Clinical Management of Pediatric Acute-Onset Neuropsychiatric Syndrome: Part II—Use of Immunomodulatory Therapies
Open Access
- 1 September 2017
- journal article
- review article
- Published by Mary Ann Liebert Inc in Journal of Child and Adolescent Psychopharmacology
- Vol. 27 (7), 574-593
- https://doi.org/10.1089/cap.2016.0148
Abstract
Introduction: Pediatric Acute-onset Neuropsychiatric Syndrome (PANS) is a clinically heterogeneous disorder with a number of different etiologies and disease mechanisms. Inflammatory and postinfectious autoimmune presentations of PANS occur frequently, with some clinical series documenting immune abnormalities in 75%–80% of patients. Thus, comprehensive treatment protocols must include immunological interventions, but their use should be reserved only for PANS cases in which the symptoms represent underlying neuroinflammation or postinfectious autoimmunity, as seen in the PANDAS subgroup (Pediatric Autoimmune Neuropsychiatric Disorders associated with Streptococcal infections). Methods: The PANS Research Consortium (PRC) immunomodulatory task force is comprised of immunologists, rheumatologists, neurologists, infectious disease experts, general pediatricians, psychiatrists, nurse practitioners, and basic scientists with expertise in neuroimmunology and PANS-related animal models. Preliminary treatment guidelines were created in the Spring of 2014 at the National Institute of Health and refined over the ensuing 2 years over conference calls and a shared web-based document. Seven pediatric mental health practitioners, with expertise in diagnosing and monitoring patients with PANS, were consulted to create categories in disease severity and critically review final recommendations. All authors played a role in creating these guidelines. The views of all authors were incorporated and all authors gave final approval of these guidelines. Results: Separate guidelines were created for the use of immunomodulatory therapies in PANS patients with (1) mild, (2) moderate-to-severe, and (3) extreme/life-threatening severity. For mildly impairing PANS, the most appropriate therapy may be “tincture of time” combined with cognitive behavioral therapy and other supportive therapies. If symptoms persist, nonsteroidal anti-inflammatory drugs and/or short oral corticosteroid bursts are recommended. For moderate-to-severe PANS, oral or intravenous corticosteroids may be sufficient. However, intravenous immunoglobulin (IVIG) is often the preferred treatment for these patients by most PRC members. For more severe or chronic presentations, prolonged corticosteroid courses (with taper) or repeated high-dose corticosteroids may be indicated. For PANS with extreme and life-threatening impairment, therapeutic plasma exchange is the first-line therapy given either alone or in combination with IVIG, high-dose intravenous corticosteroids, and/or rituximab. Conclusions: These recommendations will help guide the use of anti-inflammatory and immunomodulatory therapy in the treatment of PANS.Keywords
This publication has 109 references indexed in Scilit:
- Effect of celecoxib add-on treatment on symptoms and serum IL-6 concentrations in patients with major depressive disorder: Randomized double-blind placebo-controlled studyJournal of Affective Disorders, 2012
- Psychotropic effects of antimicrobials and immune modulation by psychotropics: implications for neuroimmune disordersNeuropsychiatry, 2012
- American College of Rheumatology guidelines for screening, treatment, and management of lupus nephritisArthritis Care & Research, 2012
- Clinical experience and laboratory investigations in patients with anti-NMDAR encephalitisThe Lancet Neurology, 2011
- Maternal history of autoimmune disease in children presenting with tics and/or obsessive–compulsive disorderJournal of Neuroimmunology, 2010
- Executive and Attention Functioning Among Children in the PANDAS SubgroupChild Neuropsychology, 2009
- Cyclooxygenase Inhibition Limits Blood-Brain Barrier Disruption following Intracerebral Injection of Tumor Necrosis Factor-α in the RatThe Journal of pharmacology and experimental therapeutics, 2007
- Risks associated with the use of intravenous immunoglobulinTransfusion Medicine Reviews, 2003
- Cognitive functioning in sydenham's chorea: Part 2. executive functioningDevelopmental Neuropsychology, 1994
- Cognitive functioning in sydenham's chorea: Part 1. attentional processesDevelopmental Neuropsychology, 1994