TY - JOUR
T1 - Clinical management of pediatric acute-onset neuropsychiatric syndrome
T2 - Part II—use of immunomodulatory therapies
AU - Frankovich, Jennifer
AU - Swedo, Susan
AU - Murphy, Tanya
AU - Dale, Russell C.
AU - Agalliu, Dritan
AU - Williams, Kyle
AU - Daines, Michael
AU - Hornig, Mady
AU - Chugani, Harry
AU - Sanger, Terence
AU - Muscal, Eyal
AU - Pasternack, Mark
AU - Cooperstock, Michael
AU - Gans, Hayley
AU - Zhang, Yujuan
AU - Cunningham, Madeleine
AU - Bernstein, Gail
AU - Bromberg, Reuven
AU - Willett, Theresa
AU - Brown, Kayla
AU - Farhadian, Bahare
AU - Chang, Kiki
AU - Geller, Daniel
AU - Hernandez, Joseph
AU - Sherr, Janell
AU - Shaw, Richard
AU - Latimer, Elizabeth
AU - Leckman, James
AU - Thienemann, Margo
N1 - Funding Information:
R.C.D. has received speaker honoraria from Biogen Idec, Bristol-Myers-Squibb, is an editorial advisory board member for MSARD, an editorial board member for Neurology: Neu-roimmunology and Neuroinflammation and European Journal of Paediatric Neurology, received publishing royalties from Biogen and Bristol-Myers-Squibb, and research support from NHMRC, Multiple Sclerosis Research Australia. M.C. is Chief Scientific Officer at Moleculera Labs, a company offering antineuronal autoantibody testing for neuropsychiatric and movement disorders. R.B. has received speaking fees from Medac (less than $5000). G.B. has received research support from NIMH, National Science Foundation, and F. Hoffman-La Roche. K.C. (Disclosures 2014) is an unpaid consultant for GSK, Lilly, and BMS. He is on the DSMB for Sunovion. In the past 2 years, he has received research support from GSK and Merck. D.G. reports grant support from NIH and book honorarium from the American Academy of Child and Adolescent Psychiatry, speaking honoraria for Advanced Institute lectures from the American Academy of Child and Adolescent Psychiatry and Massachusetts General Hospital Psychiatry Academy in educational programs supported through independent medical education grants from pharmaceu- tical companies. Current funding is from Neurocrine Bioscience for clinical trial of Tourette’s disorder. Lifetime funding includes support from the Obsessive Compulsive Disorder Foundation, The Tourette Syndrome Association, The McIngvale Family Foundation, Eli Lilly, Pfizer, and Glaxo Smith Kline. J.L. reported receiving grant support from the National Institutes of Health, the UBS Optimus Foundation, and the Open Road Alliance. He serves pro bono on the advisory boards of the Brain and Behavior Research Foundation, Fondazione Child, the European Multicentre Tics in Children Studies, How I Decide, and Empathy for Peace. He receives book royalties from John Wiley and Sons, McGraw Hill, and Oxford University Press. T.M. has received research support from the following: NIH/NIMH: 1RO1MH093381-01A1, 1R21MH087849-01A1, 1R01HD080096-01A1, R34; Centers for Disease Control and Prevention: 5 U01DD000509-02, International OCD Foundation; AstraZeneca Pharmaceuticals; Sunovion Pharmaceuticals, Inc.; F. Hoffmann-LaRoche Ltd.; PANDAS Network; Neurocrine Biosciences, Inc.; Auspex Pharmaceuticals; Teva Pharmaceuticals; Shire Pharmaceuticals; Pfizer, Inc.; Massachusetts General Hospital; Psyadon Pharmaceuticals, Inc.; and Forest Research Institute, Inc. She has received travel support from the Tourette Syndrome Association and honoraria from grand rounds lectures. She also receives book royalties from Lawrence Erlbaum, Inc. and Taylor & Francis. All other authors have no competing financial interests.
Publisher Copyright:
© Jennifer Frankovich et al. 2017.
PY - 2017/9
Y1 - 2017/9
N2 - 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.
AB - 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.
KW - Corticosteroids
KW - IVIG
KW - NSAIDs
KW - PANDAS
KW - PANS
KW - Plasmapheresis
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U2 - 10.1089/cap.2016.0148
DO - 10.1089/cap.2016.0148
M3 - Article
AN - SCOPUS:85033219753
VL - 27
SP - 574
EP - 593
JO - Journal of Child and Adolescent Psychopharmacology
JF - Journal of Child and Adolescent Psychopharmacology
SN - 1044-5463
IS - 7
ER -