1262 SECTION XI Pediatric Critical Care Immunity and Infection with bronchoalveolar lavage should be considered early, as lung lesions can progress quickly into severe acute lung injury and pa tients[.]
1262 S E C T I O N X I Pediatric Critical Care: Immunity and Infection with bronchoalveolar lavage should be considered early, as lung lesions can progress quickly into severe acute lung injury and patients can rapidly decompensate For patients receiving immunosuppressive regimens containing cyclophosphamide, rituximab, and higher-dose corticosteroids, P jirovecii pneumonia prophylaxis is typically recommended Prophylaxis against opportunistic infections may be necessary in patients with severe lymphopenia (,500/mL) In patients with a history of Herpes zoster or fungal infection, antiviral and antifungal prophylaxis should also be considered eTable 106.5 provides an overview of the most common biological therapies used in treatment of rheumatologic diseases, along with associated infectious considerations Biological therapies, such as infliximab or rituximab, may also cause mild or severe hypersensitivity reactions; thus patients must be monitored closely In some cases, patients may need to be admitted to the ICU for medication desensitization protocols.118 Managing medications can be complex in critically ill patients with rheumatologic disease As many of the medications are cleared by the kidneys or affected by dialysis, this can have important implications for dosing Balancing the complexities of the organ systems involved in rheumatologic disease with the potential toxicities and interactions of therapy is best accomplished with a multidisciplinary approach Summary In summary, rheumatologic diseases manifest with a wide variety of presentations and can affect all organ systems Therefore, pediatric intensivists need a basic understanding of the most commonly seen conditions in pediatric rheumatology In addition, many of the therapies for rheumatologic diseases have clinically significant side effects that may require close monitoring in the intensive care setting The diagnosis of rheumatologic disease is challenging It requires a thorough history and physical examination as well as consideration of a broad differential diagnosis Early identification and management can significantly reduce morbidity and mortality—providers in the intensive care setting play a vital role in providing life-saving interventions to children with rheumatologic disease Key References Aguiar CL, Soybilgic A, Avcin T, Myones BL Pediatric antiphospholipid syndrome Curr Rheumatol Rep 2015;17(4):27 Breda L, Nozzi M, De Sanctis S, Chiarelli F Laboratory tests in the diagnosis and follow-up of pediatric rheumatic diseases: an update Semin Arthritis Rheum 2010;40(1):53-72 Brunner HI, Gladman DD, Ibanez D, Urowitz MD, Silverman ED Difference in disease features between childhood-onset and adult-onset systemic lupus erythematosus Arthritis Rheum 2008;58(2):556-562 Hiraki LT, Benseler SM, Tyrrell PN, Hebert D, Harvey E, Silverman ED Clinical and laboratory characteristics and long-term outcome of pediatric systemic lupus erythematosus: a longitudinal study J Pediatr 2008;152(4):550-556 Hsu CL, Chen KY, Yeh PS, et al Outcome and prognostic factors in critically ill patients Jesus AA, Goldbach-Mansky R IL-1 blockade in autoinflammatory syndromes Annu Rev Med 2014;65:223-244 Kobayashi T, Saji T, Otani T, et al Efficacy of immunoglobulin plus prednisolone for prevention of coronary artery abnormalities in severe Kawasaki disease (RAISE study): a randomised, open-label, blindedendpoints trial Lancet 2012;379(9826):1613-1620 Lerkvaleekul B, Vilaiyuk S Macrophage activation syndrome: early diagnosis is key Open Access Rheumatol 2018;10:117-128 Minoia F, Davi S, Horne A, et al Clinical features, treatment, and outcome of macrophage activation syndrome complicating systemic juvenile idiopathic arthritis: a multinational, multicenter study of 362 patients Arthritis Rheumatol 2014;66(11):3160-3169 Radhakrishna SM, Reiff AO, Marzan KA, et al Pediatric rheumatic disease in the intensive care unit: lessons learned from 15 years of experience in a tertiary care pediatric hospital Pediatr Crit Care Med 2012;13(3):e181-e186 Petty RE, Southwood TR, Manners P, et al International League of Associations for Rheumatology classification of juvenile idiopathic arthritis: second revision, Edmonton, 2001 J Rheumatol 2004;31(2): 390-392 Radhakrishna SM, Reiff AO, Marzan KA, et al Pediatric rheumatic disease in the intensive with systemic lupus erythematosus: a retrospective study Crit Care 2005;9(3):R177-183 The full reference list for this chapter is available at ExpertConsult.com JIA, uveitis, Bechet disease, CNO, sarcoidosis, Kawasaki disease JIA, uveitis, SLE (arthritis) Blocks T-cell costimulation Interleukin-1 inhibitors Abatacept Anakinra Binds B-lymphocyte stimulating factor, inhibiting B-cell proliferation and immunoglobulin production JAK-STAT inhibitor Belimumab Tofacitinib 3h TB, cellulitis, herpes zoster, pneumonia (adult data) Viral URI, cellulitis, pneumonia Varicella, Pneumocystis jirovecii, severe bacterial infections, progressive multifocal leukoencephalopathy Viral URI, gastroenteritis, pneumonia, varicella Viral URI, pneumonia, varicella Viral URI, pneumonia Viral URI, gastroenteritis, varicella, candidiasis, pneumonia, TB, other endemic fungal infections (histoplasmosis/ coccidiomycoses) Infectious Concerns Neutropenia, hyperlipidemia Infusion reactions, headache, psychiatric symptoms (adults), TIA (adults), hypogammaglobulinemia, malignancy (adults) Infusion reactions, hypogammaglobulinemia, neutropenia Neutropenia, thrombocytopenia, hyperlipidemia, transaminitis Hyperlipidemia, transaminitis Thrombocytopenia Infusion reactions, drug-induced lupus, demyelinating conditions, psoriasis, association with malignancy risk Other Side Effects Italics indicate off label use in pediatrics AAV, ANCA-associated vasculitis; AI, adrenal insufficiency; AIHA, autoimmune hemolytic anemia; ANCA, antineutrophil cytoplasmic antibodies; CAPS, catastrophic antiphospholipid syndrome; CNO, chronic nonbacterial osteomyelitis; FMF, familial Mediterranean fever; HIDS, hyper-immunoglobulin D syndrome; JDM, juvenile dermatomyositis; JIA, juvenile idiopathic arthritis; MAS, macrophage activation syndrome; TB, tuberculosis; TNF, tumor necrosis factor; SLE, systemic lupus erythematosus; TIA, transient ischemic attack; TRAPS, TNF receptor–associated periodic syndrome; URI, upper respiratory infection JIA wk Binds CD20 receptor, induces B-cell apoptosis Rituximab wk SLE JIA, systemic JIA, uveitis, vasculitis Interleukin-6 inhibitor Tocilizumab wk 3–4 wk Variable; effect may last 3–12 mo Systemic JIA, CAPS Rilonocept 4–6 h wk wk wk 4d Half-Life AAV, AIHA, AI cytopenia, refractory SLE, refractory JDM Systemic JIA, CAPS, FMF, HIDS, TRAPS Canakinumab Systemic JIA, CAPS, MAS, periodic fever syndromes JIA, Uveitis, Bechets, CNO, Sarcoidosis JIA, CNO, Periodic fever syndromes Infliximab TNF-a inhibitors Etanercept Rheumatologic Indications Adalimumab Mechanism Biological eTABLE 106.5 Overview of Common Biological Response 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Arthritis Rheumatol 2014;66(6):1405-1413 Ogilvie EM, Khan A, Hubank M, Kellam P, Woo P Specific gene expression profiles in systemic juvenile idiopathic arthritis... Shah M, Mamyrova G, et al The myositis autoantibody phenotypes of the juvenile idiopathic inflammatory myopathies Medicine (Baltimore) 2013;92(4):223-243 64 Huber AM Juvenile dermatomyositis: