What Are the 11 Criteria for Lupus?

Learn the 11 criteria for lupus, which were developed by the American College of Rheumatology.

Systemic lupus erythematosus (SLE), the most common type of lupus, is a multi-organ autoimmune disease in which your body's immune cells attack your own organs or tissues. This can affect any organ in the body and cause inflammation.

Lupus can produce a variety of symptoms in different individuals. Based on the symptoms, the American College of Rheumatology has developed some criteria.

If you have any four out of the 11 criteria, then you are recognized to have lupus.

The following are the 11 criteria for lupus:

1. Malar rash:
   • Malar rash also known as butterfly rash is commonly presented on the face.
   • It is identified by an erythematous flat or raised rash across the bridge of the nose and cheeks, sparing the nasolabial folds. 
   • It might be temporary or spread to other parts of the face skin. 
   • Some people may not present with a malar rash.
2. Discoid rash:
   • Discoid rash develops in 20 percent of people with SLE and can leave disfiguring scars. 
   • It may appear as erythematous patches with keratotic scaling on sun-exposed skin. 
   • Scarring caused by follicular plugging may be visible in the ears.
3. Photosensitivity:
   • Many people have worsening lupus symptoms after being exposed to ultraviolet radiation, either from the sun or from artificial light.
   • After sun exposure, photosensitive people may get a skin rash known as a butterfly rash, which occurs over the nose and cheeks. Other rashes may resemble hives.
4. Oral ulcers:
   • It is estimated that 40% of people with SLE have mouth ulcers or sores.
   • These ulcers are usually painless and observed by your doctor.
5. Nonerosive arthritis:
   • Approximately, 95% of people with lupus will develop arthritis (inflammation of the joints) or arthralgias (joint aches) at some stage throughout their condition. 
   • Tenderness, swelling, or effusion are symptoms of nonerosive arthritis involving two or more peripheral joints.
6. Serositis:
   • Serositis is inflammation of one or more serous membranes.
   • Pleural or pericardial involvement affects about 16% of people with SLE.
     • Pleuritis is the inflammation of the pleura, the lining that covers the lungs. Lungs in people with SLE are affected by pleuritis. Pleural pain, pleural effusion, or rubbing sound identified by the doctor indicates pleuritis.
     • Pericarditis: Lupus pericarditis is the most common type of cardiac disease in people with lupus. Immune complexes formed with active lupus can induce pericardial inflammation. Pericarditis is documented by electrocardiogram, rubbing or pericardial effusion.
7. Renal disorder:
   • Lupus nephritis develops when lupus autoantibodies attack waste-filtering mechanisms in your kidneys. 
   • This results in renal inflammation, which can result in blood in the urine, protein in the urine, elevated blood pressure, reduced kidney function, or even kidney failure.
   • Proteinuria persists for more than 500 mg/24 hours or higher than 3+ if measurement is not done.
   • Red cell, hemoglobin, granular, tubular, or mixed cellular casts are seen in the urine.
8. Neurologic disorder:
   • Lupus can affect the nervous system at all levels, causing a spectrum of neurological diseases.
   • In the absence of provoking substances or established metabolic derangements such as uremia, ketoacidosis, or electrolyte imbalance, lupus can cause seizures or psychosis.
9. Hematologic disorder:
   • Hematologic abnormalities are common in SLE, both at diagnosis and during the disease's progression.
   • SLE's major hematologic manifestations include:
     • Hemolytic anemia: With reticulocytosis
     • Leukopenia: Leucocytes less than 4,000/mm³ total on two or more occasions
     • Lymphopenia: Lymphocytes less than 1,500/mm³ on two or more occasions
     • Thrombocytopenia: Platelets less than 100,000/mm³
10. Antinuclear antibodies (ANA):
    • ANA are autoantibodies against your cells' nucleus. The ANA test is positive in 98% of people with systemic lupus, making it the most sensitive diagnostic test for verifying the disease's diagnosis.
    • An abnormal ANA titer is measured by immunofluorescence or a similar test at any time and in the absence of medicines.
11. Immunologic disorders:
    • Anti-DNA: The anti-dsDNA antibody test (anti-double-stranded DNA [anti-dsDNA]) is a blood test that searches for antibodies to double-stranded DNA because anti-dsDNA antibodies are particularly specific for SLE and are not present in any other autoimmune diseases.
    • Anti-Smith (Sm): Anti-Sm antibody is a highly specific antibody for SLE.
    • Antiphospholipid antibody: The presence of an antiphospholipid antibody, such as the lupus anticoagulant and anticardiolipin antibody, is connected with a tendency for blood clots. Blood clots can develop anywhere in the body and cause stroke, gangrene, heart attack, and other life-threatening consequences.
      • An abnormal serum level of IgG or IgM anticardiolipin antibodies
      • A positive test result for lupus anticoagulant using a standard method
      • A false-positive syphilis serologic test that has been positive for at least 6 months and has been confirmed by Treponema pallidum immobilization or fluorescence treponemal antibody absorption testing.