PFAPA syndrome sometimes called Marshalls syndrome is a benign but enigmatic entity. Frequent recurrences can impact quality of life. Michael and Raju discuss some strategies described in literature to prevent frequent recurrences. References to the article :⁠⁠ - Vanoni, Federica & Theodoropoulou, Katerina & Hofer, Michaël. (2016). PFAPA syndrome: A review on treatment and outcome. Pediatric Rheumatology Online Journal. 14. 10.1186/s12969-016-0101-9. ⁠⁠ - ⁠⁠Batu, Ezgi & Akca, Ummusen & Basaran, Ozge & Bilginer, Yelda & Ozen, Seza. (2022). Probiotic use in the prophylaxis of periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome: a retrospective cohort study. Rheumatology international. 42. 10.1007/s00296-021-05084-y. ⁠ - ⁠⁠⁠⁠⁠Hofer M. Why and How Should We Treat Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis (PFAPA) Syndrome? Paediatr Drugs. 2020 Jun;22(3):243-250. doi: 10.1007/s40272-020-00393-4. PMID: 32342289.⁠⁠⁠⁠⁠ -⁠Kapustova Lenka,Jesenak Milos and al. The use of ketotifen as long-term anti-inflammatory prophylaxis in children with PFAPA syndrome -Frontiers in Immunology- 2023⁠

Amongst the newest 'kids on the block' this group of drugs has only recently been approved by the US FDA for use in Polyarticular JIA. Michael and Raju discuss some off label indications and add a cautionary tale to these 'magical' molecules. References to the article discussed in the podcast : Bonelli M, Kerschbaumer A, Kastrati K, Ghoreschi K, Gadina M, Heinz LX, Smolen JS, Aletaha D, O'Shea J, Laurence A. Selectivity, efficacy and safety of JAKinibs: new evidence for a still evolving story. Ann Rheum Dis. 2024 Jan 11;83(2):139-160. https://pubmed.ncbi.nlm.nih.gov/37923366/ Welzel, T., Winskill, C., Zhang, N. et al. Biologic disease modifying antirheumatic drugs and Janus kinase inhibitors in paediatric rheumatology – what we know and what we do not know from randomized controlled trials. Pediatr Rheumatol 19, 46 (2021). https://doi.org/10.1186/s12969-021-00514-4

HCQ is a crucial drug in the armamentarium in managing lupus and ass a lecturer once quipped itt should be in the tap water of these patients. This discussion examines a paper that describes blood level monitoring for optimal levels to prevent flares. References to the article : - Balevic SJ, Gonzalez D, and al-., with the CARRA Registry investigators. Pharmacokinetics of hydroxychloroquine in paediatric lupus: data from a novel, direct-to-family clinical trial. Lupus Sci Med. 2022 Nov;9 : https://pmc.ncbi.nlm.nih.gov/articles/PMC9639143/ - Haładyj E, Olesińska M, and al.- Antimalarials - are they effective and safe in rheumatic diseases? Reumatologia. 2018 : https://pmc.ncbi.nlm.nih.gov/articles/PMC6052376/ - Garg S, Chewning B, Bartels CM, and al. Reference Range of Hydroxychloroquine Blood Levels That Can Reduce Odds of Active Lupus and Prevent Flares. Arthritis Care Res (Hoboken). 2024 Feb : https://pubmed.ncbi.nlm.nih.gov/37667434/

Colchicine, a plant derivative has established roles in the management of some autoinflammatory disorders. Michael And Raju discuss the practical pediatric usage of this low cost and relatively safe drug. References to the article discussed in the podcast : Lisa K Stamp, Carl Horsley, Leanne Te Karu, Nicola Dalbeth, Murray Barclay, Colchicine: the good, the bad, the ugly and how to minimize the risks, Rheumatology, Volume 63, Issue 4, April 2024, Pages 936–944 : https://academic.oup.com/rheumatology/article/63/4/936/7455266

There are situations when children on Etanercept need to shift drugs.Otions include another TNF blocker vs other molecules. This podcast shows the way based on a recent publication. References : Comparative Effectiveness of a Second Tumor Necrosis Factor Inhibitor Versus a Non–Tumor Necrosis Factor Biologic in the Treatment of Patients With Polyarticular‐Course Juvenile Idiopathic Arthritis - Mannion - 2024 - Arthritis Care & Research - Wiley Onl https://acrjournals.onlinelibrary.wiley.com/doi/10.1002/acr.25339

By far the commonest adverse effects of Methotrexate is vomiting. In this episode Michael and Raju discuss a Dutch paper on the various mechanisms involved and some possible solutions to prevent discontinuation of this vital and otherwise safe drug. References to the article discussed in the podcast : Boris Hügle, E H Pieter van Dijkhuizen, MTX intolerance in children and adolescents with juvenile idiopathic arthritis, Rheumatology, Volume 59, Issue 7, July 2020, Pages 1482–1488, https://doi.org/10.1093/rheumatology/keaa139 Focus on Tables 1 and 2

Kikuchi disease is a rare condition presenting with non specific symptoms like fever and lymphadenopathy. It is part of the differential diagnose of inflammatory diseases. The flares respond well to a short course of steroids, but it can be the first manifestation of Systemic Lupus. References : Deb, A.; Fernandez, V.; Kilinc, E.; Bahmad, H.F.; Camps, N.S.; Sriganeshan, V.; Medina, A.M. Kikuchi–Fujimoto Disease: A Case Series and Review of the Literature. Diseases 2024, 12, 271. https://doi.org/10.3390/diseases12110271 Perry AM, Choi SM. Kikuchi-Fujimoto Disease: A Review. Arch Pathol Lab Med. 2018 Nov;142(11):1341-1346. doi: 10.5858/arpa.2018-0219-RA. PMID: 30407860. https://pubmed.ncbi.nlm.nih.gov/30407860/ Punith, SB1; Agarwal, Ayush1; Garg, Divyani1; Garg, Ajay2; Shamim, Shamim A3; Mallick, Saumya Ranjan4; Gupta, Pranjal5; Srivastava, Achal K1. Kikuchi–Fujimoto Disease Presenting as Autoimmune Limbic Encephalitis: A Case Report with Review of Literature. Annals of Indian Academy of Neurology 28(1):p 116-119, Jan–Feb 2025. | DOI: 10.4103/aian.aian_498_24 https://journals.lww.com/annalsofian/fulltext/2025/01000/kikuchi_fujimoto_disease_presenting_as_autoimmune.20.aspx

IgA Vasculitis remains the commonest form of vasculitis in childhood and Nephritis is the most important long term prognostic indicator. Michael and Raju review a paper and share some of their experiences. References to the article : Sestan M, Jelusic M. Diagnostic and Management Strategies of IgA Vasculitis Nephritis/Henoch-Schönlein Purpura Nephritis in Pediatric Patients: Current Perspectives. Pediatric Health Med Ther. 2023 Mar 7;14:89-98. doi: 10.2147/PHMT.S379862. PMID: 36915829; PMCID: PMC10008002. : https://pubmed.ncbi.nlm.nih.gov/36915829/ Parums DV. A Review of IgA Vasculitis (Henoch-Schönlein Purpura) Past, Present, and Future. Med Sci Monit. 2024 Jan 28;30:e943912. doi: 10.12659/MSM.943912. PMID: 38281080; PMCID: PMC10832303 : https://pubmed.ncbi.nlm.nih.gov/38281080/