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Lupus Diagnosis

There is no one single diagnostic test for lupus. Your rheumatologist will evaluate your symptoms, examine your body, check blood and urine tests, and eventually decide if you have lupus. Lupus is diagnosed when four clinical and laboratory features are present.

American College of Rheumatology (ACR) Criteria for the Classification of SLE

According to the ACR criteria, a person is defined as having SLE if any four or more of the 11 following criteria are present, serially or simultaneously, during any interval of observation.

1. Malar Rash: Fixed erythema, flat or raised, over the malar eminences, tending to spare the nasolabial folds

2. Discoid Rash: Erythematous raised patches with adherent keratotic scaling and follicular plugging; atrophic scarring may occur in older lesions

3. Photosensitivity: Skin rash as a result of unusual reaction to sunlight, by patient history or physician observation

4. Oral Ulcers: Oral or nasopharyngeal ulceration, usually painless, observed by a physician

5. Nonerosive Arthritis: Involving two or more peripheral joints, characterized by tenderness, swelling, or effusion

6. Pleuritis or Pericarditis:

  • Pleuritis – convincing history of pleuritic pain or rubbing heard by physician or evidence of pleural effusion, or
  • Pericarditis – documented by electrocardiogram or rub or evidence of pericardial effusion

7. Renal Disorder:

  • Persistent proteinuria greater than 0.5 grams per day or greater than 3+ if quantitation not performed, or
  • Cellular casts – may be red cell, hemoglobin, granular, tubular, or mixed

8. Neurological Disorder:

  • Seizures – in the absence of offending drugs or known metabolic derangements; e.g., uremia, ketoacidosis, or electrolyte imbalance, or
  • Psychosis – in the absence of offending drugs or known metabolic derangements; e.g., uremia, ketoacidosis, or electrolyte imbalance

9. Hematological Disorder:

  • Hemolytic anemia – with reticulocytosis, or
  • Leukopenia – < 4,000/ mm3 on 2 or more occasions, or
  • Lyphopenia – < 1,500/ mm3 on 2 or more occasions, or
  • Thrombocytopenia – < 100,000/ mm3 in the absence of offending drugs

10. Immunological Disorder:

  • Anti-DNA: antibody to native DNA in abnormal titer, or
  • Anti-Sm: presence of antibody to Sm nuclear antigen, or
  • Positive finding of antiphospholipid antibodies on:
    • An abnormal serum level of IgG or IgM anticardiolipin antibodies
    • A positive test result for lupus anticoagulant using standard method, or
    • A false-positive test result for at least six months confirmed by Treponema pallidum immobilization or fluorescent treponemal antibody absorption test

11. Positive Antinuclear Antibody: An abnormal titer of antinuclear antibody by immunofluorescence or an equivalent assay at any point in time and in the absence of drugs

Systemic Lupus International Collaborative Clinics (SLICC) Criteria for the Classification of SLE

According to the SLICC criteria, a person is defined as having SLE if any four or more of the following criteria are present (at least one clinical and one laboratory criteria) or biopsy-proven lupus nephritis with positive ANA or Anti-DNA.

Clinical Criteria
  1. Acute cutaneous lupus
  2. Chronic Cutaneous lupus
  3. Oral or nasal ulcers
  4. Non-scarring alopecia
  5. Arthritis
  6. Serositis
  7. Renal
  8. Neurologic
  9. Hemolytic anemia
  10. Leukopenia
  11. Thrombocytopenia (<100,000/mm3)
Immunological Criteria
  1. ANA
  2. Anti-DNA
  3. Anti-Sm
  4. Antiphospholipid Ab
  5. Low compliment (C3, C4, CH50)
  6. Direct Coombs' Test (do not count in the presence of hemolytic anemia)

Tests to Diagnose and Monitor SLE

The activity and severity of lupus must be assessed repeatedly over time. Evaluations are designed to look for the presence of new signs or symptoms or the worsening of signs or symptoms in the involved systems. Blood tests are used to predict and verify disease flares.

Initial Evaluation

  • CBC
  • CMP
  • Inflammatory markers
  • Organ specific evaluation
  • Presence of autoantibodies
    • ANA
    • SSA/Ro-SSB/La
    • Sm
    • RNP
    • dsDNA
    • aCL
    • LAC
    • B2GPI
  • Complement levels C3, C4, CH50
  • Evaluate renal involvement and activity
    • U/A
    • P/C ratio
    • 24-hour urine collection for protein/creatinine
    • Renal biopsy

Subsequent Evaluations

  • Organ specific evaluation: As needed to follow-up worsening or response to treatment
  • Presence of autoantibodies/complement
    • dsDNA, C3, C4: at each visit
    • acL: repeat 1-2 times peryear
    • LAC: repeat 1-2 times per year
    • B2GPI: repeat 1-2 times per year
  • Evaluate renal disease
    • U/A at each visit
    • P/C ratio if renal involvement is present

In addition, patients must be monitored for associated disorders and related problems, such as rheumatoid arthritis, Sjögren’s syndrome, pulmonary fibrosis, and pulmonary hypertension, LN, cardiac manifestations, scleroderma, gastrointestinal manifestations, nervous system disease, eye manifestations, and ear, nose, and throat manifestations.