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Basics ⬇

[Section Outline]

Author:

Susanne M.Hardy

Description!!navigator!!

Pediatric Considerations
  • Neonatal lupus may occur when maternal autoantibodies cross the placenta:
    • Associated with transient anemia and thrombocytopenia
  • Congenital heart block is the most serious complication

Geriatric Considerations
  • 10 times greater risk of MI due to atherosclerosis
  • High incidence of osteoporosis related to chronic steroid use

Risk Factors!!navigator!!

Genetics

  • More common in females than males (9:1 ratio)
  • More common in women of childbearing age
  • More common in African Americans
  • Higher frequency of systemic lupus erythematosus (SLE) and other autoimmune diseases among first-degree relatives

Etiology!!navigator!!

Diagnosis ⬆ ⬇

[Section Outline]

Signs and Symptoms!!navigator!!

History

  • Symptoms commonly accumulate and exacerbate over years, with flares and remissions. A history of fatigue, rashes, and joint pain may point to the diagnosis
  • Patients describe arthralgias out of proportion to physical findings
  • Ask about medication use (it is suggested to avoid minocycline in nondrug-induced SLE), sun exposure

Physical Exam

  • Check for fever
  • Carefully evaluate skin for rashes and vasculitis

Essential Workup!!navigator!!

Diagnostic Tests & Interpretation!!navigator!!

Lab

  • CBC:
    • Leukopenia, thrombocytopenia, normochromic normocytic anemia
    • Degree of hematologic disorders suggests degree of disease activity
  • ESR:
    • May be elevated during acute exacerbations
    • Not a good indicator of active disease
  • CRP
    • May also be elevated
    • Marked elevation may be a sign of infection
  • PTT:
    • May be elevated in patients with lupus anticoagulant
  • UA:
    • Protein (persistent proteinuria >0.5 g/d or 3+ persistently)
    • Note that marked proteinuria and reduced GFR may result from either active inflammation or scarred glomeruli
    • Casts (red blood cell)
    • Hematuria
    • WBCs
  • Complement levels (C3 and C4):
    • Low or high levels often indicate active lupus, particularly lupus nephritis
  • Send antinuclear antibody, rheumatoid factor (RF), antistreptolysin O (ASO) titer if diagnosis unclear
  • Anti-Sm and anti-dsDNA are diagnostic
  • A false-positive Venereal Disease Research Laboratory (VDRL) test is supportive of the diagnosis
  • Do not repeatedly check ANAs or other specific antibodies besides anti-dsDNA antibodies because unlikely to change over time and do not correlate with disease activity
  • Joint aspirate typically shows fluid with fewer than 3,000 WBCs
  • LP if suspicion for meningitis or encephalitis

Imaging

  • CXR:
    • Pneumonitis
    • Pneumonias
    • Pleural effusion
    • Cardiomegaly
  • ECG/echocardiogram
  • CT chest:
    • Pulmonary embolus
    • Pulmonary hemorrhage
    • Diffuse alveolar hemorrhage
  • CT head for change in mental status or neurologic findings (lupus cerebritis is a diagnosis of exclusion)
Pregnancy Prophylaxis
  • Pregnancy is not recommended during active disease owing to the high risk of spontaneous abortion
  • The effect of pregnancy on disease activity is variable

Differential Diagnosis!!navigator!!

Treatment ⬆ ⬇

[Section Outline]

Initial Stabilization/Therapy!!navigator!!

ABCs

ED Treatment/Procedures!!navigator!!

Medication!!navigator!!

Follow-Up ⬆ ⬇

[Section Outline]

Disposition!!navigator!!

Admission Criteria

  • Patients who have end-organ disease such as renal, cardiac, or CNS involvement
  • Thrombocytopenia with hemorrhage, arterial or venous thrombosis
  • Consider admission with pericarditis, myocarditis, pleural effusion or infiltrates, and evidence of vasculitis
  • Those with severe end-organ or life-threatening manifestations should be admitted to the ICU
  • Patients with lupus should be treated as immunocompromised and suspected or diagnosed infections should be treated aggressively

Discharge Criteria

  • Patients may be discharged home with mild flare-ups if afebrile, well hydrated, and not ill appearing
  • ESR should not be used as disposition criterion as it may be elevated long after a flare-up has subsided

Issues for Referral

Because lupus is a chronic disease, a rheumatologist or knowledgeable primary care physician (PCP) must follow the patient adequately

Follow-up Recommendations!!navigator!!

PCPs must educate patients regarding sun protection, immunizations, and lowering risks of atherosclerosis

Pearls and Pitfalls ⬆ ⬇

  • The diagnosis of SLE is complicated and requires a thorough history and physical exam supported by appropriate lab testing
  • Chronic steroid therapy leads to immunosuppression
  • Renal involvement confers a poor prognosis
  • Serum creatinine may be elevated, but is a poor indicator of the disease (urinalysis is more sensitive with proteinuria and /or red blood cell casts)
  • All patients with SLE should be offered annual, seasonal influenza vaccinations and be sure that pneumococcal vaccination is up to date
  • VDRL may be falsely positive
  • SLE is a risk factor for coronary artery disease
  • Most useful tests to predict an SLE flare are increased anti-dsDNA antibodies and hypocomplementemia, but not specific

Additional Reading ⬆ ⬇

The authors gratefully acknowledge John Lemos for his contribution to the previous edition of this chapter.

Codes ⬆

ICD9

ICD10

SNOMED