ACR 1997 SLE diagnostic criteria
To establish a diagnosis of SLE, any 4 of the following must be present:
  • Malar rash
  • Discoid rash
  • Photosensitivity
  • Oral ulcers
  • Nonerosive arthritis - involving 2 or more peripheral joints, characterized by tenderness, swelling, or effusion
  • Pleuritis or pericarditis
    • Defined as:
      • Pleuritis - convincing history of pleuritic pain or rubbing heard by a physician or evidence of pleural effusion
      • Pericarditis - documented by electrocardiogram or rub or evidence of pericardial effusion
  • Kidney disease
    • Defined as one of the following:
      • Persistent proteinuria > 0.5 grams per day or > 3+ on a urine dip
      • Cellular casts - may be red cell, hemoglobin, granular, tubular, or mixed
  • Seizures or psychosis
  • Positive antinuclear antibody (ANA)
  • Hematological disorder
    • Defined as one of the following:
      • Hemolytic anemia with reticulocytosis
      • Leukopenia (< 4,000/mm³ on ≥ 2 occasions)
      • Lymphopenia (< 1,500/ mm³ on ≥ 2 occasions)
      • Thrombocytopenia (< 100,000/mm³ in the absence of offending drugs)
  • Immunologic disorder
    • Defined as one of the following:
      • Anti-dsDNA antibodies
      • Anti-Sm antibodies
      • Antiphospholipid antibodies (anticardiolipin, lupus anticoagulant, ≥ 6 month false-positive Treponemal test)


LABS IN SLE
LAB Prevalence in lupus / Comment
Antinuclear antibodies (ANA)
  • > 96% of patients
  • A dilution of 1:160 is considered significant in most patients (see ANA below for more).
  • ANA should not be used to monitor disease activity. [5,6]
Anti double-stranded DNA (anti-dsDNA)
  • 70 - 80% of patients
  • Associated with kidney and skin disease
  • Anti-dsDNA antibody titers can be used to monitor disease activity [4]
Antichromatin antibodies
  • 50 - 90% of patients
  • Associated with proteinuria and drug-induced lupus [7]
Antihistone antibodies
  • 30 - 60% of patients
  • Found in 95% of patients with drug-induced lupus
  • Present in 20% of patients with RA. [7]
N-methyl-D-aspartate receptor antibodies (NMDA receptor antibodies)
  • 33 - 50% of patients
  • Associated with neuropsychiatric lupus [4]
C1q complement antibodies
  • 40 - 50% of patients
  • Associated with kidney disease [4]
Sjögren's Antibody / Anti-SSA (Anti-Ro)
  • 30 - 40% of patients
  • Patients who are ANA-positive and who have SS-A but not SS-B are very likely to have nephritis
  • Found in 60 - 70% of patients with Sjögren's syndrome [7]
Antiphospholipid antibodies (anticardiolipin, anti-Beta 2 glycoprotein I)
  • 20 - 30% of patients
  • Associated with hypercoagulable state, pregnancy loss, accelerated atherosclerosis, pulmonary hypertension, and neuropsychiatric lupus [1,3,4]
Anti-Sm antibodies (Smith antibodies)
  • 10 - 30% of patients
  • Smith antibodies have a high specificity for lupus [4,8]
Ribonucleoprotein antibodies (RNP antibodies, anti-u1)
  • 15 - 25% of patients
  • May be associated with a more benign disease course [4,8]
Sjögren's Antibody / Anti-SSB (Anti-La)
  • 10 - 15% of patients
  • Associated with congenital heart block and neonatal lupus erythematosus
  • Found in 50% - 60% of Sjögren's syndrome [4,7]
Complement levels (C3/C4)
  • Decreased levels can signify disease activity
  • Used to monitor disease activity, particularly in lupus nephritis [7]



Reference [6]
Positive ANA serum dilution % of healthy individuals
with positive ANA at this dilution
1:40 32%
1:80 13%
1:160 5%

  • JIA - juvenile idiopathic arthritis; MCTD - mixed connective tissue disease; SLE - systemic lupus erythematosus; SS - Sjögren’s syndrome; SSc - systemic sclerosis; UCTD - undifferentiated connective tissue disease; IM - inflammatory myopathies DM - dermatomyositis; PBC - primary biliary cirrhosis
  • Reference [6]
ANA pattern Associated condition
Nuclear patterns
Homogenous SLE, drug induced SLE/vasculitis, JIA
Coarse speckled MCTD, SLE, Raynaud, SSc, SS, UCTD
Fine speckled SLE, SS, SSc, IM, MCTD
Centromere SSc (limited), Raynaud’s
Nucleolar SSc, Raynaud’s, IM, overlap
Cytoplasmic patterns
Diffuse SLE, IM
Fine speckled IM, DM, PBC, interstitial lung disease



SLE treatment recommendations
General recommendations in patients without major organ involvement
  • Hydroxychloroquine and corticosteroids as needed
  • In non-responsive patients or patients who cannot reduce steroids below doses acceptable for chronic use, immunosuppressive agents such as azathioprine, mycophenolate mofetil (CellCept®) and methotrexate should also be considered
  • Decreased sun exposure (especially in skin disease)
  • Smoking cessation [13]
  • Belimumab (Benlysta®), a biological that inhibits B-cell survival factor, was approved in 2011 for refractory SLE (see Belimumab vs Placebo below). Requires monthly IV infusions. Expensive.
Lupus Nephritis (LN)
  • Incidence - affects 50 - 60% of patients during first 10 years of disease [9]
  • Pathology - Immune complexes formed from antinuclear antibodies accumulate in the kidneys and cause an influx of inflammatory cells by activating the complement cascade. Low levels of complement (C3 and C4) signify inflammatory activity and kidney damage. [2,9]
  • Monitoring (in patients with no history of LN)
    • Blood pressure every 3 months
    • Urinalysis every 6 months - look for 3+ protein by dipstick, and/or cellular casts (red blood cells, hemoglobin, granular, tubular, or mixed)
    • Spot protein/creatinine ratio every 6 months - positive if > 0.5
    • Serum creatinine every 6 months - look for unexplained increase
    • C3/C4 complement levels every 6 months - look for decrease signifying increased inflammatory activity
    • Anti-dsDNA titer every 6 months - look for increase in titer [9]
  • Treatment - Mycophenolate / Corticosteroids / Cyclophosphamide / Azathioprine / Hydroxychloroquine / Calcineurin inhibitors (e.g. tacrolimus) / Rituximab / ACE inhibitors or ARBs [9,21]
Skin disease
  • Incidence - affects > 80% of patients with SLE at some point during the disease. Lupus skin disease may occur independent of systemic lupus. [10]
  • Pathology - Immune complexes formed from antinuclear antibodies accumulate in the skin and cause an influx of inflammatory cells. Sun exposure may worsen the condition. Plaques with scarring (discoid lupus) occur when the condition is chronic. Mucosal involvement is common. [2,10]
  • Treatment
Neuropsychiatric lupus
  • Incidence - cerebrovascular disease and seizures (5 - 15% of lupus patients); severe cognitive dysfunction, major depression, acute confusional state, and peripheral nervous disorders (1 - 5% of lupus patients); psychosis, myelitis, chorea, cranial neuropathies, and aseptic meningitis (< 1% of lupus patients) [11]
  • Pathology - not completely understood. Associated with antiphospholipid antibodies (cerebrovascular disease, seizures, chorea) and NMDA-receptor antibodies (neurocognitive defects). [2,11]
  • Treatment - workup and treatment should be similar to that in non-SLE patients presenting with the same conditions [11]
Other related conditions
  • Antiphospholipid antibody syndrome - occurs in 10 - 15% of SLE patients; associated with hypercoagulable state, accelerated atherosclerosis, and pulmonary hypertension [1,21]
  • Osteoporosis - secondary to decreased sun exposure and corticosteroid treatment [12]
  • Immunosuppression from medications - increased risk of infections and cancer [12]
  • Musculoskeletal complaints and serositis - treat with NSAIDs, steroids, and hydroxychloroquine [12]
  • Raynaud's phenomenon (20% of patients) - treat with dihydropyridine CCB
  • Cardiovascular disease - lupus greatly increases the risk of cardiovascular disease particularly in younger patients (35 - 50 years) [21]
  • Plaquenil® (hydroxychloroquine) retinopathy - in low-risk patients, baseline eye exam, next exam in 5 years, then yearly. See Plaquenil for more. [12]