SLE is undoubtedly a potentially serious illness with involvement of numerous organ systems. However, it is important to recognize that most patients with SLE lead full, active, and healthy lives. Periodic increases in disease activity (flares) can usually be managed by varying medications. Since ultraviolet light can precipitate and worsen flares, patients with systemic lupus should avoid sun exposure. Sunscreens and clothing covering the extremities can be helpful. Abruptly stopping medications, especially corticosteroids, can also cause flares and should be avoided. Patients with SLE are at increased risk of infections, especially if they are taking corticosteroids or immunosuppressive medications. Therefore, any unexpected fever should be reported and evaluated.
The key to successful management of SLE is regular contact and communication with the doctor, allowing monitoring of symptoms, disease activities, and treatment of side effects.
Symptoms vary greatly from person to person. Symptoms may begin suddenly with fever, resembling a sudden, severe (acute) infection. Or symptoms may develop gradually over months or years with episodes (called flare-ups) of fever, feeling unwell, or any of the symptoms discussed below alternating with periods when symptoms are absent or minimal.
Migraine-type headaches, epilepsy, or severe mental disorders (psychoses) may be the first abnormalities that are noticed. Eventually, however, symptoms may affect any organ system.
Although nervous system involvement in systemic lupus erythematosus (SLE) is unclear and controversial, people with lupus do often experience signs associated with the body’s nervous system, such as:
- difficulty with concentration
- occasional seizures or strokes
Joint Problems: Joint symptoms, ranging from intermittent joint pains (arthralgias) to sudden inflammation of multiple joints (acute polyarthritis), occur in about 90% of people and may exist for years before other symptoms appear. In long-standing disease, marked joint deformity may occur (Jaccoud’s arthropathy) but is rare. However, joint inflammation is generally intermittent and usually does not damage the joints.
Skin and Mucous Membrane Problems: Skin rashes include a butterfly-like redness across the nose and cheeks (malar butterfly rash); raised bumps or patches of thin skin; and red, flat or raised areas on the face and sun-exposed areas of the neck, upper chest, and elbows. Blisters and skin ulcers are rare, although ulcers do commonly occur on mucous membranes, particularly on the roof of the mouth, on the inside of the cheeks, on the gums, and inside the nose. Generalized or patchy loss of hair (alopecia) is common during flare-ups. Mottled red areas on the sides of the palms and up the fingers; redness and swelling around the nails; and flat, reddish purple blotches between the knuckles on the inner surfaces of the fingers also may occur. Purplish spots (petechiae) may occur because of bleeding in the skin as a result of low platelet levels in the blood. Sensitivity to sunlight (photosensitivity) occurs in most people with lupus, particularly fair-skinned people.Lung Problems: It is common for people with lupus to feel pain when breathing deeply. The pain is due to recurring inflammation of the sac around the lungs (pleurisy), with or without fluid (effusion) inside this sac. Inflammation of the lungs (lupus pneumonitis), resulting in breathlessness, is rare, although minor abnormalities in lung function are common. Life-threatening bleeding into the lungs may rarely occur. Blockage of arteries in the lung caused by the formation of blood clots (thrombosis) can also occur.
Heart Problems: People with lupus may have chest pain due to inflammation of the sac around the heart (pericarditis). More serious but rare effects on the heart are inflammation of the walls of the coronary arteries (coronary artery vasculitis), which can lead to angina (see Coronary Artery Disease: Angina), and inflammation of the heart muscle with scarring (fibrosing myocarditis), which can lead to heart failure (see Heart Failure). The valves of the heart can rarely be involved and may need to be surgically repaired. People are at increased risk of coronary artery disease.
Lymph Node and Spleen Problems: Wide-spread enlargement of the lymph nodes is common, particularly among children, young adults, and blacks of all ages. Enlargement of the spleen (splenomegaly) occurs in about 10% of people. People may experience nausea, diarrhea, and vague abdominal discomfort. The occurrence of these symptoms may be the forewarning of a flare-up.
Nervous System Problems: Involvement of the brain (neuropsychiatric lupus) can cause headaches, mild impairment of thinking, personality changes, stroke, epilepsy, severe mental disorders (psychoses), or a condition in which a number of physical changes may occur in the brain, resulting in disorders such as dementia. The nerves in the body or spinal cord may also be damaged.
Kidney Problems: Kidney involvement may be minor and without symptoms or may be relentlessly progressive and fatal. The most common result of this impairment is protein in the urine that leads to swelling (edema) in the legs.
Blood Problems: The numbers of red blood cells, white blood cells, and platelets may decrease. Platelets assist in blood clotting, so if these numbers decrease greatly, bleeding may occur. Also, and for other reasons, the blood may clot too easily, which accounts for many of the problems that can affect other organs (such as strokes and blood clots to the lungs or recurrent miscarriages).
Gastrointestinal Tract Problems: Impairment of blood supply to various parts of the gastrointestinal tract may result in abdominal pain, damage to the liver or pancreas (pancreatitis), or a blockage or tear (perforation) of the gastrointestinal tract.
Pregnancy Problems: Pregnant women have a higher-than-normal risk of miscarriage and stillbirth.
Fibromyalgia (Fibrositis Syndrome)
Up to 20 percent of people with SLE have a simultaneous fibromyalgia (fibrositis) syndrome manifested by tender points and increased pain in the soft tissues. In addition, patients may experience:
- cognitive dysfunction
- decreased ability to concentrate
- difficulty sleeping
- lack of stamina.
People with lupus experience headaches which are unrelated to their lupus, i.e., sinus headache, tension headache and bone spurs from osteoarthritis. Approximately 20 percent of patients with SLE experience severe headaches which are related to the disease and known as lupus headache.
- The lupus headache phenomenon is similar to migraine and may be seen more often in people who also have Raynaud’s phenomenon.
- SPECT scans indicate abnormalities in blood vessel tone or the ability of a vessel to dilate or constrict.
- Lupus headache is treated like tension headaches or migraine, although corticosteroids are occasionally useful.
Signs And Symptoms of Central Nervous System Lupus
Central Nervous System (CNS) Vasculitis
CNS vasculitis is inflammation of the blood vessels of the brain. It is the most serious form of systemic lupus. CNS vasculitis is characterized by:
- high fevers
- meningitis-like stiffness of the neck.
It can rapidly progress to stupor and coma if not aggressively managed. Seizures occur when injured or scarred brain tissue becomes the focus of abnormal electrical discharges.
- These seizures may be a one-time occurrence or a persistent problem.
- Anti-convulsant medications are used to prevent seizures by controlling the brain’s abnormal electrical discharges