Systemic Lupus Erythematosus is a chronic, usually life-long, potentially fatal disease with autoimmune background! In most cases- it is characterized by unpredictable exacerbations and remissions with protean clinical manifestations. What organs are affected? Well, in systematic lupus- there is a predilection for the joints, skin, kidney, brain, lug, heart and gastrointestinal tract. Everyone should know that SLE is a complex disorder affecting a predominately young population and have several similarities with HIV infection because of multiple organ involvement, potentially life-threatening episodes, and need for sophisticated and constant monitoring. Good thing to know is that the prognosis for patients with SLE has greatly improved over the last few decades and now- at least 80-90% of all patients is surviving ten years. Several researches done in the past have shown that preventive measures such as influenza and pneumococcal vaccination, TB testing, healthy diet, and exercise could improve health status of the patient!
Incidence of the condition
Several researches done in the past have confirmed the fact that women are disproportionately affected and SLE is most common in women of child-bearing age although it has been reported in almost any age! The prevalence in the Unites States had been estimated as approximately 500,000 but a recent research done by the Lupus Foundation of America suggested a prevalence of as many as 2,000,000. A recent study identified a prevalence of 500 per 100,000 in women residing in the area surrounding Birmingham, Alabama.
Possible causes of SLE
Unfortunately – the etiology of SLE is still unknown. However, most of the experts are saying that a genetic predisposition, sex hormones, and environmental triggers likely result in the imbalanced immune response which is the most characteristic sign of the disease.
A genetic predisposition
Several researches done in the past have found the increased percentage of two antigens in patients with SLE, HLA-DR2 and HLA-DR3 which is definitely the proof that the genetics may play a major part! The role for heredity is further supported by the concordance for this illness among monozygotic twins.
Autoimmune mechanism
Although some experts are saying different- the fact is that the origin of autoantibodies which are being produced in SLE is unclear. The fact is also that experts believe that a significant role could be in antigen driven process, spontaneous B-cell hyper-responsiveness, or impaired immune regulation. Problem is that nothing of this has been proven yet! More is known about the pathogenic cellular and molecular events which are responsible for vascular lesions in SLE than the origins of autoimmunity.
The health status of a patient with SLE is related not only to disease activity, but to the damage that results from recurrent episodes of disease flare as well as the adverse effects of treatment.
Symptoms of Systematic Lupus Erythematosus
Researches done in the past have shown that in more then 80% of patients- SLE will present with involvement of the skin or joints. What is the most common complain? Well, most doctors will tell you that a common presenting complaint is a photosensitive rash often with alopecia or baldness. However, patients may present with fever accompanied by single organ involvement, such as inflammatory serositis, glomerulonephritis, neuropsychiatric disturbance or hematological disorder.
The following classification of 11 symptoms should help doctors to tell the difference between people who have lupus and people who have other connective tissue disorders:
- Rash, which is extremely specific because it is a butterfly shaped rash over the cheeks and across the bridge of the nose
- Kidney problems (protein leak)
- Central nervous system problems
- Blood problems (anemia)
- Problems with the immune system (risk of infection)
- Discoid rash (scaly, disk-shaped sores on the face, neck and/or chest)
- Sensitivity to sunlight
- Oral ulcers
- Arthritis (pain, stiffness in joints)
- Serositis- inflammation of the lining around the heart, lungs, abdomen
- Antinuclear antibodies (autoantibodies that react against the body's own cells)
Other symptoms include:
- Muscle aches
- Nausea
- Vomiting and diarrhea
- Anemia
- Fatigue
- Fever
- Skin rash
- Swollen glands Lack of appetite
- Sensitivity to cold which is known as Raynaud's phenomenon
- Weight loss
Diagnosis of Lupus
X-rays imaging
Every patient should know that simple plain radiographs are not routinely useful in the diagnosis or management of SLE, but X-rays, however, can be used to evaluate symptomatic patients for necrosis, characteristic for this condition. Chest x-rays and chest CT scans could also be extremely useful in making difference between infectious and inflammatory lung disease.
Electrocardiograms - ECG
Logically- electrocardiograms (ECG) are useful in demonstrating changes characteristic to pericarditis and it may also be required to exclude myocardial infarction in SLE patients with chest pain.
Electroencephalogram (EEG), CT and MRI
Well, almost everyone could assume that electroencephalograms could be very useful in evaluating patients with possible neuropsychiatric lupus. Experts are saying that they are abnormal in 75% of patients with acute diffuse cerebral dysfunction. Brain CT scans are also nonspecific and have proven less sensitive than MRI in evaluating the central nervous system. MRI is now recognized as the most sensitive technique to identify bone necrosis.
Biopsies
Biopsies are also commonly required for the diagnosis of SLE. Occasionally, biopsies of the skin are useful to distinguish cutaneous manifestations of lupus from coincidental skin diseases. Renal biopsies are most useful when evaluated by light microscopy, immunofluorsence, electron microscopy, WHO classification, NIH activity and chronicity indices.
Treatment of Systematic Lupus Erythematosus
Management of SLE can be a challenge. Treatment depends on symptoms and their severity. Careful and frequent medical evaluation is therefore important for monitoring symptoms and adjusting treatment as necessary.
Conservative therapy –medications
Everyone should know that conservative treatment is appropriate for patients with muscle or joint pain, fatigue, skin manifestations and other features that are not life-threatening, which is extremely important! These conservative options include:
- Nonsteroidal anti-inflammatory drugs (NSAIDs) such as ibuprofen (Motrin, Advil)and naproxen (Naprosyn)
- Anti-malarial medications such as Hydroxychloroquine (Plaquenil).
More aggressive therapy is required for life-threatening and more serious manifestations such as kidney inflammation, lung or heart involvement, and central nervous system symptoms. Treatment in these circumstances might involve:
- high dose corticosteroids such as prednisone (Deltasone)
- other immunosuppressive drugs such as Azathioprine (Imuran), Cyclophosphamide (Cytoxan), and cyclosporine (Neoral, Sandimmune)
Prognosis for Lupus patients
Although the treatment for SLE has improved and long-term survival has increased significantly, it still remains a chronic disease that can limit activities.
Very often quality of life is compromised by symptoms like fatigue and joint pain, which are not life threatening. The best way to control lupus is:
- to be very careful to take all your medications as prescribed
- visit your physician regularly
- learn as much as you can about lupus and medications
Several researches done in the past have shown that simple maintaining an active lifestyle will usually help keep joints flexible and may prevent cardiovascular complications. It is also proven that patients with lupus should avoid excessive sun exposure because the ultraviolet rays can cause a skin rash to flare. That’s why- wearing simple protective clothing and using sunscreen when going out side should protect against such complications. There are several controversies about pregnancy and lupus condition! That’s why- experts are now saying that young women with lupus who wish to have a baby should carefully plan their pregnancies and of course- everything with the detailed guidance of doctor. Although it could be very complicated- good thing would be if they try to time their pregnancies for a period when the disease is least active. While carefully monitor the pregnancy, pregnant woman should also avoid certain medications such as Cyclophosphamide, cyclosporine, and mycophenolate.
Estrogen and lupus
There are several controversies about estrogen and lupus because of the possibility that the use of estrogen may induce or worsen lupus. Good thing is that some recent research has shown that estrogen can actually trigger some mild or moderate flares of lupus, but does not cause very severe exacerbation of symptoms. However, just to be sure and because estrogen can increase the risk of blood clots, it should be avoided in patients with lupus.