Systemic lupus erythematosus (SLE) or Lupus is an autoimmune inflammatory disease in which the bodies own immune system attacks its own tissues. The word Lupus comes from Latin meaning “wolf”. It became known as lupus because the butterfly facial rash resembles the facial markings on a wolf. Symptoms are different in different people and can range from mild to severe. The disease can have periods of flair ups and low activity. The disease flairs can come anytime and it can involve any organ.
Signs and symptoms include:
- Joint pains
- Rash (facial, butterfly rash, sun sensitivity)
- Muscle aches
- Weight loss or gain
- Hair loss
- Kidney disease
- Gastrointestinal symptoms
- Pulmonary (pleuritis, fluid buildup, pneumonia)
- Cardiac disease (pericarditis)
- Enlarged lymph nodes
- Enlarged spleen, liver
- Depression, anxiety
Lupus is more common in African Americans, Asians, and Hispanics vs. Caucasians in the US. It’s seen more common in higher dense cities and less in rural areas. More than 65% of people with SLE develop it between the ages of 16 and 55. Only 15% of patient’s develop SLE after age 55. African Americans and Hispanics have more severe kidney disease from lupus than Caucasians. They’re also less likely to respond to treatments for lupus nephritis than Caucasian patients. Disease outcomes are also worst in lower educated and poorer patients. This could be due to lower compliance with medications and less access to care. Men with lupus tend to get more kidney problems, skin lesions, hypertension, cardiovascular and neurologic problems as compared to women. Women tend to get Raynaud’s phenomenon more often. Raynaud’s phenomenon is excessive constriction of blood vessels (usually in hands) in response to stress and cold temperatures.
The main cause of lupus is not known but researchers believe it results from a combination of factors like genetics, hormones, environmental and immune changes. Genetics are usually not the sole cause for the development of lupus, usually a environmental trigger like a viral infection can trigger its development. Infections such as EBV (causes mono), herpes zoster (cause of shingles) and CMV have been implicated. Antibodies produced by the body against the viruses can attack our own tissue by mistake due to similar receptors.
Lupus is much more common in women suggesting that estrogen and other hormones may play a role in its development. Hormones such as estrogen, testosterone, and progesterone affect the activity of the immune system. Estrogen activates lymphocytes, macrophages and stimulates the release of cytokines like IL-1. It prolongs the life span of B cells, which increases the chances for autoimmune diseases.
Fever, fatigue and weight loss are present in most people who have lupus. Fatigue can be disabling sometimes and is not necessarily related to the severity of the disease. Sleep disturbance, depression and increased pain can also occur along with fatigue from lupus. A healthier psychological and social outlook can decrease these symptoms. Fever is seen in half of the patients, especially when the disease is active. This can confuse doctors sometimes because it becomes harder to find out weather the fever is from the lupus or from other causes like infection or cancer.
Joint pains and arthritis is seen in up to 95% of people with lupus. They may be one of the first signs of lupus. The pain usually moves from one joint to the other and often after a day. The joints of the knees, fingers, wrist and knuckles are usually involved. Other less common joints include ankles, elbows, shoulders, hips and neck. Some morning stiffness may also be present but it usually doesn’t last long.
Osteoporosis is a significant problem in people with lupus. The loss of calcium and minerals occurs more in the spine and ribs than extremities in people with lupus. Calcium, vitamin D, and medications like Fosamax are important in preventing fractures in people who develop osteoporosis.
Fibromyalgia also occurs with increase rates in people who have lupus (5-25% of patients). It significantly affects their quality of life and causes more debility. Lot of the medications for lupus like steroids and immunosuppressants can lead to fibromyalgia like symptoms.
Weight changes can occur in association with lupus, either due to the disease itself or its treatment. Weight loss usually occurs due to decreased appetite, medication side effects or from GI diseases like GERD, stomach ulcers. Hydoxychloroquine, an anti-malaria drug commonly used for lupus can cause stomach cramps, weight loss, diarrhea, nausea and vomiting. Steroids like Prednisone can lead to weight gain when used for long periods.
The rash which occurs with lupus is known as the butterfly rash when it occurs on the face. It usually gets worst with sunlight. It’s located on the cheeks and nose area. Some people develop discoid lupus, which involves more skin inflammation and scarring. Vasculitis, which is inflammation around blood vessels, can show up as different types of rashes in lupus.
Venous and arterial blood clots also occur more commonly with SLE especially in those with antiphospholipid antibodies. Antiphospholipid syndrome (APS) is a multisystem disorder with increased risk of blood clots and the presence of antiphospholipid antibodies in the blood. APS is sometimes diagnosed in females with frequent miscarriages which is one its signs. Lupus patients have about 10-26% chance of developing venous or arterial clots. Medications like hydroxychloroquine are sometimes used to prevent blood clots in patients with lupus.
Kidney disease occurs in about half the patients with lupus and can cause significant burden. People with lupus may have increased protein or blood in the urine. The kidney filtration function may also be decreased which means a higher blood creatinine level. Lupus nephritis is the term used to describe the inflammation involving the kidneys in people with lupus. Without treatment, lupus nephritis can lead to kidney failure. A kidney biopsy is usually recommended for lupus nephritis to confirm the diagnosis and to help guide treatment.
Gastrointestinal symptoms are common in patients with lupus. It can present with difficulty or painful swallowing, chest pain, heartburn, stomach ulcers, abdominal pain, bloating, bowel obstruction, diarrhea, pancreatitis and hepatitis.
Lung involvement can include pleuritis, which is inflammation around the lungs. Pleuritis can cause chest pain with deep breathing. Other symptoms include cough, shortness of breath and fever. Fluid can accumulate in the chest cavity due to inflammation and sometimes needs to be removed if excessive.
Heart disease can develop with increased frequency in people with SLE. Increased inflammation, oxidative stress and long-term use of steroids can increase risk for heart disease in lupus. It’s important to recognize the important risk factors involved and work on prevention. Lot of people with lupus are not aware of this increased risk for heart disease. Common lifestyle recommendations include no smoking, regular exercise, weight management, and healthy diet. Cholesterol lowering medications like statins are used to help control lipids and inflammation. Atorvastatin and Fluvastatin are the preferred statins since they don’t require dose adjustment with kidney disease. The doctor may also recommend a baby aspirin. Hydroxychloroquine has been shown to have positive effects on the cardiovascular system in lupus patients. Lastly, blood pressure should be managed properly since it’s a major cause of heart disease.
There are also psychiatric conditions that occur more frequently in patients with SLE. These include cognitive dysfunction, headaches, depression, anxiety, changes in personality, irritability, emotional instability, seizures and psychosis. These disease processes can occur directly due to the disease or from medications like prednisone and other steroids.
Eye diseases such as dry eyes, optic neuropathy and retinal disease can develop. Keratoconjunctivitis sicca is the development of dry eyes due to low tear production from the tear glands.
Anemia is common in SLE and can occur due to prolonged inflammation, medication side effect, low iron, low B12 or autoimmune destruction. The doctor can get a blood test to evaluate for anemia. White cell count can also be low especially when the disease is active. An enlarged spleen which can occur in some people can be a cause of low white cells since the spleen will hold on to these white cells. Other causes include viral infections like mononucleosis, CMV and side effects from medications. Platelet counts can also be lower in patients with lupus.
People with lupus should see a Rheumatologist regularly to monitor the disease and prevent complications. A healthy diet with plenty of fruits and vegetables, regular exercise, and stress management all play an important role in prevention and controlling the disease.
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