Pediatric Rheumatology: High-Yield Strategies in Primary Care

Educational Objectives

The goal of this program is to improve the management of rheumatic conditions in pediatric patients. After hearing and assimilating this program, the clinician will be better able to:

1. Diagnose the cause of joint symptoms in children.
2. Recognize mimics of juvenile idiopathic arthritis.

Summary

Evaluation and Management of Joint Symptoms in Pediatric Patients

Overview: the clinician must delineate between arthritis and arthralgia and note the duration of symptoms; arthritis is easier to identify in the presence of joint swelling or effusion; arthritis definition — patients must have at least 2 of 3 symptoms, ie, limitation of range of motion with pain along the joint line, painful range of motion, and/or warmth and redness; for a diagnosis of juvenile idiopathic arthritis (JIA), symptoms must be present for ≥6 wk; having symptoms for <4 wk is classified as acute arthritis; symptoms lasting 4 to 6 wk could be persisting reactive arthritis or early, evolving JIA

Symptoms of JIA vs biomechanical issues: children with JIA are more symptomatic in the morning and after prolonged inactivity; inflammatory joint pain (IJP) improves with movement, while biomechanical problems worsen as the day progresses; children aged 12 mo to 3 yr with oligoarticular JIA may have loss of gross motor skills (eg, scooting or crawling instead of walking)

Physical examination: with unilateral chronic knee swelling, look for leg-length discrepancies; inflamed joints tend to grow faster; look for bony overgrowth (thin legs with bulky knees); Foster and Jandial (2013) describe use of the pediatric Gait, Arms, Legs and Spine (pGALS) examination; compare the affected side with the contralateral side; refer to a rheumatologist for arthritis lasting ≥6 wk

Five broad categories of arthritis: infectious, reactive or postinfectious, orthopedic or internal derangement, hematologic or oncologic problems, and rheumatoid arthritis (RA); characterization as acute, chronic, or subacute affects the ranking among these categories; acute — infectious or orthopedic etiology may be ranked higher, depending on the situation; chronic — consider RA; hematologic or oncologic problems may be chronic (eg, leukemia mimics JIA [causes pain at night]); look for red flags of malignancy (tiredness, easy bruising); malignancy affects unusual joints (eg, shoulder, talocuboid)

Managing acute arthritis: for septic joints, refer to the emergency department (ED); consult with infectious disease (ID) specialists for osteomyelitis or orthopedists for internal derangement or injury

Diagnosis and management of chronic arthritis: order an interferon-γ release assay (eg, QuantiFERON test) because tuberculosis mimics JIA; an antinuclear antibody (ANA) test can be performed for chronic arthritis lasting >6 wk; refer asymptomatic younger children with suspected JIA to an ophthalmologist for uveitis screening; ≈50% of children with JIA respond to nonsteroidal anti-inflammatory drugs (NSAIDs); proton pump inhibitors help to prevent gastritis and ulcers (occurs in the first 4-6 wk on NSAIDs)

Other Rheumatic Conditions

Rheumatic conditions with fever: persistent fever — fever for ≥8 days; may occur in secondary hemophagocytic lymphohistiocytosis (HLH) or macrophage activation syndrome (MAS); obtain ferritin levels; recurrent fever — ≥3 episodes of unexplained fever separated by ≥1 wk of feeling well within a 6 mo timeframe; to identify, ask families or check electronic medical records; rheumatic conditions associated with fever — vasculitides (eg, Henoch-Schonlein purpura [HSP/IgA vasculitis], Kawasaki disease [KD]), systemic onset JIA (SJIA), fever syndromes (FS), lupus, and secondary HLH (MAS); laboratory tests aid in diagnosis

Differentiating FS from SJIA: ask about preceding and associated symptoms; families often are able to predict onset of a fever in a child with FS; when fever spikes in SJIA, an evanescent rash develops, then resolves once the fever subsides; FS can mimic viral illnesses, with symptoms such as vomiting, diarrhea, joint pain, abdominal pain, or conjunctivitis

Diagnosing conditions with fever: vasculitides — HSP and KD are among the most common conditions that occur acutely with high spiking fevers; SJIA — rash appears on rubbing of the skin; the clue to identifying FS is recurring fever; lupus — common symptoms are cutaneous (malar rashes); arthritis — common in small joints, fingers, and toes; MAS — children appear unwell

Laboratory tests:ferritin level is elevated in SJIA and lupus; obtain C-reactive protein levels; children with indolent vasculitides, lupus, or SJIA have markedly elevated erythrocyte sedimentation rate; urinalysis is important if there are concerns for vasculitis or lupus because renal involvement is typical; children with FS feel well between fever episodes (except those with familial Mediterranean fever)

Adolescent female patients with fatigue and weight loss: difficult to diagnose; can be granulomatosis with polyangiitis (GPA) or eosinophilic granulomatosis with polyangiitis (EGPA); consider lupus-type conditions and the vasculitides (indolent subtypes); mimics of rheumatic conditions — inflammatory bowel disease (IBD), immunodeficiencies, and other autoimmune conditions that are not rheumatic

Key diagnostic clues: EGPA — consider in children with sudden acute flare-ups of asthma; GPA — consider in adolescents who develop sudden sinusitis, otitis media, or hearing problems; discoid lupus — presents similarly to tinea infection; consider in cases of hair loss and no response to antifungal therapy; chronic unilateral conjunctivitis — can be seen in some rheumatic conditions; relapsing polychondritis — ear swelling that spares the lobe; septal perforations — occur in vasculitis; ≥3 ulcers in 1 yr — consider rheumatic or IBD; rashes — consider lupus or dermatomyositis

Frequent falls with weakness and tiredness: infectious myositis — consider in cases of viral infections (influenza B or parainfluenza);causes acute-onset bilateral calf pain; idiopathic inflammatory myositis — consider in children who have sudden difficulty washing their hair, putting on pants, squatting, getting out of bed, and ascending stairs; symptoms progressively worsen

Juvenile dermatomyositis: common in girls (typically presenting at 7 yr of age); examination findings — heliotrope rash (purple rash around the eye) and Gottron papules (similar to eczema); inability to lift the arms due to shoulder-girdle weakness; inability to lie flat and lift the head is concerning (indicates potential for problems with swallowing and/or breathing and anterior neck muscle weakness); Gower sign; diagnostic testing — although creatine kinase level tends to rise first, all muscle enzyme levels should be assessed

Caring for patients with rheumatic conditions: avoid live vaccines in immunosuppressed patients; consider stress-dose steroids for patients on long-term steroid therapy when stressors are present (eg, first episode of vomiting or diarrhea, high fever, significant pain, procedures); for children with SJIA, lupus, EGPA, or Wegener granulomatosis who have a fever, check ferritin levels to rule out secondary HLH; chest pain and dyspnea are concerning in older adolescents with lupus because of their elevated risk for myocardial infarction (obtain electrocardiography); for surgical procedures with high risk for infection, hold immunosuppressive medications for ≥5 half-lives

Readings

Brix N, Rosthøj S, Glerup M, et al. Identifying acute lymphoblastic leukemia mimicking juvenile idiopathic arthritis in children. PLoS One. 2020;15(8):e0237530. Published 2020 Aug 11. doi:10.1371/journal.pone.0237530; Foster HE, Jandial S. pGALS - paediatric Gait Arms Legs and Spine: a simple examination of the musculoskeletal system. Pediatr Rheumatol Online J. 2013;11(1):44. Published 2013 Nov 12. doi:10.1186/1546-0096-11-44; Martini A, Lovell DJ, Albani S, et al. Juvenile idiopathic arthritis. Nat Rev Dis Primers. 2022;8(1):5. Published 2022 Jan 27. doi:10.1038/s41572-021-00332-8; Pachman LM, Nolan BE, DeRanieri D, et al. Juvenile dermatomyositis: new clues to diagnosis and therapy. Curr Treatm Opt Rheumatol. 2021;7(1):39-62. doi:10.1007/s40674-020-00168-5; Soon GS, Laxer RM. Approach to recurrent fever in childhood. Can Fam Physician. 2017;63(10):756-762; Xu L, Liu KX, Senna MM. A practical approach to the diagnosis and management of hair loss in children and adolescents. Front Med (Lausanne). 2017;4:112. Published 2017 Jul 24. doi:10.3389/fmed.2017.00112.

Disclosures

For this program, members of the faculty and planning committee reported nothing relevant to disclose.

Acknowledgements

Dr. Harry was recorded at the Practical Pediatrics Conference 2023, held March 24-25, 2023, in Winston-Salem, NC, and presented by Wake Forest University School of Medicine and the Northwest Area Health Education Center. For information on upcoming CME activities from these presenters, please visit https://northwestahec.wakehealth.edu. Audio Digest thanks the speakers, Wake Forest University School of Medicine and the Northwest Area Health Education Center for their cooperation in the production of this program.

CME/CE INFO

Accreditation:

The Audio- Digest Foundation is accredited by the Accreditation Council for Continuing Medical Education to provide continuing medical education for physicians.

The Audio- Digest Foundation designates this enduring material for a maximum of 0.75 AMA PRA Category 1 Credits™. Physicians should claim only the credit commensurate with the extent of their participation in the activity.

Audio Digest Foundation is accredited as a provider of continuing nursing education by the American Nurses Credentialing Center's (ANCC's) Commission on Accreditation. Audio Digest Foundation designates this activity for 0.75 CE contact hours.

Lecture ID:

PD692701

Expiration:

This CME course qualifies for AMA PRA Category 1 Credits™ for 3 years from the date of publication.

Instructions:

To earn CME/CE credit for this course, you must complete all the following components in the order recommended: (1) Review introductory course content, including Educational Objectives and Faculty/Planner Disclosures; (2) Listen to the audio program and review accompanying learning materials; (3) Complete posttest (only after completing Step 2) and earn a passing score of at least 80%. Taking the course Pretest and completing the Evaluation Survey are strongly recommended (but not mandatory) components of completing this CME/CE course.

Estimated time to complete this CME/CE course:

Approximately 2x the length of the recorded lecture to account for time spent studying accompanying learning materials and completing tests.