarthritis-64.3%, polyarthralgias-26.7%, lymphadenopathy-73.3%, cutaneous involvement-53.3% (2-xerosis, 2-annular erythema, 1-erythema nodo-sum, 2-Raynaud phenomenon, 2-nonspecifc spotty rashes, 1-hemorrhagic rash). At the time of diagnosis 7 pts (46.7%) had isolated involvement of salivary glands, 8 pts (53.3%)-combined with involvement of lacrimal glands. The decrease in salivary gland function was recorded in 80% of cases, hypolacrimia-in 46.7%, 1 patient had isolated hypolacrimia. Recurrent parotitis was present in 6 pts (40.0%). At time of diagnosis pulmonary involvement had 20.0% of pts, 1 patient had renal tubular acidosis. 8 pts (53.3%) had various hematological disorders: anemia-in 3 pts (20.0%), leukopenia-in 6 (40.0%). ANA Hep-2 were detected in 100% pts (in titer 1/640-4, 1/1280-7, 1/2560-3, 1/20480-1, with mixed patterns in all pts: speckled + homogeneous-9 pts, speckled + homogeneous+cytoplasmic-6 pts), anti-Ro-in 12 pts (80.0%), anti-La-in 8 pts (53.3%), RF+-in 9 pts (60.0%). 6 pts (40.0%) had polyclonal hypergammaglob-ulinemia, max 42%. 2 pts (13.3%) had concomitant autoimmune non-rheumatic disease;1-cutaneous psoriasis, 1-autoimmune thyroiditis. The treatment of each patient was justifed by the main individual manifestations: 93.3% received glucocorticoids, 26.7%-methotrexate, 33.3%-hydroxychloroquine, 6.7%-mycophenolate mofetil. Treatment with biologics (B) was received by 13 (93.3%) pts (7-rituximab (RTM), 6-abatacept (ABA)) with a good response in 10 pts, including improvement in the function of the salivary and lacrimal glands in 7 pts. 1 patient received 2B-RTM and ABA sequentially due to the development of MAS 7 days after 1st RTM infusion. B was discontinued in 3 pts: 1 due to development of hemorrhagic vasculitis 2 days after the 1st RTM infusion, 1-COVID-19 with lung involvement (CT 3-4) 2 weeks after the 1st RTM infusion, 1-inefficiency of ABA during 15 months. Conclusion: In our pediatric rheumatologic department pts with pSS made up less than a quarter of all pts with SS. The diagnosis was verifed delayed in all pts, which can be explained by a wide range of nonspecifc manifestations at the onset. However, the manifestations of SS that were present at the time of diagnosis were brought under control on the background of complex therapy, including the prescription of B, with a good efficacy and safety profile of therapy.