The Pathology and Treatment of Lupus Erythematosus

Systemic lupus erythematosus, commonly known as lupus, is an autoimmune disease that results in tissue damage and inflammation all over the body. It is a hypersensitivity reaction that causes the immune system to create auto-antibodies that end up attacking the body’s own organs and damages the joints, kidneys, lungs, heart, and brain. Lupus can occur between alternating periods wherein the illness is active, known as flares, and wherein it subsides, as in remission.

At any time, an estimated 5 million people around the world are believed to have lupus. Studies show that women of childbearing age are up to nine times more likely to have lupus than men. In the 18th century, it was believed that lupus – Latin for wolf – was contracted from a wolf bite. Today, the exact cause of lupus still remains unknown, but many experts believe that genetics plays a role and that some people are innately prone to developing infections that may lead to lupus.

The symptoms of lupus are similar to those of the common illness, making it difficult to diagnose early. Lupus cases can range from mild to fatal. Experts have developed several kinds of diagnostic examinations to test for lupus, as well as different forms of medication and treatment for alleviating its symptoms and bettering the lives of those inflicted.

Causes of Systemic Lupus Erythematosus

The likely factors that may contribute to a susceptibility to lupus are genetics and the environment. Lupus erythematosus has been known to run in families and be influenced by several kinds of genes. The classes I-III of the human leukocyte antigen (HLA) gene system, where random mutations are likely to occur or become inherited, have all been linked to lupus. Other genes that increase risk are PTPN22, IRF5, STAT4, TNFSF4, CDKN1A, and ITGAM.

Known environmental triggers for lupus include sunlight and any source of ultraviolet radiation, which may stimulate an internal reaction and cause lesions on the skin, and different kinds of medication. Lupus brought about by taking certain types of drugs is known as drug-induced lupus erythematosus. Anticonvulsants and anti-seizure drugs – such as procainamide, phenytoin, hydralazine, and Isoniazid, as well as up to 400 more other types of drugs – can all lead to this condition.

The symptoms of drug-induced lupus often disappear once the patient is taken off of the trigger medication. Neonatal lupus is a very rare form that is brought on when a mother passes auto-antibodies to her child. This type of lupus breeds discoid, or cutaneous, lupus, which is limited in symptoms to the skin. The higher susceptibility of women to lupus suggests that the hormone estrogen may play a role in contracting the disease. Other potential risk factors are infections, colds or any types of viral sicknesses, and physical trauma or injury.

Symptoms of Systemic Lupus Erythematosus

Lupus erythematosus is part of the group of diseases referred to as “the great imitators”, because they copy the symptoms of other illnesses and are often mistaken for them. Patients go through the common illness symptoms of fever, fatigue, headache, chest pain, and breathing difficulties. Up to 30% of lupus cases present skin rashes and other dermatological symptoms. The malar rash is a key symptom and manifests in a butterfly-shaped red blotches on both cheeks on the face. Skin lesions also develop all over the body. Patients will also experience dry eyes and may have small tears on the tissues of their eyes after rubbing.

Raynaud’s phenomenon also occurs, wherein the tips of the fingers and toes turn blue or white during periods of great stress or when exposed to cold temperatures. Patients experience joint pain and swelling all over the body, as well as an inflammation on different regions of the heart and lungs, which both lead to further cardiac and pulmonary complications. Lupus also affects the nervous system, leading to hypertension, cognitive dysfunction, increased intracranial pressure, and psychosis. In certain cases, patients may also experience periods of confusion or severe disorientation. In pregnant mothers that experience a flare of the disease, lupus is likely to cause a spontaneous abortion.

Diagnosing Lupus

Lupus is often difficult to diagnose because of its similarity to other illnesses and in how its symptoms often vary for different people. The American College of Rheumatology has established eleven symptoms, wherein at least four must be met for a patient to be officially diagnosed with lupus. These symptoms are malar and discoid rashes, neurological and immunological disorders, renal disorder, anemia, photosensitivity, arthritis, mucosal ulcers, serotosis in the lungs and heart, and a positive antinuclear antibody test. A useful mnemonic for remembering these symptoms is SOAP BRAIN MD.

One of the typical tests that doctors perform to check for these symptoms is a complete blood count to test for anemia and monitor platelets and white blood cells. Doctors may also perform an erythrocyte sedimentation rate test to check how fast the patient’s red blood cells settle; a faster than normal rate indicates infection or inflammation. The lupus band test uses direct immunofluorescence to check for any antinuclear antibodies in the patient’s blood.

A biopsy can test tissue samples from different organs to determine if they have experienced any damage and what the extent of it may be. A chest X-ray and an echocardiogram can be used to test for pulmonary and cardiac inflammation. Doctors also usually assess the patient’s kidneys and liver to test how well they operate. A urine analysis that shows high levels of protein or red blood cells usually indicates a positive lupus diagnosis.

 Treatment Options

As of yet there is no cure for lupus, and any present forms of treatment are targeted toward suppressing occurrences of flares. Corticosteroids, such as prednisone, are able to counter the inflammatory symptoms of lupus. Nonsteroidal anti-inflammatory drugs are similarly used to treat swelling and inflammation, as well as fever. Anti-malarial drugs, like Plaquenil, can also relieve symptoms. Immunosuppressant drugs, such as Cytoxan, Cellcept, Arava, and Imuran, can subdue the immune system and prevent it from initiating an autoimmune response.

Antibody production is treated through intravenous immunoglobulin therapy, which is a recommended form of treatment, as it does not put the patient at risk for any harmful side-effects. Renal transplantation is the prescribed treatment for kidney failure, which develops as a complication of lupus nephritis. Other measures patients can take to control Lupus are to minimize exposure to the sun or to any strong sources of indoor lighting. It is also important for them to lead a healthy lifestyle, with constant exercise, a healthy diet, and proper amounts of rest. Alternative forms of medication that help fight inflammation and infection include dehydroepiandrosterone supplements, fish oil, flaxseed, and copious amounts of vitamin D.

Further research about the disease

The current goal of lupus research is to find both a definitive cause and a cure. Other studies are targeted toward improving treatment for patients and in helping to alleviate the debilitating symptoms of the disease. A medical study named BLISS-76 focused on the drug Belimumab that has successfully reduced lupus symptoms in patients by stimulating lymphocytes to attack foreign invaders. Another new study by the Lupus Foundation of America, called UNVEIL, sheds light on how patients and their families deal with everyday life under the influence of the disease.