Rheumatologic Emergencies



Rheumatologic Emergencies


Susa Benseler



Introduction



  • Juvenile idiopathic arthritis (JIA)


  • Pediatric systemic lupus erythematosus (pSLE)


  • Juvenile dermatomyositis (JDM)


  • Vasculitis


  • Macrophage activation syndrome (MAS)


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



  • Five main types based on number of joints involved during first 6 months of disease and involvement of other organs:



    • Oligoarthritis: < 5 joints, 50% of JIA



      • Children with anti-nuclear antibodies (ANA) often also have uveitis


    • Polyarthritis: ≥ 5 joints


    • Systemic arthritis: 10-20% of JIA



      • High fevers, rash, and inflammation of other organs, in addition to arthritis



    • Enthesitis-related arthritis: often affects spine, hips, and entheses; mainly boys > 8 years


    • Psoriatic arthritis: arthritis and psoriasis


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



  • Septic arthritis: If in doubt, tap the joint


  • Reactive arthritis, transient synovitis


  • Leukemia


  • Tumors (osteoid osteoma, malignant bone or cartilage tumors)


  • Trauma


  • Inflammatory/allergic diseases: serum sickness, HSP, drug reactions


  • Rare joint diseases


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



  • Diagnosis requires presence of 4 of 11 criteria


  • Remember criteria with mnemonic: “MD SOAP BRAIN”


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

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Jun 22, 2016 | Posted by in EMERGENCY MEDICINE | Comments Off on Rheumatologic Emergencies

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