Systemic lupus Erythematosus (SLE), commonly also known asLupus is a chronic autoimmune inflammatory disease characterized by acute and chronic inflammation ofvarious tissues of the body, People with lupus produce abnormal antibodies (abnormal Defense System ) in their bloodthat target tissues within their own body rather than foreign infectiousagents, the immune system of the body mistakenly attacks healthy tissue. It canaffect the skin, joints, kidneys, lungs, brain, and other organs. Lupus is Latinfor “wolf”: the disease was so-named in the 13th century as the rashwas thought to appear like a wolf’s bite.
There are several kinds of Lupus:
• Systemiclupus Erythematosus (SLE) is the form of the disease that most people arereferring to when they say “lupus.” The word “systemic”means the disease can affect many parts of the body. The symptoms of SLE may bemild or serious.
• Discoidlupus erythematosus is a chronic skin disorder in which a red, raised rashappears on the face, scalp, or elsewhere. The raised areas may become thick andscaly and may cause scarring. The rash may last for days or years and mayrecur. A small percentage of people with discoid lupus have or develop SLElater.
• Subacutecutaneous lupus erythematosus refers to skin lesions that appear on parts ofthe body exposed to sun. The lesions do not cause scarring.
• Drug-inducedlupus is a form of lupus caused by medications. Many different drugs can causedrug-induced lupus. Symptoms are similar to those of SLE (arthritis, rash, fever,and chest pain) and they typically go away completely when the drug is stopped.
• Neonatallupus is a rare disease that can occur in newborn babies of women with SLE,Sjögren’s syndrome, or no disease at all.
Here I am going to discuss about SLE only
Fast Facts
Lupusis an autoimmune disease.
Lupusoccurs ten times more often in women than in men.
Treatmentdepends on the organs involved .
Involvementof the kidneys or/and the brain is the most serious manifestation of lupus.
Peoplecan live well with lupus if they actively work toward good health.
Sunexposure can lead to lupus flares. Cover your skin or use a sun-blocking cream,SPF 50 or greater.
Carefullyplan your pregnancies; lupus can flare during pregnancy and can affect its outcome.
Prevalence
SLE can occur in both males and females, More than 90% ofcases of SLE occur in women, frequently starting at childbearing age, and areaffected about ten times more often than men (women men ratio 10:1). While itmost commonly begins between the ages of 15 and 45, a wide range of ages can beaffected. Rate of SLE varies between countries from 20 to 70 per 100,000
Cause
The precise reason for the abnormal autoimmunity that causeslupus is not known. Inherited genes, viruses, ultraviolet light, and certainmedications may all play some role.
Signs and Symptoms
No two cases of lupus are exactly alike. Signs and symptomsmay come on suddenly or develop slowly, may be mild or severe, and may betemporary or permanent. Most people with lupus have mild disease characterizedby episodes — called flares — when signs and symptoms get worse for a while,then improve or even disappear completely for a time.
The signs and symptoms of lupus that a person experiencewill depend on which body systems are affected by the disease. The most commonsigns and symptoms include:
Patients may present with any of the followingmanifestations
Common symptoms include:
Patients may present with any of the followingmanifestations.
• Constitutional(eg, fatigue, low grade fever with no other cause, weight loss, Generaldiscomfort, uneasiness, or ill feeling (malaise).
• Musculoskeletal(eg, pain and swelling in joints with early morning stiffness, muscular pain).
• Mouthsores.
• Skin (eg,Skin rash: A “butterfly” rash develops in about half the people withSLE. The rash is mostly seen over the cheeks and bridge of the nose. It can bewidespread. It gets worse in sunlight, Sensitivity to sunlight, discoid lupus,Hair loss).
• Fingers andtoes that turn white or blue when exposed to cold or during stressful periods(Raynaud’s phenomenon)
• Kidney(eg, acute or chronic renal failure, acute nephritic disease)
• Brain andnervous system: Headaches, numbness, tingling, seizures, vision problems, andpersonality changes
• Lungs(eg, Chest pain when taking a deep breath, pleural effusion, pneumonitis,pulmonary hypertension, interstitial lung disease)
• DigestiveSystem (eg, nausea, vomiting ,dyspepsia, abdominal pain)
• Heart:Valve problems, inflammation of heart muscle.
• Bloodvessels: Blood vessels may become inflamed (vasculitis), affecting the wayblood circulates through the body. The inflammation may be mild and may notrequire treatment or may be severe and require immediate attention.
• Blood:People with lupus may develop anemia, leucopenia (a decreased number of whiteblood cells), or thrombocytopenia (a decrease in the number of platelets in theblood, which assist in clotting). Some people with lupus may have an increasedrisk for blood clots.
• Pregnancy: it can cause recurrent miscarriages.
In patients with suggestive clinical findings, a familyhistory of autoimmune disease should raise further suspicion of SLE.
Diagnosis
Lupus can be hard to detect because it is a complex diseasethat has many symptoms, and they can come on slowly. As experts in diagnosingand treating autoimmune diseases such as lupus, Rheumatologists can bestdetermine whether a patient has lupus and advise them about treatment options.
People with lupus often have symptoms that are not specificto lupus. These include fever, fatigue, weight loss, blood clots, and hair lossin spots or around the hairline. They may also have heartburn, stomach pain,and poor circulation to the fingers and toes. Pregnant women can havemiscarriages.
A diagnosis of lupus is made based on symptoms, a physicalexamination and blood tests. Tests can help to rule out other conditions.
If your doctor suspects you have lupus based on yoursymptoms, a series of blood tests will be done in order to confirm thediagnosis. The most important blood screening test is Antinuclear Antibody(ANA).The ANA test is commonly used to look for autoantibodies that attack thecomponents of cell’s nucleus, or ” command”center ,triggering autoimmune disorderlike Lupus. If ANA is negative, youdon’t have lupus. However, if ANA is positive, you might have lupus and willneed more specific tests. These blood tests include antibodies to anti-dsDNAand anti-Sm, which are specific to the diagnosis of lupus.
Treatment
At present, there is no cure for lupus. However, lupus canbe effectively treated with drugs, and most people with the disease can leadactive, healthy lives. Lupus is characterized by periods of illness, calledflares, and periods of wellness, or remission. Understanding how to preventflares and how to treat them when they do occur helps people with lupusmaintain better health. Treatment for lupus depends on signs and symptoms.
The goals of the treatment plan are to :
• Preventflares
Treat flares when they occur and reduce organ damage andother problems
• Treatmentsmay include drugs to
Reduce swelling and pain
• Preventor reduce flares
• Help theimmune system
• Reduce orprevent damage to joints
• Balancethe hormones
The medications most commonly used to control lupus include:NSAIDs, Antimalarial drugs, corticosteroids, immune suppressants and biologics.
Most people with lupus can live normal lives. Treatment oflupus has improved, and people with the disease are living longer. Here are acouple of tips that may help you when living with lupus:
Forma support system. A good doctor-patient relationship and support from familyand friends can help you cope with this chronic and often unpredictableillness.
Getinvolved in your care. Learn as much as you can about lupus, yourmedications, and what kind of progress to expect. Take all your medications asyour doctor prescribes, and visit your Rheumatologist often to prevent seriousproblems. This lets your doctor keep track of your disease and change yourtreatment as needed. If you do not live near a Rheumatologist, you may need tohave your primary care doctor manage your lupus with the help of aRheumatologist.
Stayactive. Exercise helps keep joints flexible and may prevent heartdisease and strokes. This does not mean overdoing it. Switch off doing light tomoderate exercise with times of rest.
Avoidexcess sun exposure. Sunlight can cause a lupus rash to flare and may eventrigger a serious flare of the disease itself. When outdoors on a sunnyday, wear protective clothing (long sleeves, a big-brimmed hat) and use lots ofsunscreen.
Ifyou are a young woman with lupus and wish to have a baby, carefully plan yourpregnancy. With your doctor’s guidance, time your pregnancy for whenyour lupus activity is low. While pregnant, avoid medications that can harmyour baby.
Dr. Mukhtar Masoodi is Consultant Rheumatologist, FlorenceHospital Chanpore Srinagar Kashmir.