- 4 of the 11 criteria in the following list are needed to make the diagnosis:
- Malar rash
- Discoid rash
- Photosensitivity rash
- Oral ulcers
- Arthritis
- Serositis
- Neurologic disorders
- Hematologic disorders
- Immunologic disorders
- Renal disorders
- Antinuclear antibodies
SIGNS AND SYMPTOMS 
- Systemic:
- Skin:
- Malar rash (butterfly maculopapular facial)
- Discoid rash (raised red patches)
- Photosensitivity rash (subacute cutaneous lupus)
- Bullous rash (large blisters)
- Musculoskeletal:
- Myalgias
- Joint pain
- Arthritis:
- Defined as 2 or more peripheral joints
- Polyarthritis, symmetric, or migratory
- Heart:
- Vascular:
- Lungs:
- Dyspnea
- Tachypnea
- Pleural rub
- Rales
- Nervous system:
- GI:
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
Physical Exam
- Check for fever
- Carefully evaluate skin for rashes and vasculitis
ESSENTIAL WORKUP
- Thorough history and physical exam needed to distinguish between major and minor flare-ups
- Major flare-ups:
- CBC
- Electrolytes, BUN, creatinine, glucose
- UA
- ESR
- Chest radiograph, ECG, and pulse oximetry for cardiorespiratory symptoms
DIAGNOSIS TESTS & INTERPRETATION
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:
- Amylase is elevated in mesenteric ischemia and pancreatitis
- 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
- 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 Considerations
- 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
[Outline]
INITIAL STABILIZATION/THERAPY
ABCs
ED TREATMENT/PROCEDURES
- Mainstays include NSAIDs, corticosteroids, antimalarials, and immunosuppressive drugs
- Special attention must be given to CNS and renal involvement as well as infections; these are the main determinants of morbidity
- Mild flare-ups—arthralgias, myalgias, fatigue, and rash:
- NSAIDs (careful with lupus nephritis), acetyl salicylic acid (ASA), topical steroids for rash, sunscreen
- Topical steroids for most cutaneous manifestations
- If not sufficient, begin low-dose prednisone
- Major flare-ups—life- or organ-threatening:
- Methylprednisolone
- Anticoagulation for thrombosis; give blood products early if needed
- Psychotropics for neuropsychiatric symptoms
- Anticonvulsants for seizures
- If poor response, consult rheumatology before starting cytotoxic medications
- Chronically:
- Prednisone taper
- NSAIDs
- Rheumatologist initiated:
- Hormonal therapy, mycophenolate mofetil, rituximab, and autologous marrow stem cell transplant are under investigation
MEDICATION
- Methylprednisolone: 15 mg/kg/d IV up to 1 g; consult rheumatologist for peds dosing
- Prednisone: 5–30 mg (peds: < 0.5 mg/kg) PO daily for minor flare
- Prednisone: 1–2 mg/kg/d PO for major flares in adults
- Ibuprofen: 800 mg (peds: 5–10 mg/kg) PO TID
[Outline]
DISPOSITION
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
PCPs must educate patients regarding sun protection, immunizations, and lowering risks of atherosclerosis
[Outline]