Recently, the American College of Rheumatology (ACR) created two new task forces to address concerns for pediatric patients during the COVID-19 pandemic. The first task force is the COVID-19 Pediatric Rheumatology Clinical Guidance Task Force and the other is the Multi-System Inflammatory Syndrome in Children (MIS-C) and COVID-19 Related Hyperinflammation Task Force.
On June 18, 2020, both task forces have released new clinical guidance and recommendations for the care of pediatric patients in the context of COVID-19. All recommendations are based on current knowledge and are expected to be updated as new scientific evidence is found and published.
Clinical Guidance for Pediatric Patients with Rheumatic Disease
In this guidance, the Task Force recommends routine ophthalmologic surveillance of patients who are at high risk for chronic uveitis, or with a history of uveitis. Those patients should continue on schedule via in-person visits with slit lamp examination. Additionally, children with rheumatic disease should continue their routine childhood vaccinations (unless contraindicated) including the flu vaccine.
Medications
For pediatric patients who do not have COVID-19 exposure or infection, they may continue or begin treatment regiments with NSAIDs, hydroxychloroquine (HCQ), angiotensin-converting enzyme inhibitor (ACEi)/ angiotensin II receptor blocker (ARBs), colchicine, conventional DMARD (CDMARD), biologic DMARDs (bDMARDs) and targeted synthetic DMARDs (tsDMARDs). Glucocorticoids may be continued or initiated, using the lowest dose possible to control underlying disease.
Additionally, pediatric patients without COVID-19 exposure or infection that have life and/or organ threatening manifestations, high dose oral or intravenous “pulse” glucocorticoids and cyclophosphamide may be initiated to control underlying disease.
For pediatric patients who have close or household exposure to COVID-19, initiation of high dose oral or intravenous glucocorticoids should be delayed for 1-2 weeks, if deemed safe by the treating provider, for pediatric patients with non-life and/or organ threatening manifestations. For those patients with life and/or organ threatening manifestations, the initiation of high dose oral or intravenous glucocorticoids should not be delayed.
Patients with ongoing treatment and an asymptomatic COVID-19 infection, NSAIDs, HCQ, colchicine, cDMARDs, bDMARDs, tsDMARDs, cyclophosphamide or rituximab may be continued, if necessary, to control underlying disease.
Pediatric patients with a probable or confirmed COVID-19 infection should temporarily delay or withhold cDMARDs, bDMARDs (except IL-1 and IL-6 inhibitors), and tsDMARDs, and IL-1 and IL-6 inhibitors may be continued, if necessary, to control underlying disease.
Clinical Guidance for Pediatric Patients with Multisystem Inflammatory Syndrome in Children (MIS-C) Associated with SARS-CoV-2 and Hyperinflammation in COVID-19
Under this guidance, direction is provided on diagnostic evaluation of MIS-C as are general recommendations for cardiac management, immunotherapy treatment, and anti-blood clotting therapies in MIS-C.
The guidance notes that MIS-C and Kawasaki Disease unrelated to COVID-19 infections may share overlapping clinical features, including conjunctival injection, oropharyngeal findings (red and/or cracked lips, strawberry tongue), rash, swollen and/or erythematous hands and feet, and cervical lymphadenopathy.
Guidance Recommendations
For cardiac management, EKGs should be performed at a minimum of every 48 hours in MIS-C patients who are hospitalized, as well as during follow-up visits.
For anti-blood clotting therapy, the guidance recommends treatments of daily, low dose aspirin, of no more than 81 mg/day, be used in patients with MIS-C and Kawasaki Disease-like features and/or those with a high platelet count (≥450,000/𝜇L). This treatment should be continued until normalization of platelet count and confirmed normal coronary arteries at ≥4 weeks after diagnosis. Treatment with aspirin should be avoided in patients with a platelet count of ≤80,000/𝜇L.
Children with severe respiratory symptoms due to COVID-19 with any of the following should be considered for immunotherapy: acute respiratory distress syndrome, shock/cardiac dysfunction, elevated lactate dehydrogenase enzyme, D-dimer, IL-6, IL-2R, and/or ferritin, and depressed lymphocyte count, albumin, and/or platelet count. Glucocorticoids may be considered for use as immunomodulatory therapy in patients with COVID-19 and hyperinflammation.