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3.
Semin Arthritis Rheum ; 55: 152047, 2022 08.
Article in English | MEDLINE | ID: covidwho-1882505

ABSTRACT

OBJECTIVES: Coronavirus disease 2019 (COVID-19) caused by SARS-CoV-2, has opened a new era in the practice of pediatric rheumatology since it has been associated with inflammatory complications such as vasculitis and arthritis. In this review, we aimed to present a detailed analysis of COVID-19 associated pediatric vasculitis. METHODS: A systematic review of the English literature was performed through Pubmed/MEDLINE and Scopus up to January 1st, 2022. Articles including data about the patients with 1) onset of vasculitis <18 years of age, 2) evidence of SARS-CoV-2 exposure, 3) evidence of vasculitis diagnosis (imaging, histopathologic evidences or fulfilling the specific diagnostic/classification criteria) were included in the final analysis. Patients with Kawasaki disease-like vasculitis associated with multisystem inflammatory syndrome in children (MIS-C) were excluded. RESULTS: A total of 25 articles describing 36 patients with COVID-19 associated pediatric vasculitis (median age 13 years; M/F: 2.3) were included. The most frequent phenotype was IgA vasculitis (n=9) followed by chilblains (n=7) and ANCA associated vasculitis (AAV) (n=5). Skin (58.3%) and renal (30.5%) involvements were the most common manifestations of vasculitis. The majority of patients received corticosteroids (40%), while rituximab (14.2%) and cyclophosphamide (11.4%) were the most frequently used immunosuppressive drugs. Remission was achieved in 23 of 28 patients. Five patients (4 with central nervous system vasculitis; 1 with AAV) died. CONCLUSION: Although COVID-19 associated pediatric vasculitis is very rare, awareness of this rare entity is important to secure earlier diagnosis and treatment. The clinical features of COVID-19 associated pediatric vasculitis subtypes look similar to those in pediatric vasculitis not associated with COVID-19. Whether COVID-19 is the reason of the vasculitis or only the trigger remains unknown.


Subject(s)
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis , COVID-19 , COVID-19/complications , Child , Humans , Rituximab , SARS-CoV-2 , Systemic Inflammatory Response Syndrome
4.
Ann Rheum Dis ; 81(5): 601-613, 2022 05.
Article in English | MEDLINE | ID: covidwho-1784782

ABSTRACT

OBJECTIVE: Autoinflammatory type I interferonopathies, chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature/proteasome-associated autoinflammatory syndrome (CANDLE/PRAAS), stimulator of interferon genes (STING)-associated vasculopathy with onset in infancy (SAVI) and Aicardi-Goutières syndrome (AGS) are rare and clinically complex immunodysregulatory diseases. With emerging knowledge of genetic causes and targeted treatments, a Task Force was charged with the development of 'points to consider' to improve diagnosis, treatment and long-term monitoring of patients with these rare diseases. METHODS: Members of a Task Force consisting of rheumatologists, neurologists, an immunologist, geneticists, patient advocates and an allied healthcare professional formulated research questions for a systematic literature review. Then, based on literature, Delphi questionnaires and consensus methodology, 'points to consider' to guide patient management were developed. RESULTS: The Task Force devised consensus and evidence-based guidance of 4 overarching principles and 17 points to consider regarding the diagnosis, treatment and long-term monitoring of patients with the autoinflammatory interferonopathies, CANDLE/PRAAS, SAVI and AGS. CONCLUSION: These points to consider represent state-of-the-art knowledge to guide diagnostic evaluation, treatment and management of patients with CANDLE/PRAAS, SAVI and AGS and aim to standardise and improve care, quality of life and disease outcomes.


Subject(s)
Autoimmune Diseases of the Nervous System , Nervous System Malformations , Rheumatology , Skin Diseases , Erythema Nodosum , Fingers/abnormalities , Humans , Quality of Life
5.
Pediatr Res ; 2022 Mar 29.
Article in English | MEDLINE | ID: covidwho-1768802

ABSTRACT

BACKGROUND: Coronavirus disease 19 (COVID-19) may have a severe course in children. Multisystem inflammatory syndrome in children (MIS-C) is the post-COVID complication characterized by an exaggerated inflammation, observed in children. However, data on the underlying pathophysiology are sparse. We therefore aimed to assess the cytokine and chemokine profiles of children with MIS-C and compare these to life-threatening severe SARS-CoV-2 and healthy controls (HCs) to shed light on disease pathophysiology. METHODS: Samples of 31 children with MIS-C, 10 with severe/critical COVID-19 and 11 HCs were included. Cytokine and chemokine profiles were studied and compared in between groups. RESULTS: Most cytokines and chemokines related to IL-1 family and IFN-γ pathway (including IL-18 and MIG/CXCL9) and IL-17A were significantly higher in the MIS-C group when compared to the severe/critical COVID-19 group and HCs. IP-10/CXCL10 and IL-10 were higher in both MIS-C patients and severe/critical COVID-19 compared to HCs. CONCLUSION: Our results suggest that IL-1 and IFN-γ pathways play an important role in the pathophysiology of MIS-C. IMPACT: This study defines a pattern of distinctive immune responses in children with MIS-C and in patients with severe/critical COVID-19. As the COVID-19 pandemic continues, biomarkers to identify MIS-C risk are needed to guide our management that study results may shed light on it.

6.
J Pediatr ; 245: 213-216, 2022 06.
Article in English | MEDLINE | ID: covidwho-1696725

ABSTRACT

Neurologic complications have been associated with multisystem inflammatory syndrome in children, possibly involving autoimmune mechanisms. Here, we report a 6-year-old girl who developed myasthenia 11 weeks after severe acute respiratory syndrome coronavirus 2 infection and 8 weeks after the onset of severe multisystem inflammatory syndrome in children.


Subject(s)
COVID-19 , COVID-19/complications , Child , Female , Humans , Systemic Inflammatory Response Syndrome
7.
Eur J Paediatr Neurol ; 37: 139-154, 2022 Mar.
Article in English | MEDLINE | ID: covidwho-1693668

ABSTRACT

BACKGROUND: Since December 2019, coronavirus disease 2019 (COVID-19) has become a global pandemic caused by highly transmissible severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). Although respiratory disease and multisystem inflammatory syndrome in children (MIS-C) are main clinical presentations in children, numerous neurological manifestations are being described increasingly. We aimed to investigate new onset neurological symptoms associated with SARS-CoV-2 in pediatric patients in order to establish a possible relationship as well as to understand the underlying pathophysiological mechanisms between SARS-CoV-2 infection and neurological findings. METHODS: We analyzed retrospectively children who had neurologic manifestations temporally associated with SARS-CoV-2 infection at Hacettepe University Ihsan Dogramaci Children's Hospital. We performed a literature search between March 20, 2020 and March 30, 2021. Articles that report children with COVID-19 related neurological manifestations were included. RESULTS: We have observed 15 consecutive cases with new onset neurological manifestations along with confirmed SARS-CoV-2 infection. Age at hospitalization ranged from three months to 17 years. Ten patients had central nervous system involvement, and most common manifestation was encephalopathy (5/10), which is also one of the most common manifestations of the patients mentioned in the relevant 39 articles we reviewed. CONCLUSION: Children with COVID-19 can present with neurologic findings such as encephalopathy, seizures, cerebrovascular events as well as abnormal eye movements. Clinical suspicion and awareness are required to show the association between neurologic manifestations and COVID-19.


Subject(s)
COVID-19 , Nervous System Diseases , COVID-19/complications , Child , Humans , Infant , Nervous System Diseases/epidemiology , Nervous System Diseases/etiology , Retrospective Studies , SARS-CoV-2 , Systemic Inflammatory Response Syndrome , Turkey/epidemiology
8.
J Clin Apher ; 37(3): 281-291, 2022 Jun.
Article in English | MEDLINE | ID: covidwho-1689485

ABSTRACT

BACKGROUND: Multisystem inflammatory syndrome in children (MIS-C) can be life threatening in severe cases because of uncontrolled inflammation and multi-organ failure. In this study, we report the effect of plasma exchange in the treatment of MIS-C and to emphasize the effect of its early application on outcome. METHOD: In this retrospective observational study, the medical records of children with severe MIS-C admitted to pediatric intensive care unit (PICU) between April 2020 and January 2021 were reviewed. Severe MIS-C patients were treated according to protocol consisting of plasma exchange (PE), intravenous immune globulin, steroids, and anakinra which we called the "PISA" protocol referring to the initials. The patients were divided into two groups as early plasma exchange (E-PE) and late plasma exchange (L-PE) according to the elapse time between hospital admission and the administration of PE. Groups were compared in terms of outcome variables. Primary study outcome was 28-day mortality. Secondary outcome variables were acute phase response time, length of immunomodulatory treatment, frequency of patients requiring mechanical ventilation (MV) and inotropic support, length of inotropic support and MV, length of hospital and PICU stays. RESULTS: Eighteen pediatric patients with MIS-C were included in the study. Seventeen (95%) of the patients presented with decompensated shock and required inotropic support. One of the 17 patients needed extracorporeal membrane oxygenation support (ECMO) PISA protocol was used in all patients. There was no mortality in the E-PE group while the mortality rate was 20% in the L-PE group. Acute phase reactant response was faster in the E-PE group and immunomodulatory treatments could be reduced earlier; the frequency of patients requiring inotropic and mechanical ventilation (MV) support was lower in the E-PE group; the duration of inotropic support, duration of MV, and length of stay in hospital and PICU were significantly shorter in the E-PE group. CONCLUSION: We suggest that in selected cases, timely administration of PE is a beneficial rescue therapy for MIS-C related hyperinflammation presenting with severe cardiovascular collapse.


Subject(s)
COVID-19 , SARS-CoV-2 , COVID-19/complications , Child , Humans , Plasma Exchange , Systemic Inflammatory Response Syndrome/therapy
9.
Mod Rheumatol ; 32(2): 467-472, 2022 Feb 28.
Article in English | MEDLINE | ID: covidwho-1569714

ABSTRACT

OBJECTIVE: To evaluate the course of coronavirus-19 (COVID-19) infection in paediatric familial Mediterranean fever (FMF) patients and to investigate the risk factors for COVID-19 infection. METHODS: Medical records of 100 consecutive paediatric FMF patients and their COVID-19 infection status were evaluated. Age- and gender-matched control group consisted of 51 patients with positive results for severe acute respiratory syndrome coronavirus 2. RESULTS: Twenty-five of 100 paediatric FMF patients were detected to have COVID-19 infection. A history of contact with a COVID-19 case was present in ∼95% of patients in both the FMF and control groups with COVID-19 infection. Asymptomatic infection was detected in two patients in the paediatric FMF group (8.0%) and 17 patients in the control group (33.3%) (P = .017). Mild disease was observed in 23 paediatric FMF patients (92.0%) and 28 control patients (54.9%) (P = .001), whereas moderate disease was present in only 6 control patients (11.7%) (0 vs 11.7%, P = .074). Severe or critical disease was not observed in any patients. CONCLUSION: Paediatric FMF patients receiving colchicine had no moderate COVID-19 disease compared to the control group. We suggest that colchicine use may tune down the severity of the disease even if it does not prevent COVID-19 infection.


Subject(s)
COVID-19 , Familial Mediterranean Fever , COVID-19/complications , Child , Colchicine/therapeutic use , Familial Mediterranean Fever/complications , Familial Mediterranean Fever/diagnosis , Familial Mediterranean Fever/drug therapy , Humans , SARS-CoV-2
10.
Mod Rheumatol ; 2021 Nov 30.
Article in English | MEDLINE | ID: covidwho-1546003

ABSTRACT

OBJECTIVES: Multisystem inflammatory syndrome in children (MIS-C) is a rare but severe condition associated with coronavirus disease 2019 (COVID-19). Here we aimed to raise awareness for the symptoms of MIS-C in patients with rheumatic diseases, emphasizing the challenges of the differential features. METHODS: We retrospectively evaluated the demographic and clinical characteristics, laboratory and imaging findings, treatments, and outcomes of six MIS-C patients with previous rheumatic disease. RESULTS: Three of the patients had familial Mediterranean fever (FMF), one had juvenile dermatomyositis (JDM), one had systemic juvenile idiopathic arthritis (JIA), and another patient had oligoarticular JIA. All FMF patients presented with fever and abdominal pain, two also had chest pain. The patient with systemic JIA presented with fever, rash, and myalgia. All patients had elevated inflammatory markers and high d-dimer levels. Chest imaging of two FMF patients showed infiltrations compatible with pneumonia. One FMF patient had mildly decreased systolic functions with a shortening fraction of 48% in his echocardiography. Intravenous immunoglobulin and methylprednisolone were administered to all patients. Anakinra was given to four patients. CONCLUSIONS: Clinical and laboratory signs of MIS-C may overlap with the findings of various rheumatic diseases, and this may cause a delay in diagnosis.

11.
Rheumatol Int ; 42(1): 51-57, 2022 01.
Article in English | MEDLINE | ID: covidwho-1491096

ABSTRACT

The impact of the COVID-19 pandemic, and implemented restrictions on the frequency of pediatric rheumatic diseases remain unknown, while they have probably prevented common infections in children. We present the effects of the COVID-19 on our pediatric rheumatology practice in a main referral center. We retrospectively reviewed the medical records of patients presenting to pediatric rheumatology department in 4 years before March 2020 and compared it to the pandemic year (March 2020-March 2021). Since there was an overall decrease in patient numbers, we calculated the percentage according to the total number of that year. A total of 32,333 patients were evaluated. The mean annual number of patients decreased by 42% during the COVID-19 pandemic. When follow-up visits (25,156) were excluded, there were 2818 new diagnoses of rheumatic diseases. In the pre-pandemic period, familial Mediterranean fever (FMF) (n = 695, 28.1%) was the most frequent, whereas in the pandemic period multisystem inflammatory syndrome in children (MIS-C) (n = 68, 19.2%) was the most common diagnosis. There were no significant differences in the percentages of juvenile idiopathic arthritis, autoimmune diseases, rare autoinflammatory diseases, and other vasculitides. However, there was a significant decrease in patients diagnosed with FMF, IgA vasculitis (IgAV), acute rheumatic fever (ARF), classic Kawasaki disease (KD), and macrophage activation syndrome (MAS) (all p < 0.05). During the pandemic year, the percentage of most common diseases did not differ. On the other hand, we suggest that the decreases in IgAV, KD (classic), and MAS, which parallels the decrease in ARF, confirm the role of infections in the pathogenesis for these diseases.


Subject(s)
COVID-19 , Rheumatic Diseases/epidemiology , Adolescent , Child , Child, Preschool , Female , Humans , Male , Pandemics , Prevalence , Retrospective Studies
12.
Rheumatol Int ; 42(3): 469-475, 2022 03.
Article in English | MEDLINE | ID: covidwho-1439720

ABSTRACT

The effects of biological disease-modifying antirheumatic drugs (bDMARDs) in the clinical course of COVID-19 on children with underlying rheumatologic diseases have not been fully demonstrated. To evaluate the course of COVID-19 infection in patients with rheumatic disease receiving bDMARD treatment. This was a retrospective, multicenter study conducted in pediatric patients infected by SARS-CoV-2 and under bDMARDs therapy. The study population consisted of 113 patients (72 female/41 male). The mean age of the patients was 12.87 ± 4.69 years. The primary diagnosis of the cohort was as follows: 63 juvenile idiopathic arthritis, 35 systemic autoinflammatory diseases, 10 vasculitides, and five cases of connective tissue diseases. The mean duration of the primary disease was 4.62 ± 3.65 years. A total of 19 patients had additional comorbid diseases. Thirty-five patients were treated with canakinumab, 25 with adalimumab, 18 with etanercept, 10 with infliximab, nine with tocilizumab, six with rituximab, four with anakinra, three with tofacitinib, and one with abatacept. The median exposure time of the biological drug was 13.5 months. Seventy-one patients had symptomatic COVID-19, while 42 were asymptomatic. Twenty-four patients required hospitalization. Five patients presented with MIS-C. The hospitalized patients were younger and had a shorter duration of rheumatic disease compared to ambulatory patients, although the difference was not statistically significant. Steroid usage, presence of fever, and dyspnea were more common among the hospitalized patients. A worsening in the course of both COVID-19 and current disease was not noticed under bDMARDs, however, to end with a strong conclusion multicentric international studies are required.


Subject(s)
Antirheumatic Agents/therapeutic use , Biological Products/therapeutic use , COVID-19/complications , Rheumatic Diseases/complications , Adolescent , Child , Disease Progression , Female , Humans , Male , Retrospective Studies , Rheumatic Diseases/drug therapy
13.
Rheumatol Int ; 42(5): 879-889, 2022 May.
Article in English | MEDLINE | ID: covidwho-1400097

ABSTRACT

To compare the clinical and laboratory findings of multisystem inflammatory syndrome in children (MIS-C), patients with Kawasaki disease (KD) and with macrophage activating syndrome due to systemic juvenile idiopathic arthritis (sJIA-MAS) on real-life data. Patients diagnosed with MIS-C, KD, and sJIA-MAS from 12 different centers in Turkey who were followed for at least 6 months were included in the study. Demographic, clinical, and laboratory findings of all patients were analyzed. A total of 154 MIS-C, 59 KD, and 31 sJIA-MAS patients were included. The median age of patients with MIS-C were higher than those with KD while lower than those with sJIA-MAS (8.2, 3, 12 years, respectively). Myalgia (39.6%), cardiac (50.6%), gastrointestinal (72.7%), and neurological (22.1%) involvements were more common in patients with MIS-C compared to others. MIS-C patients had lower levels of lymphocyte (950 vs 1700 cells/µl) and thrombocyte (173,000 vs 355,000 cells/µl) counts and higher pro-BNP (1108 vs 55 pg/ml) levels than KD. Ferritin levels were higher in patients with MIS-C compared to patients with KD while they were lower than patients with sJIA-MAS (440, 170, 10,442 ng/ml, respectively). Patients with MIS-C had a shorter duration of hospitalization than sJIA-MAS (p = 0.02) while they required intensive care unit admission more frequently (55 vs 8 patients, p < 0.001). The median MAS/sJIA score of MIS-C patients was - 1.64 (- 5.23 to 9.68) and the median MAS/sJIA score of sJIA-MAS patients was -2.81 ([- 3.79] to [- 1.27]). MIS-C patients displayed certain differences in clinical and laboratory features when compared to KD and sJIA-MAS. Definition of the differences and similarities between MIS-C and the other intense inflammatory syndromes of childhood such as KD and MAS will help the clinicians while making timely diagnosis.


Subject(s)
Arthritis, Juvenile , Macrophage Activation Syndrome , Mucocutaneous Lymph Node Syndrome , Arthritis, Juvenile/complications , Arthritis, Juvenile/diagnosis , Biomarkers , COVID-19/complications , Child , Ferritins , Humans , Macrophage Activation Syndrome/diagnosis , Macrophage Activation Syndrome/etiology , Macrophages , Mucocutaneous Lymph Node Syndrome/complications , Mucocutaneous Lymph Node Syndrome/diagnosis , Systemic Inflammatory Response Syndrome
15.
Pediatr Nephrol ; 36(11): 3771-3776, 2021 11.
Article in English | MEDLINE | ID: covidwho-1237500

ABSTRACT

BACKGROUND: The rising number of infections due to Severe Acute Respiratory Syndrome Coronavirus-2 (popularly known as COVID-19) has brought to the fore new antiviral drugs as possible treatments, including favipiravir. However, there is currently no data regarding the safety of this drug in patients with kidney impairment. The aim of this paper, therefore, is to share our experience of the use of favipiravir in pediatric patients affected by COVID-19 with any degree of kidney impairment. METHODS: The study enrolled pediatric patients aged under 18 years and confirmed as suffering from COVID-19 and multisystem inflammatory syndrome in children (MIS-C) with any degree of kidney injury, who were treated with favipiravir at the time of admission. RESULTS: Out of a total of 11 patients, 7 were diagnosed with MIS-C and 4 with severe COVID-19. The median age of the cases was 15.45 (9-17.8) years and the male/female ratio was 7/4. At the time of admission, the median serum creatinine level was 1.1 mg/dl. Nine patients were treated with favipiravir for 5 days, and 2 patients for 5 days followed by remdesivir for 5-10 days despite kidney injury at the time of admission. Seven patients underwent plasma exchange for MIS-C while 2 severely affected cases underwent continuous kidney replacement therapy (CKRT) as well. One severe COVID-19 patient received plasma exchange as well as CKRT. Serum creatinine values returned to normal in mean 3.07 days. CONCLUSIONS: Favipiravir seems a suitable therapeutic option in patients affected by COVID-19 with kidney injury without a need for dose adjustment.


Subject(s)
Acute Kidney Injury/physiopathology , Amides/administration & dosage , COVID-19/complications , COVID-19/drug therapy , Pyrazines/administration & dosage , Renal Elimination , Systemic Inflammatory Response Syndrome/drug therapy , Acute Kidney Injury/drug therapy , Acute Kidney Injury/immunology , Acute Kidney Injury/virology , Adenosine Monophosphate/administration & dosage , Adenosine Monophosphate/analogs & derivatives , Adenosine Monophosphate/pharmacokinetics , Adolescent , Alanine/administration & dosage , Alanine/analogs & derivatives , Alanine/pharmacokinetics , Amides/pharmacokinetics , COVID-19/immunology , COVID-19/virology , Child , Creatinine/blood , Dose-Response Relationship, Drug , Drug Therapy, Combination , Female , Glomerular Filtration Rate , Humans , Male , Pyrazines/pharmacokinetics , SARS-CoV-2/isolation & purification , Systemic Inflammatory Response Syndrome/complications , Systemic Inflammatory Response Syndrome/immunology , Systemic Inflammatory Response Syndrome/virology , Treatment Outcome
17.
Clin Rheumatol ; 40(8): 3227-3237, 2021 Aug.
Article in English | MEDLINE | ID: covidwho-1083261

ABSTRACT

OBJECTIVE: We aimed to describe the typical clinical and laboratory features and treatment of children diagnosed with multisystem inflammatory syndrome in children (MIS-C) and to understand the differences as compared to severe/critical pediatric cases with COVID-19 in an eastern Mediterranean country. METHODS: Children (aged <18 years) who diagnosed with MIS-C and severe/critical pediatric cases with COVID-19 and were admitted to hospital between March 26 and November 3, 2020 were enrolled in the study. RESULTS: A total of 52 patients, 22 patients diagnosed with COVID-19 with severe/critical disease course and 30 patients diagnosed with MIS-C, were included in the study. Although severe COVID-19 cases and cases with MIS-C share many clinical and laboratory features, MIS-C cases had longer fever duration and higher rate of the existence of rash, conjunctival injection, peripheral edema, abdominal pain, altered mental status, and myalgia than in severe cases (p<0.001 for each). Of all, 53.3% of MIS-C cases had the evidence of myocardial involvement as compared to severe cases (27.2%). Additionally, C-reactive protein (CRP) and white blood cell (WBC) are the independent predictors for the diagnosis of MIS-C, particularly in the existence of conjunctival injection and rash. Corticosteroids, intravenous immunoglobulin (IVIG), and biologic immunomodulatory treatments were mainly used in MIS-C cases rather than cases with severe disease course. There were only three deaths among 52 patients, one of whom had Burkitt lymphoma and the two cases with severe COVID-19 of late referral. CONCLUSION: Differences between clinical presentations, acute phase responses, organ involvements, and management strategies indicate that MIS-C might be a distinct immunopathogenic disease as compared to pediatric COVID-19. Conjunctival injection and higher CRP and low WBC count are reliable diagnostic parameters for MIS-C cases. Key Points • MIS-C cases had longer fever duration and higher rate of the existence of rash, conjunctival injection, peripheral edema, abdominal pain, altered mental status, and myalgia than in severe/critical pediatric cases with COVID-19. • Higher CRP and low total WBC count are the independent predictors for the diagnosis of MIS-C. • MIS-C might be a distinct immunopathogenic disease as compared to pediatric COVID-19.


Subject(s)
COVID-19 , Child , Humans , Pandemics , SARS-CoV-2 , Systemic Inflammatory Response Syndrome , Turkey/epidemiology
18.
Semin Arthritis Rheum ; 50(6): 1262-1268, 2020 12.
Article in English | MEDLINE | ID: covidwho-872487

ABSTRACT

OBJECTIVES: The COVID-19 pandemic is a global health problem. We, as the EMERGE (EMErging RheumatoloGists and rEsearchers) group of PReS (Pediatric Rheumatology European Society) analyzed how the pandemic has affected pediatric rheumatology practice. METHODS: An online survey was developed to assess changes in pediatric rheumatology practice due to the pandemic. Results were analyzed using descriptive statistics. RESULTS: From 70 countries, 493 pediatric rheumatologists (80.3% in pediatric rheumatology practice for ≥5 years) responded to the survey. Around 70% disagreed that the pandemic led to reduced prescription of nonsteroidal anti-inflammatory drugs, conventional synthetic and biologic disease-modifying antirheumatic drugs. Almost half were more likely to taper corticosteroids faster. One-fifth hesitated to switch the major immunosuppressant during a flare. Patients encountering difficulties obtaining hydroxychloroquine and tocilizumab due to shortages were noted by 192 (38.9%) and 44 (8.9%), respectively. Twenty to 30% indicated that their patients had experienced a flare or delay in diagnosis/intervention due to postponed appointments.53% mentioned use of phone calls/smartphone applications while 47% shifted towards video consultations for patient care. Respondents indicated an increased number of patients with Kawasaki disease (30%), macrophage activation syndrome (15.6%), unusual vasculitic rashes (31.4%), and hyperinflammation (33.5%) during the pandemic. CONCLUSION: This is the largest survey to date addressing changes in pediatric rheumatology practice due to the COVID-19 pandemic. Primary changes were due to delays in clinic appointments, increase in use of virtual technologies, and concerns about the use of immunosuppressive therapies. An increased number of patients with Kawasaki disease/hyperinflammation mentioned by the respondents is noteworthy.


Subject(s)
COVID-19 , Practice Patterns, Physicians'/statistics & numerical data , Rheumatology/methods , Adult , Aged , Anti-Inflammatory Agents, Non-Steroidal/therapeutic use , Antirheumatic Agents/therapeutic use , Child , Cross-Sectional Studies , Female , Global Health , Humans , Hydroxychloroquine/therapeutic use , Male , Middle Aged , Pandemics , Pediatrics/methods , SARS-CoV-2 , Surveys and Questionnaires
19.
Rheumatol Int ; 40(12): 2105-2115, 2020 12.
Article in English | MEDLINE | ID: covidwho-763449

ABSTRACT

Children with Coronavirus disease 2019 (COVID-19) are being reported to have manifestations of hyperinflammatory states and/or Kawasaki-like disease. In this study, we investigated children with typical and atypical Kawasaki disease (KD) likely to be associated with COVID-19. We have reported four children with Kawasaki-like disease probably associated with COVID-19. The clinical features were consistent with incomplete KD in three patients. SARS-CoV-2 RT-PCR was positive in one and the serology was positive in one patient with negative RT-PCR. Corticosteroids, anakinra, intravenous immunoglobulin (IVIG), and acetylsalicylic acid were used in the treatment. Three patients recovered after the treatment while one patient died. The literature review revealed 36 articles describing 320 children with Kawasaki-like disease associated with COVID-19. SARS-CoV-2 RT-PCR was negative in 120 (65.5%) of 183 patients while the serology was positive in 130 (83.8%) of 155 patients. The therapeutic options have included IVIG, acetylsalicylic acid, tocilizumab, anakinra, enoxaparin, and methylprednisolone. Pediatric COVID-19 cases may present with atypical/incomplete Kawasaki-like disease. Thus, pediatricians need to be aware of such atypical presentations resembling KD for early diagnosis of COVID-19.


Subject(s)
Coronavirus Infections/complications , Mucocutaneous Lymph Node Syndrome/etiology , Pneumonia, Viral/complications , Betacoronavirus , COVID-19 , Child , Child, Preschool , Coronavirus Infections/diagnosis , Fatal Outcome , Female , Humans , Infant , Male , Mucocutaneous Lymph Node Syndrome/physiopathology , Mucocutaneous Lymph Node Syndrome/virology , Pandemics , Pneumonia, Viral/diagnosis , SARS-CoV-2
20.
Rheumatol Int ; 40(8): 1193-1213, 2020 08.
Article in English | MEDLINE | ID: covidwho-526706

ABSTRACT

COVID-19 (coronavirus disease 2019) pandemic caused by SARS-CoV-2, is a global public health issue threatening millions of lives worldwide. Although the infection is mild in most of the affected individuals, it may cause severe clinical manifestations such as acute respiratory distress syndrome or cytokine storm leading to death. Children are affected less, and most experience a milder disease. As rheumatologists, we deal with the uncontrolled response of the immune system, and most of the drugs we use are either immune modulators or immunosuppressants. Thus, the rheumatologists participate in the multidisciplinary management of COVID-19 patients. On the other hand, our patients with rheumatic diseases constitute a vulnerable group in this pandemic. In this review, a systematic literature search was conducted utilizing MEDLINE/PubMed and Scopus databases, and 231 COVID-19 patients with rheumatic diseases have been identified. Only one of these patients was a child. Among these, 9 (3.9%) died due to COVID-19. In light of the current data, the aspects of COVID-19 resembling rheumatic diseases, the possible reasons for why children are affected less severely, the hypothetic role of available vaccines in preventing COVID-19, the unique position of patients with rheumatic diseases in this pandemic, and the use of anti-rheumatic drugs in COVID-19 treatment are discussed.


Subject(s)
Betacoronavirus , Coronavirus Infections/drug therapy , Pneumonia, Viral/drug therapy , Rheumatic Diseases/drug therapy , BCG Vaccine/immunology , COVID-19 , Child , Coronavirus Infections/prevention & control , Female , Humans , Male , Measles-Mumps-Rubella Vaccine/immunology , Pandemics/prevention & control , Pneumonia, Viral/prevention & control , SARS-CoV-2
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