Lupus: Systemic Lupus Erythematosus (SLE)
Signs, Symptoms, and Diagnosis
Lupus is a complex disease with varying manifestations. Some people have several different symptoms; others have few. The symptoms in some individuals are severe, while those in others remain mild. Both genetic (inherited) and environmental factors influence the development and severity of lupus symptoms. Because of these characteristics, doctors sometimes have difficulty in correctly diagnosing lupus.
People with lupus have periods in which they are feeling well, called remission, and periods of worsening symptoms, called flares. Lupus patients can often predict the onset of flares due to specific warning signs, such as worsening fatigue and/or onset of headache, fever, dizziness, rash, and/or pain.14 Being able to recognize warning signs is important because catching and treating flares early can prevent them from becoming severe.
The most commonly occurring symptoms of lupus include10,15:
- Intense fatigue
- Painful and/or swollen joints
- Muscle pain
- Red rash on the face and/or in response to sitting in the sun
- Pain in the chest after taking a deep breath
- Unexplained fever
- Edema (swelling), often in the legs or around the eyes
- Mouth sores
- Unexplained hair loss
- Raynaud’s phenomenon, which is characterized by cold fingers and/or toes that are pale or purple in color.
In 1982, the American College of Rheumatology published a method for doctors to use for diagnosing lupus (Table 1). They then updated these criteria in 1997, and they have remained the same ever since. Lupus is generally diagnosed when a person exhibits four or more of these criteria.
Table 1. Eleven criteria used for the diagnosis of lupus, as defined by the American College of Rheumatology.
These criteria are based on the common lupus signs and symptoms. Lupus is diagnosed when any four or more criteria are present.15,16
|Malar rash||A red rash on the cheeks and the bridge of the nose; often called a "butterfly rash"||Physical exam, medical history|
|Discoid rash||Raised, hard patches of scaly skin||Physical exam, medical history|
|Photosensitivity||A red skin rash caused by exposure to sunlight||Physical exam, medical history|
|Oral ulcers||Sores in the mouth, usually painless||Physical exam, medical history|
|Nonerosive arthritis||Inflammation in one or more joints, making them feel tender and swollen. Cartilage, which is protective tissue surrounding the bone, remains intact||Physical exam, medical history, X-ray|
|Pleuritis and/or pericarditis||Inflammation of the lining of the lung or heart, respectively; may cause pain when breathing deeply; growing tired easily||Lung function test; chest X-ray to look for fluid in the lungs; cardiac stress test; echocardiogram, which uses sound waves to visualize the heart|
|Neurologic disorder||Reduced or abnormal brain function, headaches, seizures, memory loss, difficulty concentrating||Physical exam, medical history, brain MRI (magnetic resonance imaging); produces a high-resolution image of the brain.|
|Kidney disorder||Usually no symptoms; signs are blood or high levels of protein in the urine.||Urinalysis|
|Blood disorder||Anemia (low red blood cell levels) with associated fatigue, dizziness, shortness of breath; increased susceptibility to infection; slow clotting, excessive bleeding||CBC (complete blood count); test for abnormal cell counts of platelets, red blood cells, lymphocytes, and/or leukocytes|
|Immunologic disorder||Possible increased susceptibility to infection, inflammation in various organ systems||Assorted tests to detect antibodies from a blood sample.|
|Positive anti-nuclear antibodies||Possible increased susceptibility to infection, inflammation in various organ systems||ANA (antinuclear antibody) test; test for the presence of antibodies that bind the cell nucleus, which is where the DNA that make up genetic material is stored|
Doctors assess lupus severity by calculating a SLEDAI score, which stands for Systemic Lupus Erythematosus Disease Activity Index. Based on the presence or absence various lupus signs and symptoms over the proceeding 10 days, a total score is calculated. For example, protein present in the urine would result in 1 point, and another point would be added if a new rash had appeared. One point is assigned for each symptom or sign present and the greater the score, the more severe the disease at that time. A mild or moderate flare is defined as a change in SLEDAI score of 3 or more points; a severe flare is diagnosed when the SLEDAI score has increased by 12 or more points.15,16