Systemic Lupus Erythematosus
Cause
Systemic Lupus Erythematosus occurs when the body identifies its own body tissue as non-self by mistake and starts making auto-antibodies against it. Inappropriate immune response leads to inflammation and thus causes damage to these body tissues.Symptoms
SLE may cause general symptoms such as fever, fatigue, mouth sores, hair loss, stomach pain, nausea and vomiting, abnormal heart rhythm, chest pain with deep breaths, headaches, seizures, difficulty breathing, and hemoptysis. SLE also causes a characteristic butterfly shaped rash on the nose and cheeks called malar rash with exposure to the sun. A raised oval discoid rash is also seen in some cases.Diagnosis
For diagnosis of SLE various tests are done including blood tests, urine analysis, chest X-ray and kidney biopsy. Blood tests are most important for the diagnosis and include complete blood cell count and antibody tests such as antinuclear antibody, anti-double strand DNA and anti-phospholipid.Treatment
There is no cure for SLE and treatment is aimed at managing the symptoms. Treatment is provided based on the type and severity of the symptoms and includes non-steroidal anti-inflammatory drugs (NSAIDS), anti-malarial drugs, corticosteroid and immune-suppressants. A drug belonging to a new class of drugs called biologics has been recently approved for treatment of mild to moderate SLE. SLE symptoms may go away completely or flare up suddenly. Thus, frequent and regular heath checkups are required to manage the disease.Systemic Sclerosis
Systemic sclerosis is an autoimmune disorder of the connective tissues where the body’s immune system mistakenly attacks its own cells. Systemic sclerosis is characterized by hardening of the skin, scar formation (fibrosis), blood vessel disease and inflammation associated with an over active immune system. The disease usually affects people between the ages of 30-50 years with women more prone to the condition than men.
Types of Systemic Sclerosis
Systemic sclerosis can be classified based on the location and involvement of internal organs into two types:
- Localized Systemic Sclerosis
- Systemic Sclerosis
Localized systemic sclerosis involves skin changes in isolated areas, either as morphea patches or linear systemic sclerosis. Morphea is localized patchy areas of skin that become hard and slightly pigmented and can sometimes cause multiple lesions on the skin. Linear Systemic Sclerosis is more common in children and features bands of hardened skin localized usually to the arm or leg on one side of the body. Localized systemic sclerosis is the milder form of the disease and will not progress to Systemic Sclerosis, a more severe form.
Systemic sclerosis affects connective tissues of the skin and also that of the internal organs such as the heart, lungs, kidneys and digestive tract. The tissues of the affected organs may become hard and fibrous decreasing their efficiency. Systemic sclerosis is also further categorized as limited or diffuse:
- Limited disease: This is a slower and more benign illness, typically confined to the fingers, hands, and face. It is also referred to as CREST syndrome which stands for Calcinosis, Raynaud phenomenon, Esophageal dysfunction, Sclerodactyly, and Telangiectasia.
- Diffuse disease: This type presents with rapid skin thickening progressing to skin hardening and a high risk of developing fibrous hardening of the internal organs including the lungs, heart, bowels, esophagus and kidneys. Diffuse disease is a very serious form and can ultimately be fatal.
Causes and Risk Factors
The cause of systemic sclerosis is unknown.
Research studies indicate certain inherited genes may be responsible along with exposure to environmental factors such as silica dust and paint thinners or exposure to certain viruses. It is known that people with systemic sclerosis develop extra collagen in the skin and organs which leads to the symptoms associated with the disease.
Symptoms
The symptoms depend upon the type of systemic sclerosis and which organs are affected such as the skin, heart, lungs, kidneys, and digestive tract. Commonly seen symptoms of systemic sclerosis include:
- Thick, tight skin on the fingers
- Sores (ulcers) on the fingertips or toes
- Hair loss
- Red spots on the hands and face
- Pain, stiffness, and swelling of the fingers and joints
- Calcium deposits in connective tissues
- Joint pain
- Raynaud’s phenomenon i.e. narrowing of blood vessels in the hands or feet
- Swelling of the esophagus, the tube between your throat and stomach
- Constipation or diarrhea
- Shortness of breath
- Dry cough and wheezing
Diagnosis
Systemic sclerosis may be diagnosed through the following tests:
- Blood tests: To assess elevated levels of certain antibodies produced by the immune system
- Tissue sample: A biopsy of the affected skin is taken for examination in the laboratory
- Skin assessment: This involves a thorough evaluation of the skin to assess for clues of involvement of the internal organs.
Depending upon the condition of the patient and the results of the initial evaluation the doctor may recommend further specific diagnostic tests. These tests may include chest X-rays, lung-function test, CAT scan of the lungs, EKG and echocardiograms.
Treatment
There is no cure for systemic sclerosis but rather symptoms are controlled through medications such as immunosuppressants, corticosteroids, vasodilators, and blood pressure medications.
The skin lesions associated with systemic sclerosis can be camouflaged through cosmetic procedures such as exposure to ultraviolet light or laser surgery.
Physical or occupational therapy may be recommended to manage pain and to improve your strength and mobility.
In some cases, such as finger ulcers in severe Raynaud’s disease, surgical amputation may be recommended. Similarly, patients with severe pulmonary hypertension may require lung transplants.
Patients with a mild form of systemic sclerosis may not require any treatment.
Complications
Possible complications related to systemic sclerosis are as follows:
- Heart failure
- Pulmonary hypertension
- Cancer
- Kidney failure from elevated blood pressure
- Esophageal stricture
- Malabsorption of food nutrients
- Pulmonary Fibrosis (scarring of the lungs)
Sjogren's Syndrome
Sjogren’s syndrome is an auto-immune disease caused by an attack of the immune system on the body’s glands that produce tears and saliva.
Causes and risk factors
The cause of the disease is not known. The presence of certain genes in an individual increases the risk of the disease. It is sometimes linked to other auto-immune diseases such as lupus and rheumatoid arthritis and is mostly seen in people above 40 years of age with women being more prone to it.
Symptoms
The main symptom of sjogren’s syndrome is dryness of the eyes and mouth. Other symptoms can include joint swelling and pain, vaginal dryness, persistent dry cough and dry skin. It may also spread to other parts of the body such as blood vessels, nerves, lungs, kidneys and digestive system.
Diagnosis
Dry eyes and mouth can occur due to many other diseases. Thus, diagnosis of Sjogren’s syndrome requires various tests. These include tests to confirm the decrease in the secretion of tears (shirmer test) and saliva (spit test) and blood tests to detect antibodies such as anti-nuclear antibodies, rheumatoid factor, anti-SSA and anti-SSB antibodies. Biopsy of the salivary gland in the inner lip is also obtained.
Complications
Dryness of the eyes may cause light sensitivity, blurred vision and in severe cases may lead to corneal ulcers. Similarly, severe dryness of the mouth can cause dental cavities and yeast infections in the mouth. Other rare complications may include effects on lungs, kidneys or liver function, numbness in hands and feet (peripheral neuropathy) and cancer of the lymph glands.
Treatment
Sjogren’s syndrome cannot be cured. Treatment is aimed at managing the symptoms. Dry eyes are treated by artificial tears and medication. In severe cases surgery may be advised to block or plug the tear ducts. Medications are also given for dry mouth to stimulate saliva production. Anti-fungal medication may be prescribed in case of yeast infection in the mouth. Immuno-suppressant and an anti-malarial drug are given to control wider range of symptoms.
Most patient’s with Sjogren’s syndrome live healthy lives. They should go for regular medical care and follow–up to prevent any complications.
Polymyalgia Rheumatica and Giant Cell Arthritis
Polymyalgia Rheumatica (PMR) is a rheumatic disorder of the joints and connective tissue characterized by muscle pain and stiffness in the shoulders, neck and hip areas.
Giant cell arteritis, also called temporal arteritis, is a condition that results in inflammation of the arteries of the scalp, neck and can also extent to major blood vessels originating from heart called aorta and its branches to arms. It often starts off with flu like symptoms and is characterized by headaches, pain and tenderness over the temples. Giant Cell Arteritis is a far more serious disease than Polymyalgia Rheumatica because it can cause heart attack, stroke and permanent blindness.
These two conditions are related as they frequently occur together, either at the same time or following resolution of symptoms of one of the disorders.
Causes and symptoms
The most common symptoms of polymyalgia rheumatica are muscle pain and stiffness in the neck, shoulders and hips. This is usually most noticeable in the morning and may occur after a period of inactivity. Other general symptoms include fever, weakness, anemia and weight loss.
People with giant cell arteritis usually start off with flu like symptoms such as fatigue, fever, and loss of appetite. Other symptoms that occur can include headache, pain and tenderness over the temples, double vision, dizziness or problems with coordination and balance. Giant cell arteritis may also cause pain in the jaw or tongue when you chew or open your mouth wide. Ulceration of the scalp occurs in rare cases.
The cause of both polymyalgia rheumatica and giant cell arteritis is not known. However, genetic predisposition, immune system problems and environmental factors seem to play an important role in development of these disorders.
Diagnosis
The diagnosis of polymyalgia rheumatica and giant cell arteritis is based on your medical history, physical examination and symptoms. Your doctor may suggest blood tests such as an erythrocyte sedimentation rate and C-reactive protein test to measure for inflammation in the body. Since the presence of inflammation could be due to other rheumatic diseases or arthritis, a rheumatoid factor test is also ordered to rule out rheumatoid arthritis. A negative rheumatoid factor test suggests a diagnosis of polymyalgia rheumatica.
Diagnosis of giant cell arteritis begins with a physical examination. If the temporal artery is tender to touch and has reduced pulse, this indicates an inflamed temporal artery that may be due to giant cell arteritis. The diagnosis is confirmed by temporal artery biopsy.
Treatment
There is no cure for polymyalgia rheumatica, but symptoms can be easily managed and controlled with medications.
Giant cell arteritis is a far more serious disease than polymyalgia rheumatica which if not treated promptly carries a small but definite risk of blindness. Therefore, treatment for giant cell arteritis is started as soon as it is suspected even before it is confirmed by the temporal artery biopsy.
Corticosteroids are the drug of choice for the treatment of both polymyalgia rheumatica and giant cell arteritis. The symptoms usually go away in 6 months to two years with treatment. Medication is then gradually reduced and stopped. The condition may reappear however and require medication again.
It is very important to note that corticosteroids should be taken strictly as advised by the doctor and should never be stopped abruptly as complications can occur. Your doctor will gradually decrease the medicine based on your test results and symptoms.
Polymyalgia rheumatica and giant cell arteritis are common, closely related inflammatory conditions that often occur together or one after the other. With careful monitoring and appropriate treatment, most people with polymyalgia rheumatica and giant cell arteritis can lead a normal active life.
Your doctor may also advice you on diet and supplements to reduce inflammation and avoid adverse effects of steroids.
Vasculitis
Vasculitis is an autoimmune disorder where the immune system attacks its own blood vessels causing inflammation. This results in narrowing or closing of the blood vessels. It may sometimes lead to stretching and bulging of the blood vessels (aneurysm) which may burst and bleed and can lead to death. Vasculitis can occur at any age and causes serious complications depending on which organ systems are involved.
Symptoms
Depending on the part of the body that is affected, symptoms will vary. The areas affected with vasculitis are listed below with their respective symptoms:
- Skin –various kinds of spots and bruises
- Joints – Arthritis
- Lungs – shortness of breath and sometimes blood with coughing (hemoptysis)
- Gastrointestinal tract – mouth sores, stomach pain and intestinal wall bleeding; in severe cases it can be fatal
- Nose and ear – sinus or nose ulcers and middle ear infection or hearing loss in some cases
- Eyes – redness, blurred vision, sensitivity to light and even blindness in rare cases
- Brain – headache, stroke-like symptoms and even paralysis in severe cases
- Nerves – Numbness in different parts of the body and sharp severe pain in the hands and legs
Other general symptoms can include fever, weight loss, aches and pain. The condition may suddenly go in remission or flare up in certain cases.
Risks
Vasculitis is mostly linked to other auto-immune disorders and may occur in patients suffering already with lupus, scleroderma or rheumatoid arthritis. It may also co-occur with leukemia and lymphoma. Smoking and hepatitis B and C infection also increases the risk for developing vasculitis.
Diagnosis
Diagnosis is done by the doctor based on the medical history, physical examination, symptoms and result of various diagnostic tests. Tests that the doctor orders to confirm vasculitis may include:
- Blood test for Erythrocyte sedimentation rate, C-reactive protein, hemoglobin and hematocrit and antineutrophil cytoplasmic antibodies
- Biopsy of the affected blood vessel or organization
- Blood pressure
- Urine-analysis
- ECG
- Echocardiography
- Chest X-ray
- Lung function test
- Abdominal ultrasound
- Angiography of affected blood vessels
Treatment
There is no cure for vasculitis therefore treatment is aimed at reducing pain and inflammation by prescribing non-steroidal anti-inflammatory drugs and corticosteroids. In severe cases cytotoxic drugs are given to kill the immune cells involved in inflammation. Rarely surgery is required to repair aneurysms that can occur.
Support of family and friends is equally important for the patient to deal with this chronic disease.
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