What is a Rheumatologist?
A Rheumatologist is an internist or pediatrician who is specially trained to diagnose and treat arthritis and other diseases of the joints, muscles and bones. Many rheumatologists conduct research to determine the cause and better treatments for these disabling and sometimes fatal diseases.
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What do Rheumatologists treat?
Rheumatologists treat arthritis, certain autoimmune diseases, musculoskeletal pain disorders and osteoporosis. There are more than 100 types of these diseases, including rheumatoid arthritis, osteoarthritis, gout, lupus, back pain, osteoporosis, fibromyalgia and tendonitis. Some of these are very serious diseases that can be difficult to diagnose and treat.
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When should I see a Rheumatologist?
If musculoskeletal pains are not severe or disabling and last just a few days, it makes sense to give the problem a reasonable chance to be resolved. But sometimes, pain in the joints, muscles or bones is severe or persists for more than a few days. At that point, you should see your physician. Many types of rheumatic diseases are not easily identified in the early stages. Rheumatologists are specially trained to do the detective work necessary to discover the cause of swelling and pain. It’s important to determine a correct diagnosis early so that appropriate treatment can begin early. Some musculoskeletal disorders respond best to treatment in the early stages of the disease.Because some rheumatic diseases are complex, one visit to a rheumatologist may not be enough to determine a diagnosis and course of treatment. These diseases often change or evolve over time. Rheumatologists work closely with patients to identify the problem and design an individualized treatment program.
common conditions faq
What is rheumatoid arthritis?
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RA is a chronic disease that causes pain, stiffness, swelling, and limitation in the motion and function of multiple joints. Though joints are the principal body parts affected by RA, inflammation can develop in other organs as well. The stiffness seen in active RA is typically worst in the morning and may last anywhere from one to two hours to the entire day. This long period of morning stiffness is an important diagnostic clue, as not many other arthritic diseases behave this way. For example, osteoarthritis does not generally cause prolonged morning stiffness. While RA can affect any joint, the small joints in the hands and feet tend be involved more frequently than others. This produces a pattern of joint disease that rheumatologists regard as characteristic of RA.
Other symptoms that can occur in RA include:
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Loss of energy
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Low-grade fevers
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Loss of appetite
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Dry eyes and mouth from an associated condition known as Sjogren’s syndrome
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Firm lumps called rheumatoid nodules beneath the skin in areas such as the elbow and hands
Who gets rheumatoid arthritis?
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RA is the most common form of inflammatory arthritis. More than 2 million Americans suffer from RA. About 75 percent of those affected are women, and 1–3% of women may develop rheumatoid arthritis is their lifetime. The disease most often begins between the fourth and sixth decades of life; however, RA can develop at any age.
What causes rheumatoid arthritis?
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OA arises from problems with the cartilage that cushions the ends of bones. This slippery material serves as the body’s “shock absorber,” reducing friction in the joints as the body moves. When the cartilage is damaged or begins wearing away, tendons and ligaments can stretch painfully or, worse, bones can come into contact.
While no one factor appears to cause cartilage damage, researchers point to excess weight that adds to joint stress; sports- and work-related activities and injuries; and a family history of joint and cartilage weakness as contributing to OA. Age, in and of itself, is not a definitive cause of OA, but can worsen the deterioration process.
How is rheumatoid arthritis diagnosed?
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RA can be difficult to diagnose because it may begin gradually with subtle symptoms. Many diseases, especially early on, behave in a manner similar to RA. For this reason, patients suspected of having RA should be evaluated by a rheumatologist, a physician with the necessary skill and experience to reach a precise diagnosis and develop the most appropriate treatment plan. The diagnosis of RA is based on the symptoms described and physical examination findings such as warmth, swelling and pain in the joints. Certain laboratory abnormalities commonly found in RA can help in establishing a diagnosis. Tell-tale abnormalities include:
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Anemia (a low red blood cell count);
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Rheumatoid factor (an antibody eventually found in approximately 80% of patients with RA, but in only 30% at the start of the arthritis); and
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An elevated erythrocyte sedimentation rate or “sed rate” (a blood test that in most patients with RA tends to correlate with the amount of inflammation in the joints).
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X-rays can be very helpful in diagnosing RA but may not show any abnormalities in the first 3–6 months of arthritis. X rays are useful in determining if the disease is progressing.
It is important to remember that for most patients with this disease (especially those who have had symptoms for less than six months), there is no single test that “confirms” a diagnosis of RA. Rather, diagnosis is established by skillfully evaluating the appropriate symptoms, physical examination findings, laboratory tests and X-rays.
How is rheumatoid arthritis treated?
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Therapy for patients with RA has improved dramatically over the past 25 years. Current treatments offer most patients good to excellent relief of symptoms and the ability to continue to function at or near normal levels. Since there is no cure for RA, the goal of treatment is to minimize patients’ symptoms and disability by introducing appropriate medical therapy early on, before the joints are permanently damaged. No single therapy is effective for all patients, and many patients will need to change treatment strategies during the course of their disease.
What is osteoarthritis?
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Osteoarthritis (OA) is the most common joint disease affecting middle-age and older people. It is characterized by progressive damage to the joint cartilage—the slippery material at the end of long bones—and causes changes in the structures around the joint. These changes can include fluid accumulation, bony overgrowth, and loosening and weakness of muscles and tendons, all of which may limit movement and cause pain and swelling.
Most commonly affected are the weight-bearing joints—the knees, hips and spine. Osteoarthritis in the knee and hip areas can generate chronic pain or discomfort during standing or walking. Deterioration of disks between spine vertebrae can cause back and neck stiffness and pain.
OA also can affect the fingers and any joints with previous injury from trauma, infection or inflammation. Some patients may develop bony knobs or “nodes” that enlarge finger joints, causing pain, stiffness or numbness and later restrict use of the fingers.
Most of those individuals with OA do experience joint pain during activity which can be relieved by rest. Those with later stage OA may suffer more severe pain and unstable joints, causing a sensation in the knees of “giving way” or “locking.”
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Some OA patients also experience overall stiffness in the morning or after prolonged inactivity, such as riding in a car. This stiffness typically lasts no more than half an hour.
What causes osteoarthritis?
RA is the most common form of inflammatory arthritis. More than 2 million Americans suffer from RA. About 75 percent of those affected are women, and 1–3% of women may develop rheumatoid arthritis is their lifetime. The disease most often begins between the fourth and sixth decades of life; however, RA can develop at any age.
Who gets osteoarthritis?
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OA strikes people of all ages, but is more common in older populations. In fact, 70% of people over the age of 70 have X-ray evidence of the disease. However, only half ever develop symptoms. Women are affected more often than men, especially with OA of the fingers and the knees.
How is osteoarthritis diagnosed?
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OA is suspected when patients have pain in the commonly involved joints. It can be confirmed by physical examination showing bony enlargement, fluid accumulation, cracking sensation during movement, muscle weakness and joint instability. X-rays also are useful in making the diagnosis. Occasionally blood work may be necessary to rule out other diseases.
How is osteoarthritis treated?
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The goal of treatment is to reduce pain and improve function of the affected joints. This can be achieved with a combination of physical measures, drug therapy and, sometimes, surgery.
Physical measures – Exercise, support devices and thermal therapy usually are effective. Some forms of unproven alternative treatment such as spa, massage, acupuncture and chiropractic manipulation can help relieve pain for a short duration, but usually are costly and require repeated treatments.
Drug Therapy – Available forms of drug therapy include topical agents such as capsaicin cream, oral pain relievers such as acetaminophen, and nonsteroidal anti-inflammatory drugs (NSAIDs) for swelling and inflammation. For more serious pain, stronger medications such as narcotics may be required. Joint injections with corticosteroids or a form of lubricant called hyaluronic acid (HA) derivatives have proven effective for some patients.
Surgery – Arthroscopy and/or joint replacement is considered when the joint is seriously damaged, or the patient is in intractable pain and experiencing significant loss of function.
Supplements – Many nutritional supplements have been used for treatment of OA, but most lack good research data to support their effectiveness and safety. Recent study from the National Institute of Health showed that patients with moderate to severe pain from knee OA might benefit from chondroitin/glucosamine sulfate supplementation. However, to ensure safety and avoid drug interaction, consult your doctor or pharmacist before using any of these agents, especially in combination with prescribed drugs.
What is Lupus?
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Systemic lupus erythematosus (also called SLE or lupus) is a chronic inflammatory disease that can affect the skin, joints, kidneys, lungs, nervous system, and/or other organs of the body. The most common symptoms include skin rashes and arthritis, often accompanied by fatigue and fever. The clinical course of SLE varies from mild to severe, and typically involves alternating periods of remission and relapse.
What causes Lupus?
SLE is an autoimmune disorder which develops when the body’s own immune system, which normally protects against cancers and invading infections, begins to attack the patient’s own tissues (known medically as a “loss of self-tolerance”). This occurs first through the production of “auto-antibodies” (antibodies are immune system cells that attack foreign microbes; auto-antibodies attack a person’s own cells). As the attack continues, other immune system cells join the fight. This leads to inflammation, blood vessel abnormalities (vasculitis) and deposition of immune system cells in organs which causes tissue damage.
It is not known why this inflammatory reaction begins, but it probably occurs because of some combination of inborn or hereditary predispositions and environmental factors (such as viruses, the ultraviolet rays in sunlight, Silica dust, and allergies to medications). Recent research suggests that people affected by lupus may have a defect in the normal biological process of clearing old and damaged cells from the body, which then causes an abnormal stimulation of the immune system.
Who gets Lupus?
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SLE occurs 10 times more often in women than in men. The disorder typically develops in people in their twenties and thirties. SLE is more common in certain ethnic groups, particularly in blacks and Asians, who also tend to be more severely affected.
How is Lupus diagnosed?
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Diagnosis of SLE may be suspected on the basis of symptoms, but is confirmed by a series of blood tests. Of particular interest is the antinuclear antibody (ANA), which is present in virtually all the patients with lupus. Other tests such as the anti-double strand DNA (dsDNA) and anti-smith antibodies (Sm) are more specific and are used to confirm the diagnosis of lupus. The levels of certain complement proteins (a part of the immune system) are also used to help diagnose and monitor the disease.
If anti-phospholipid antibodies are present, this not only helps to establish a diagnosis of lupus, but also indicate there is an increased risk of specific complications. These include an increased risk of miscarriage and an increased risk for developing blood clots that may lead to stroke or lung injury.
Typical clinical features include:
• Fever, fatigue, and weight loss
• Arthritis involving multiple joints for several weeks
• Butterfly-shaped rash over the cheeks or other rashes
• Skin rash appearing in areas exposed to the sun
• Sores in the mouth or nose for more than a month
• Loss of hair, sometimes in spots or around the hairline
• Seizures, strokes and mental disorders
• Blood clots in different locations
• Miscarriages in some patients
• Blood or protein in the urine or tests that suggest poor kidney function
• Low blood counts (anemia, low white blood cells or low platelets)
Other symptoms include chest pain when the patient breathes deeply, heartburn, abdominal pain and poor circulation to the fingers and toes.
All of these symptoms can develop gradually, making lupus hard to diagnose. (See section below on the role of a rheumatologist.)
How is lupus treated?
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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 treatment is appropriate for patients with muscle or joint pain, fatigue, skin manifestations (such as rashes), and other features that are not life-threatening. Conservative options include nonsteroidal anti-inflammatory drugs (NSAIDs) such as ibuprofen (Motrin, Advil) and naproxen (Naprosyn) and 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) and other immunosuppressive drugs such as azathioprine (Imuran), cyclophosphamide (Cytoxan), and cyclosporine (Neoral, Sandimmune). Recently mycophenolate mofetil (CellCept) has been used to treat severe lupus kidney disease. Sometimes several medications must be combined to control the disease and prevent tissue damage.
Treatment depends upon an individual assessment of risks and benefits. Most immunosuppressive medications, for instance, may cause significant side effects such as increased risk of infections, nausea, vomiting, hair loss, diarrhea, high blood pressure, and osteoporosis. Rheumatologists may also reduce or discontinue a medication after the disease goes into remission for a period of time.
Clinical trials to evaluate new treatments are always being undertaken, with the hope that more promising drugs will be identified and made more widely available.
What is osteoporosis?
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Osteoporosis is a silent disease of the bones that makes them weaken and prone to fracture. Bone is living tissue that is in a constant state of regeneration, as old bone is removed (bone resorption) and replaced by new bone (bone formation). By their mid-30s, most people begin to gradually lose bone strength as the balance between bone resorption and bone formation shifts, so that more bone is lost than can be replaced. As a result, bones become thinner and structurally weaker.
The disease is “silent” because there are no symptoms when you have osteoporosis, and the condition may come to attention only after you break a bone. When you have osteoporosis, this can occur even after a minor injury, such as a fall. The most common fractures occur at the spine, wrist and hip. Spine and hip fractures in particular may lead to chronic pain, long-term disability and even death. The goal of treating osteoporosis is to prevent such fractures in the first place.
What causes osteoporosis?
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Many factors will increase your risk of developing osteoporosis and suffering a fracture. Some of these risk factors can be changed, while others cannot. Recognizing your own risk factors is important so that you can take steps to prevent this condition from developing or treat it before it becomes worse. Major risk factors include:
• Older age (starting in the mid-30s but accelerating after 50)
• Non-Hispanic white and Asian ethnic background
• Small bone structure
• Family history of osteoporosis or osteoporosis-related fracture in a parent or sibling
• Previous fracture following a low-level trauma, especially after age 50
• Sex hormone deficiency, particularly estrogen deficiency, both in women (e.g. menopause) and men
• Anorexia nervosa
• Cigarette smoking
• Alcohol abuse
• Low dietary intake or absorption of calcium and vitamin D
• Sedentary lifestyle or immobility
• Medications: glucocorticoid medications such as prednisone Prelone) (see fact sheet on glucocorticoid-induced osteoporosis); excess thyroid hormone replacement; the blood thinner heparin Calciparine, Liquaemin, etc.); certain anti-convulsant medications such as phenytoin (Dilantin) and ethotoin (Peganone), etc.
• Certain diseases can affect bone, such as endocrine disorders (hyperthyroidism, hyperparathyroidism, Cushing’s disease, etc.) and inflammatory arthritis (rheumatoid arthritis, ankylosing spondylitis, etc.)
Who gets osteoarthritis?
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Osteoporosis is more common in older individuals and non-Hispanic white women, but can occur at any age, in men as well as in women, and in all ethnic groups.
In the U.S., about 8 million women and 2 million men have osteoporosis. Those over the age of 50 are at greatest risk of developing osteoporosis and suffering related fractures. In this age group, one in two women and one in six men, will suffer an osteoporosis-related fracture at some point in their life. Non-Hispanic white and Asian people are most likely to experience osteoporosis and osteoporosis-related fractures. Hispanic and non-Hispanic black people can also develop osteoporosis and related fractures, but have a lower risk when compared to non-Hispanic whites and Asians.
How is osteoporosis diagnosed?
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A simple test that measures the bone mineral density (BMD) at different parts of your body, such as your spine and your hip, can help determine if you have osteoporosis. Dual energy x-ray absorptiometry (DEXA) is the best current test to measure BMD. The test is quick and painless; it is similar to having an x-ray taken, but uses much less radiation. Even so, pregnant women should not have this test done in order to avoid any risk of damaging the developing fetus.
The results of the DEXA test are scored in comparison to the BMD of young, healthy individuals, resulting in a measurement called a T-score. If your T-score is –2.5 or lower, you are considered to have osteoporosis and therefore at high risk for a fracture. T-scores between –1.0 and –2.5 are generally considered to show “osteopenia.” The risk of fractures is generally lower in people with osteopenia when compared with those with osteoporosis, but if bone loss continues, the risk for fracture increases.
How is osteoporosis treated?
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To maintain bone health:
• Make sure there is enough calcium in your diet (1000 mg per day of calcium for women before menopause and 1500 mg per day for women who are postmenopausal and are not taking estrogen replacement therapy).
• Get adequate vitamin D intake, which is important for calcium absorption and to maintain muscle strength (400IU per day until age 60, 600-800 IU per day after age 60).
• Get regular exercise, especially weight bearing exercise. A number of medications are also used for the prevention and treatment of osteoporosis:
• Bisphosphonates: Alendronate (Fosamax) and risedronate Actonel) are pills that need to be taken on an empty stomach with water. These medications help slow down bone loss and have been shown to decrease the risk of fractures.
• Calcitonin (Calcimar, Miacalcin): This medication is a hormone made from the thyroid gland and is usually given as a nasal spray or as an injection under the skin. It may help prevent spine fractures, and is also helpful to control pain after an osteoporotic vertebral (spine) fracture.
• Estrogen or Hormone Replacement Therapy: Estrogen therapy alone or in combination with another hormone, progestin, has been shown to decrease the risk of osteoporosis and osteoporotic fractures in women. However, the combination of estrogen with a progestin has been shown to increase the risk for breast cancer, strokes, heart attacks and blood clots. Estrogens alone may increase the risk of strokes. Given the complexity of this decision, consult with your doctor about whether hormone replacement therapy is appropriate for you.
• Selective Estrogen Receptor Modulators (SERMs): These medications mimic estrogens good effects on bones without some of the serious side effects such as breast cancer. Raloxifene Evista) decreases spine fractures in women, and is approved for use only in women at this time.
• Teriparatide (Forteo): Teriparatide is a form of parathyroid hormone that helps stimulate bone formation. It is approved for use in postmenopausal women and men at high risk for osteoporotic fracture. It is given as a daily injection under the skin and can be used for up to 2 years. If you have ever had radiation treatment to your bones or if you have parathyroid hormone levels that are already too high, you should not take this medication.
How to prevent osteoporosis?
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Lifestyle changes may be the best way of preventing osteoporosis:
• Make sure you are getting enough calcium in your diet roughly 1000-1500 mg/day, but will depend on your age)
• Make sure you are getting enough vitamin D (between 400-800 IU/day)
• Stop smoking
• Avoid excess alcohol intake
• Engage in weight-bearing exercises
• Treat underlying medical conditions that can cause osteoporosis
• Minimize or change medications that can cause osteoporosis; never stop taking any medication without speaking with your doctor first
• If you are at high risk for falls, consider using hip protectors e.g. SAFEHIP ®), which will help prevent a hip fracture if you fall
What is scleroderma?
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There are actually several types of scleroderma and related diseases with complications ranging from minor to life-threatening. Therefore, the terminology describing the various forms of scleroderma can be confusing.
The two broad categories are a) “localized scleroderma” which indicates distinct skin lesions and b) “systemic sclerosis” (scleroderma) which indicates more uniform skin involvement and the potential for internal organ disease. Other diseases that also affect the skin may be confused with scleroderma including scleredema, scleromyxedema, eosinophilic fasciitis, and nephrogenic fibrosing dermopathy.
Localized Scleroderma
Each of the several forms of localized scleroderma is a disorder of skin and sometimes the deeper tissues. The most visible effects of the disease are skin lesions often referred to as morphea. In some cases, this localized scleroderma is just a cosmetic problem. However, for those with more widespread skin lesions over their body (generalized morphea or linear scleroderma), in which thickness and scarring spreads down to the underlying structures including fat, muscle and, on rare occasion, bone, the disease can be more serious. Another pattern of localized scleroderma called en coup de sabre can particularly involve the face. Localized scleroderma including deep and extensive lesions can prevent normal motion of joints and interfere with daily activities. However, localized scleroderma does not affect internal organs of the body.
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Systemic scleroderma is divided into two sub-sets: a) “limited” cutaneous systemic sclerosis (scleroderma) in which skin involvement is limited to forearms, hands, legs, feet, and face; and b) “diffuse” cutaneous systemic sclerosis (scleroderma) which can affect the skin over almost any body area. The skin changes are caused by an increase of collagen and other proteins that lead to thickening and hardening of the skin. This accumulation also can occur in other organs including the lungs, the heart and the gastrointestinal tract. Internal organs involvement can include kidneys, lungs, heart, gastrointestinal tract, and the vascular system.
The earliest changes in the body in systemic scleroderma are:
• Activation of the immune system which may cause tissue damage, particularly in the lungs, as well as inflammation and swelling in the skin. When the disorder affects the immune system, causing injury to blood vessels and multiple organs, it becomes systemic sclerosis. The extent of skin and internal organ involvement dictates whether the diagnosis is limited cutaneous systemic sclerosis or diffuse cutaneous systemic sclerosis.
• Damage of blood vessels. For instance, Raynaud’s phenomenon, an abnormal reaction of blood vessels to the cold, is caused by structural damage of vessels in the hands, as well as elsewhere in the body. These changes then lead to fibrosis, or scarring, in multiple organs.
What causes scleroderma?
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The cause of scleroderma is not yet proven. Genetic factors appear to predispose patients to the disease, but whether scleroderma is the result of some combination of genetic factors and other exposures is unknown. For instance, some data suggests that exposure to industrial solvents or an environmental agent may play a role in predisposing to scleroderma. Scleroderma-like syndromes also have been clearly linked to agents as varied as contaminated rapeseed oil, polyvinylchloride, and a contaminant in one preparation of L-tryptophan. That said, the vast majority of patients with scleroderma do not have a history of exposure to any suspicious toxins.
Who gets scleroderma?
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Scleroderma is a relatively rare illness affecting only 75,000 – 100,000 people in the United States. Of these, 75% percent are women usually diagnosed between the ages of 30 and 50 years. Twins and family members of patients with scleroderma or other autoimmune connective tissue diseases, such as systemic lupus erythematosus, appear to be at a slightly increased risk. Children can get scleroderma, although the pattern and extent of disease may be different in children.
How is scleroderma diagnosed?
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A scleroderma diagnosis is based primarily on a combination of a person’s description of symptoms (history) and physical examination findings. Laboratory tests and x-rays may help in evaluating a patient with suspected scleroderma or find that someone actually has another disease, but no one test makes the diagnosis certain. For instance, blood tests for autoantibodies are often used in making the diagnosis, but the presence or absence of these antibodies is not, in and of itself, conclusive.
Scleroderma is diagnosed as the result of an unusual thickening or swelling of the skin, especially on the hands and extending up the arms, as well as dilated blood vessels in the face, hands, nail folds and elsewhere. Some patients develop cutaneous deposits of calcium (calcinosis), and characteristic involvement of other organs including the lungs, muscle inflammation, and the kidneys.
Almost all (more than 90%) of people with scleroderma also have Raynaud’s phenomenon. Many people with scleroderma also have additional heartburn and difficulty swallowing. However, since Raynaud’s phenomenon and heartburn can be caused by many other conditions, they are not specific disease indicators.
How is scleroderma treated?
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Unfortunately, while some treatments have proven effective in treating the disorder, scleroderma is not yet curable. Much research has gone into addressing the various manifestations of the disease, but no drug has been found that can arrest or reverse the skin thickening that is the hallmark of disease.
For patients with Raynaud’s phenomenon, a variety of drugs that dilate blood vessels (“vasodilators”) such as calcium channel blockers or alpha receptor blockers are available. Several newer vasodilators are now being considered for treatment of Raynaud’s phenomenon and digital ulceration in scleroderma, and we await more definitive data on these drugs’ effectiveness. Keeping one’s fingers and toes warm and adequately protected from exposure to cold, as well as keeping one’s core body (trunk) warm has proven effective in preventing attacks and subsequent damage. Protective measures to avoid trauma to the finger tips are also important.
Similarly, there are drugs that can alleviate the symptoms of heartburn and prevent damage to the esophagus, especially “proton pump inhibitors.” These drugs, coupled with physical measures such as elevating the head of the patient’s bed, can make a difference. Still other drugs can improve bowel function.
For those with scleroderma kidney disease, early recognition is critical. Therefore, patients with diffuse scleroderma should monitor their blood pressure several times a week. Early intervention with a type of blood pressure medication called angiotensin converting enzyme inhibitors (ACE inhibitors) has been shown to be extremely effective in treating early scleroderma renal involvement and helping to prevent renal damage. The use of these drugs has been a major advance for patients with scleroderma.
There are two main types of lung disease that some patients with scleroderma may develop: interstitial lung disease (inflammation and scarring) and pulmonary hypertension (increased pressure in the arteries of the lung).
Many experts in the field believe that cyclophosphamide is effective in treating the interstitial lung disease in scleroderma, and clinical trials are underway assessing the effectiveness of this drug and other agents for this condition. Pulmonary hypertension can occur in both the limited and diffuse forms of scleroderma. A number of agents have become available in the last 10 years to treat pulmonary hypertension, including prostacyclin-like drugs (epoprostenol, treprostinol) and the endothelin receptor antagonists (bosentan). These treatments, and those being tested in current research trials, may provide significant benefit to patients with lung disease in scleroderma.
For patients with weakness and muscle disease associated with scleroderma, glucocorticoids (such as prednisone) and/or immunosuppressive medications may be effective treatments.
Much research is ongoing into new treatments for scleroderma. Patients and their families should know that experts remain optimistic and work towards a cure will continue.
What is sjogren's syndrome?
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Sjögren’s syndrome is an inflammatory disease that can affect many different parts of the body, but most often affects the tear and saliva glands. Patients with this condition may notice irritation, a gritty feeling, or painful burning in the eyes. Dry mouth or difficulty eating dry foods, and swelling of the glands around the face and neck are also common. Some patients experience dryness of other mucous membranes (such as the nasal passages, throat, and vagina) and skin.
“Primary” Sjögren’s syndrome occurs in people with no other rheumatologic disease. “Secondary” Sjögren’s occurs in people who do have another rheumatologic disease, most often systemic lupus erythematosus and rheumatoid arthritis.
Most of the complications of Sjögren’s syndrome occur because of decreased tears and saliva. Patients with dry eyes are at increased risk for infections around the eye and may have damage to the cornea. Dry mouth may cause an increase in dental decay, gingivitis (gum inflammation), and oral yeast infections (thrush) that may cause pain and burning. Some patients have episodes of painful swelling in the saliva glands around the face.
Complications in other parts of the body occur rarely in patients with Sjögren’s syndrome. Pain and stiffness in the joints with mild swelling may occur in some patients, even in those without rheumatoid arthritis or lupus. Rashes on the arms and legs related to inflammation in small blood vessels (vascultis) and inflammation in the lungs, liver, and kidney may occur rarely and be difficult to diagnose. Neurological complications that cause symptoms such as numbness, tingling, and weakness have also been described in some patients.
What causes sjogren's syndrome?
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The cause of Sjögren’s syndrome is not known, but it is considered an autoimmune disorder. People with this disease have abnormal proteins in their blood suggesting that their immune system, which normally functions to protect the body against cancers and invading infections, is reacting against their own tissue. The decreased production of tears and saliva seen in Sjögren’s syndrome occurs when the glands that produce these fluids are damaged by inflammation. Research suggests that genetic factors and possibly viral infections (as yet unidentified) may predispose people to developing this condition.
Who gets sjogren's syndrome?
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Between 1 and 4 million Americans, or roughly 1 to 2 percent of the population, have Sjögren’s syndrome. This condition can affect people of any age, but symptoms usually appear between the ages of 45 and 55. It affects 10 times as many women as men. About half of affected patients also have rheumatoid arthritis or other connective tissue diseases, such as systemic lupus.Between 1 and 4 million Americans, or roughly 1 to 2 percent of the population, have Sjögren’s syndrome. This condition can affect people of any age, but symptoms usually appear between the ages of 45 and 55. It affects 10 times as many women as men. About half of affected patients also have rheumatoid arthritis or other connective tissue diseases, such as systemic lupus.
How is sjogren's syndrome diagnosed?
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Diagnosis depends on a combination of symptoms, physical findings, blood tests, and sometimes special studies. Dry eyes and mouth may be early signs of the condition but require further investigation because these symptoms can be caused by many other conditions or medications. Special tests may be used to assess any decrease in tear or saliva production (an example would be the Schirmer test for tear production. An eye examination is helpful in detecting any eye changes seen in Sjögren’s. Blood tests can determine the presence of antibodies (immune system cells that help destroy foreign invaders) typical of the disease, including anti-nuclear antibodies (ANA), anti-SSA and SSB antibodies, or rheumatoid factor. Biopsies of saliva glands around the face or under the surface of the inner lip may also sometimes be used to establish a diagnosis.
How is sjogren's syndrome treated?
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Treatment is designed to lessen the most bothersome symptoms. Dry eyes usually respond to the use of artificial tears applied regularly during the day or to gels applied at night. Other measures, such as plugging or blocking tear ducts, can be used in more severe cases. Eyedrops that reduce inflammation in the glands around the eyes (cyclosporine- Restasis) may be used to increase tear production. Dry mouth can be relieved by drinking water, chewing gum, or using saliva substitutes. Some patients benefit from using prescription medications that stimulate saliva flow, such as pilocarpine (Salagen) or cevimuline (Evoxac). If patients develop yeast infections, these can be relieved by anti-fungal therapies. The currently available treatments may help relieve some of the dryness but usually some dryness persists.All patients should receive regular dental care in order to prevent cavities and tooth loss that may occur as a complication of the disorder. Patients with dry eyes should see an ophthalmologist (eye doctor) regularly for signs of damage to the cornea. Patients with excessive redness and pain in the eyes should be evaluated for infections. Hydroxychloroquine (Plaquinel), an antimalarial drug used in lupus and rheumatoid arthritis, may be helpful in some patients with Sjögren’s syndrome by reducing joint pain and rash experienced by some patients. Patients with rare but serious systemic symptoms, such as fever, rashes, abdominal pain, or lung or kidney problems, may require treatment with corticosteroids such as prednisone (Deltasone and others) and/or immunosuppressive agents methotrexate (Rheumatrex), azathioprine (Imuran), mycophenolate (Cellcept), cyclophosphamide (Cytoxan).
What is psoriatic arthritis?
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Psoriatic arthritis is a form of arthritis associated with psoriasis, a chronic disease in which scaly red and white patches develop on the skin. Like rheumatoid arthritis, it results when the body’s immune system, which normally exists to protect against invaders, goes into overdrive and causes excessive inflammation.
Psoriatic arthritis can affect any joint within the body. When psoriasis affects fingernails (causing them to be pitted, thickened, or discoloured), the joints at the end of the fingertips are particularly likely to develop arthritis. Psoriatic arthritis can also cause a sausage-like swelling of fingers and toes, a condition known as dactylitis that can be confused with an infection.
Symptoms of psoriatic arthritis vary in nature and intensity, and may change in the same person over time. In some people, psoriatic arthritis affects the same joint on both sides of the body (e.g., both knees). In others, one joint is affected but not the other (e.g., one knee). Sometimes only the fingers and toes are affected; the spine may be affected, resulting in difficulty bending.
Like the skin disease it is associated with, psoriatic arthritis can cause symptoms that periodically show up, or flare, and then subside. Although psoriatic arthritis was originally thought to be mild and non-progressive (that is, that it would not worsen over time), recent data suggest that many of those affected will experience persistent inflammation and need treatment to prevent joint damage that can affect your ability to get day to day things done. Fortunately treatments are available and effective for most people.
Occasionally a condition known as spondylitis—involving pain in the back or neck—develops as a result of psoriatic arthritis. Another condition that may develop is enthesopathy, characterized by tender spots at sites in the body where tendons and ligaments join bones. This can cause symptoms such as pain at the back of the heel or in the sole of the foot.
What causes psoriatic arthritis?
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The exact cause of psoriatic arthritis is unknown, but researchers suspect that the disorder develops because of a combination of genetic (hereditary) and environmental (external) factors. As many as 40 percent of people with psoriatic arthritis have a family history of psoriasis or arthritis, indicating that they are prone to develop arthritis because of their genetic makeup, especially when their immune system is stimulated by an environmental trigger such as an infection. (Psoriasis itself is not infectious, but occasionally it can be triggered by a streptococcal throat infection.)
Who gets psoriatic arthritis?
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Psoriatic arthritis usually develops between ages 30 to 50, but can begin in childhood. Men and women are equally at risk. Only about 15 percent of people with psoriasis develop psoriatic arthritis. At times, the arthritis can develop before the onset of the skin disorder.
How is psoriatic arthritis diagnosed?
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Diagnosis of psoriatic arthritis is made on the basis of the disorder’s characteristic clinical features. Joints may be swollen and painful. Before a precise diagnosis can be made, skin and nail changes typical of psoriasis must be demonstrated, and occasionally skin biopsies (small samples of skin removed for analysis) are performed. Blood tests from some patients will reveal elevated erythrocyte sedimentation rate (ESR) (a blood test that measures inflammation), mild anemia, and elevated levels of uric acid. Other types of arthritis that cause similar signs and symptoms, such as gout and rheumatoid arthritis, must be ruled out.
How is psoriatic arthritis treated?
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Unlike rheumatoid arthritis, which usually requires constant treatment, psoriatic arthritis may only require therapy when symptoms arise. When they subside, therapy can be stopped until further problems develop. Initial treatment usually consists of non-steroidal anti-inflammatory drugs (NSAIDs) such as ibuprofen (Motrin or Advil) or naproxen (Naprosyn). If the arthritis does not respond, disease modifying anti-rheumatic drugs (DMARDs) may be used. These include sulfasalazine (Azulfidine), methotrexate (Rheumatrex) cyclosporine, (Neoral, Sandimmune) and the more recently available “anti-TNF agents” such as etanercept (Enbrel) and infliximab (Remicade) and adalimumab (Humira). The anti-malarial drug hydroxchloroquine (Plaquenil) may be effective, but some people taking this will experience a flare of their psoriasis, so it is usually avoided. Azathioprine (Imuran) may benefit those with severe forms of psoriatic arthritis. For severely swollen joints, corticosteroid injections can be useful at controlling the inflammation. Surgery can be helpful to repair or replace badly damaged joints.
What is spondyloarthritis?
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The term spondyloarthritis (also known as spondyloarthropathy) covers a group of closely related inflammatory diseases including arthritis of the spine (sacroiliitis or spondylitis) and peripheral joints; as well as inflammation in the area where ligaments and tendons attach to bones (enthesitis or enthesopathy). These diseases can cause pain in the spine, legs and arms as joints, ligaments, and tendons become inflamed and/or predispose patients to spinal vertebral fractures. Skin rashes, eye, and intestinal problems can also occur.
Diseases that fall under spondyloarthritis umbrella can include: 1) ankylosing spondylitis ; 2) reactive arthritis (known previously as Reiter’s syndrome ) 3) psoriatic arthritis and psoriatic spondylitis, and 4) the arthritis or spondylitis associated with the inflammatory bowel diseases, ulcerative colitis and Crohn’s disease. Still other patients may develop undifferentiated spondyloarthritis. This means they have symptoms or signs of one of the illnesses above, but don’t develop the full blown disease.
What causes spondyloarthritis?
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The exact cause of spondyloarthritis is unknown. However, researchers point to hereditary factors as playing an important role since these illnesses tend to occur more often in family members of patients who have spondyloarthritis. These patients usually share common genetic markers called HLA-B27, which occurs in about seven percent of the population.
Other infections, such as chlamydia (which can cause urethritis or burning on urination) and bacteria that cause intestinal dysentery (such as types of salmonella, shigella, etc.), can trigger a certain type of reactive arthritis that is a form of spondyloarthritis. Beyond these, no specific infection has been linked to other types of the disease.
Who gets spondyloarthritis?
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Spondyloarthritis tends to impact those in their teens and 20s, and young men two to three times more frequently than young women. (Psoriatic arthritis does affect young men and women equally). Family members of patients with spondyloarthritis run the highest risk of contracting these diseases, particularly those with HLA genes.
The highest frequency appears in the far north in cultures such as Alaskan and Siberian Eskimos and Scandinavians Lapps (Samis), as well as in certain Native America tribes in the western U.S. and Canada. African-Americans are least frequently affected. About one in 200 Caucasians have spondyloarthritis.
How is spondyloarthritis diagnosed?
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Diagnosis is made following a careful history and physical examination of inflammatory back pain or arthritis of the leg as it differs from other types of arthritis such as rheumatoid arthritis.
Additional tests such as X-rays of the sacroiliac joints and spine can confirm the presence of spondylitis. (Researchers are currently developing MRI scans that will also diagnose the disease). If symptoms and signs indicate, the physician will also check for the presence of the HLA-B27 gene.