Rheumatologic Emergencies

Susa Benseler

Introduction

Juvenile Idiopathic Arthritis (JIA)

Arthritis (inflammation of the synovium) with onset < 16 years of age
Previously called juvenile rheumatoid arthritis
Name changed to reflect difference between juvenile and adult forms of arthritis
Likely due to genetic, infectious, and environmental factors
Arthritis in children may resemble joint pain associated with infections, cancer, bone disorders, and other inflammatory disorders: must exclude before giving diagnosis of JIA

Types of JIA

Clinical Presentation

Active arthritis: evidence of swelling, effusion, or two of the following: heat, limited or tender range of movement
Additional symptoms:
- Fevers, weight loss, fatigue (sJIA), rash (sJIA, psoriasis), organomegaly, lymphadenopathy (sJIA)
- Enthesitis, tendonitis
- Uveitis, red eye, synechiae, impaired vision

Investigations

Inflammatory markers: ESR, CRP, CBC
ANA, RF, HLA B27
LDH to rule out malignancy
Consider PPD to rule out TB
X-rays to rule out tumors, fractures, lytic lesions, osteomyelitis
- Look for osteopenia, effusions, joint damage

Differential Diagnosis

Management

Rule out important differential diagnoses: septic joint, malignancy, trauma
Symptoms < 6 weeks: physician reassessment in 2 weeks, consider ophthalmology or optometry assessment for uveitis
NSAIDs:
- Naproxen 20 mg/kg/d divided bid or indomethacin 2 mg/kg/d divided tid for at least 2 weeks (give repeats)
- Potential adverse effects: headaches, stomach pain, nausea
Consult rheumatology if:
- Persistent arthritis (> 6 weeks)
- Severe (poly) or systemic arthritis

Pediatric Systemic Lupus Erythematosus (pSLE)

Multisystem autoimmune disease with substantial variability in presentation and course

Clinical Features

New rashes, facial redness, mouth ulcers, nosebleeds, joint pain, chest pain
CNS symptoms including hallucinations, headaches, seizures, other neurologic deficits
Constitutional symptoms: fever, fatigue, weight loss, hair loss

Classification Criteria

Investigations

Inflammatory markers: ESR, CRP, CBC/diff
C3 and C4 complement, IgG
Creatinine, urea, albumin, protein, LFTs
Urinalysis including dipstick, microscopy, and protein:creatinine ratio
Autoantibodies: ANA, ds-DNA (IF and ELISA), anti-SM, anti-Ro, anti-LA, anti-cardiolipin, RF
Lupus anticoagulans (anti-phopholipid screen)
Chest X-ray, ECG

Table 39.1 Classification Criteria for SLE

Malar rash	Fixed erythema over malar eminences, sparing nasolabial folds
Discoid rash	Erythematous raised patches
Serositis	Pleuritis: pleuritic pain or audible rub or pleural effusion OR
	Pericarditis: documented by ECG or rub or evidence of pericardial effusion
Oral ulcers	Visible oral or nasopharyngeal ulceration, usually painless
Arthritis	Nonerosive arthritis involving ≥ 2 joints with tenderness, swelling, or effusion
Photosensitivity	Skin rash due to unusual reaction to sunlight
Blood (hematologic disorder)	Hemolytic anemia OR
	Leukopenia—WBC < 4,000/mm³ OR
	Lymphopenia < 1,500/mm³ OR
	Thrombocytopenia—platelets < 100,000/mm³
Renal (Nephritis)	Persistent proteinuria > 0.5 grams per day or > 3+ OR cellular casts
ANA	Abnormal titer of antinuclear antibody
Immunologic disorder	Anti-DNA: antibody to native DNA in abnormal titer OR
	Anti-Sm: presence of antibody to Sm nuclear antigen
Neurologic disorder	Seizures OR psychosis