Ankylosing spondylitis

Description

• Seronegative spondyloarthropathies share common genetics and are characterized by seronegativity (rheumatoid factor negative); sacroiliitis and other axial inflammatory spine involvement; enthesitis (inflammatory changes of the attachment of tendon or ligament to bone) as the primary pathologic phenomenon; and extra-articular manifestations, including anterior uveitis, dermatitis, and colitis

• Ankylosing spondylitis typically presents in or before the fourth decade of life and affects men more than women (3:1 ratio)

• There is a genetic predisposition with a strong link to the HLA-B27 gene

• Insidious onset of chronic (more than 3 months) inflammatory low back pain that is sometimes worse at night and improves with exercise but not with rest is the cardinal historical feature

• Inflammatory sacroiliitis must be present to establish the diagnosis

• Over time, the disease will progress to involve the lumbar, thoracic, and cervical spine, with ascending stiffening correlating with spinal ligament calcification and characteristic radiographic changes; involvement of the thoracic region leads to decreased chest expansion and pulmonary symptoms

• Enthesitis is the primary pathologic feature, and peripheral tendonitis, plantar fasciitis, dactylitis, and costochondritis may be present; enthesopathic features may be axial or peripheral

• Asymmetric monoarthritis or oligoarthritis including involvement of the axial large joints (ie, hips and shoulders) and sometimes peripheral arthritis, usually of the lower extremities, is common

• Extra-articular features, including anterior uveitis (seen in 24%-40% of patients), conduction disturbances, aortitis and valvular heart disease, restrictive lung disease and upper lobe fibrosis, colitis, cauda equina syndrome, and C1-C2 subluxation, are seen in more than one third of patients and may be the presenting symptom or sign

• Physical examination findings may be normal early in the disease process, but later findings may include pain on sacroiliac joint maneuvers or direct palpation; decreased range of motion in the spine, hips, and shoulders; decreased chest expansion; dactylitis (sausage digits); tendonitis; and peripheral arthritis

• Radiographic changes indicative of sacroiliitis are key for establishing the diagnosis but may not be present in the first several years of disease; therefore, magnetic resonance imaging (MRI) of the sacroiliac joints may be more helpful for diagnostic purposes

• Other laboratory tests are usually not helpful, as results may be normal or show only nonspecific changes of inflammation. Although HLA-B27 antigen testing may yield a positive result, it does not confirm the diagnosis

Epidemiology

Prevalence:

• 0.1% to 1.4% depending on the population studied

• 5% to 6% in HLA-B27–positive persons

• Ankylosing spondylitis is found to be the etiology of chronic low back pain in 4% to 5% of patients

Demographics:

• Presents most commonly between the ages of 16 and 40, with a median age of onset of 23 years, although there can be a significant delay in diagnosis from the onset of symptoms

• Three times more frequent in men than in women

• More common in white patients, typically, but not universally, reflecting the associated prevalence of the HLA-B27 gene in this population

• Seven percent of the white population in North America is HLA-B27 positive, and more than 90% of white patients with ankylosing spondylitis in North America are HLA-B27 positive; the percentage of patients with ankylosing spondylitis who are HLA-B27 positive is lower in other populations

• Patients who are HLA-B27 positive have a 5% to 6% chance of developing ankylosing spondylitis, and patients who are HLA-B27 positive and have a first-degree relative with ankylosing spondylitis have a 10% to 20% of developing the disease. The HLA-B27 gene factor makes up approximately one third of the overall genetic risk

• Although lower socioeconomic status is not a risk factor, it portends a poorer outcome due to failure to seek medical assistance for back pain and resulting deformity

Anatomy

Ankylosing spondylitis is a systemic disease, meaning it can affect the entire body in some people. It can cause fever, loss of appetite, and fatigue, and it can damage other organs besides the joints, such as the lungs, heart, and eyes. Most often though, only the low back is involved.

The eye is the most common organ affected by ankylosing spondylitis. Eye inflammation (iritis) occurs from time to time in one-fourth of people with ankylosing spondylitis. Iritis results in a red, painful eye that also leads to photophobia, increased pain when looking at a bright light. It is a potentially serious condition requiring medical attention by an ophthalmologist. Luckily, it rarely causes blindness but it can affect vision while the inflammation is present.

Less frequently, ankylosing spondylitis may be associated with a scaly skin condition called psoriasis. In rare cases, typically when the ankylosing spondylitis has been present for many years, ankylosing spondylitis may cause problems with the heart or lungs. It can affect the large vessel called the aorta that moves blood from the heart into the body. Ankylosing spondylitis can cause inflammation where the heart and aorta connect leading to possible enlargement of the aorta.

Symptoms similar to that seen in ankylosing spondylitis may also may occur along with such conditions as psoriasis, inflammatory bowel disease, or Reiter’s syndrome. It is thought that bowel inflammation is somehow tied to the development of ankylosing spondylitis and this is the reason that people with inflammatory bowel disease, i.e. Crohn’s disease or ulcerative colitis are at an increased risk of the illness.

Causes:

The cause of ankylosing spondylitis is not known. Some cases may be associated with a problem in a specific gene.

Risk Factors

Factors that increase your chance of ankylosing spondylitis include:

• Family members with ankylosing spondylitis

• Having a marker on HLA-B27 gene

• Inflammatory bowel disease

• Ulcerative colitis

• Crohns disease

Symptoms:

The severity of symptoms can vary from mild to very severe.

Common symptoms may include:

• Stiffening and pain (arthritis) of the:

• Lower back

• Sacroiliac joint, where the back and hip meet, possibly radiating down the legs

• Pain that is often worse at night

• Stiffness that is worse in the morning

• Symptom improvement with exercise or activity

• Occasionally, pain and stiffness in other joints:

Knee

• Upper back

• Rib cage

• Neck

• Shoulders

• Feet

• Chest pain, which may suggest heart, heart valve, or lung problems

• Eye pain, visual changes, increased tearing

Less common symptoms may include:

• Fatigue

• Loss of appetite or weight loss

• Fever

• Numbness (if arthritic spurs compress the spinal nerves)

• blood in the urine or swelling from kidney disease

• irregular heart beat

•

Diagnosis

The doctor will ask about your symptoms and medical history. A physical exam will be done. Diagnosis is based on common symptoms of ankylosing spondylitis, such as:

• Dramatic loss of motion of the lower back and spine

• Pain in the lower back

• Limited chest expansion when taking deep breaths

Blood tests may be done to check for:

• HLA-B27 gene marker

• Abnormalities in the blood

• Signs of autoimmune disease

Images of involved joints may be taken with:

• X-ray

• MRI scan

• CT scan

Treatment

Medications

Medication may help to control pain and inflammation. They may include:

• Over-the-counter medication such as nonsteroidal anti-inflammatory drugs (NSAIDs)

• Prescription medication that suppresses the inflammation such as:

• Prescription NSAIDs

• Corticosteroids

• Disease-modifying antirheumatic drugs (DMARDS)

• Tumor necrosis factor (TNF)-inhibitors

The basics of treatment include:

• Education

• Attention to posture

• Exercise

• Medications

•

Should these approaches fail to provide adequate relief and should the spine, hips, knees or shoulders become damaged or painful, there are a number of reconstructive surgical procedures available, including spine surgery, total hip arthroplasty, total knee arthroplasty, or total shoulder arthroplasty.

Health care team

You should choose a doctor experienced in treating arthritis. Your doctor will work with you to decide when you need the help of other health professionals, such as physical or occupational therapists.

Effective treatment of ankylosing spondylitis relies on a partnership between you and your care providers.

Exercise and therapy

Regular exercise is an essential part of the overall management of ankylosing spondylitis. Your physical therapist with arthritis experience can design a program of exercises to meet your needs. Exercises that strengthen the back and neck will help maintain or improve your posture. Deep breathing exercises and aerobic exercises will help keep the chest and rib cage flexible. Swimming is an excellent way to exercise since it promotes flexibility of the spine; movement of the neck, shoulder, and hip joints; and deep breathing.

If you sometimes feel too stiff and sore to exercise, try taking a hot bath or shower to loosen up. Begin your exercises slowly and plan to do them when you are the least tired or have the least pain.

Whether your overall medical condition would permit an exercise regime would be a good topic for you to discuss with your internist, family doctor, or rheumatologist. The initiation of a fitness program in someone who has never participated in one before certainly should be done under the guidance of a physician or physical therapist.

Physical therapy is not believed to prevent progression of AS, but it may minimize symptoms in some patients.

Posture

Make every effort to keep your spine straight. Sleep on a hard mattress. Try to sleep on your stomach without a pillow under your head. You also can try sleeping on your back with a thin pillow or one that supports the hollow of your neck. Keep your legs straight rather than sleeping in a curled position. If you find it difficult to sleep in these positions, talk to a physical therapist about other possible options.

When walking or sitting, keep your spine as straight as you can with your shoulders squared and your head up. A test for correct posture can be done by standing with your back against the wall; your heels buttocks, shoulders, and head should be able to touch the wall all at once. Be sure that chairs and work surfaces are designed so that you don’t slump or stoop.

Corsets and braces, in general, are of little value in treating ankylosing spondylitis. You are much better off maintaining good posture by exercising properly

.

Medications

Medication is usually an essential and ongoing part of treatment. While medications do not cure ankylosing spondylitis, they do relieve pain and stiffness, allowing you to exercise, maintain good posture, and continue normal activities.

Several types of medication help treat ankylosing spondylitis. Nonsteroidal anti-inflammatory drugs (NSAIDs) reduce inflammation and relieve pain. Typical NSAIDs include indomethacin, piroxicam, or naproxyn. Side effects of NSAIDs include stomach upset leg swelling and rarely ulcers or bleeding from the stomach. Newer NSAIDs, known as the COX-2 inhibitors (rofecoxib, celecoxib) may be able to relieve inflammation and painwith fewer side effects. Aspirin has been found to be of little use in treating ankylosing spondylitis. Higher doses of NSAIDs are usually required to relieve the inflammation in addition to the pain.

A medication called sulfasalazine has been shown to reduce the inflammation and symptoms of ankylosing spondylitis, but it is not known whether sulfasalazine may slow or halt the progression of the disease. Some of the new medications that affect an inflammatory substance called TNF are being investigated as possible agents that may affect the course of theillness.

Whichever medications your doctor prescribes for you, be sure to take them as directed, even when you seem to be feeling fine. Also, talk to your doctor about possible side effects and what to do if they occur. If your symptoms worsen, call your doctor

.

Surgery

Surgery is a rare measure used in the management of ankylosing spondylitis. Joint replacement surgery is enabling many people to regain the use of joints that have been affected by ankylosing spondylitis and other forms of arthritis. Hip, knee, and shoulder replacements can be successful in ankylosing spondylitis. Rarely surgery can be done to straighten the spine but requires a significant expertise and should only be done by those with experience in the this area.

Strategies for coping

People who develop a chronic illness such as ankylosing spondylitis learn over time to cope with emotional ups and downs.

Learning to cope with ankylosing spondylitis often requires accepting changes. You may need to make changes in your relationships, work habits, and leisure-time activities. You may have to deal with changes in your appearance. All of these possible changes may leave you sad, stressed, depressed, or angry. Sometimes it helps to talk about these feelings with a family member, close friend, counselor, or someone else who has ankylosing spondylitis.

Asking for help

There may be times when you and your family are faced with problems caused by your disease that you do not know how to solve. You might want to talk to a counselor who has experience working with people who have arthritis. If so, your doctor probably can recommend one. It also may help to get to know other families who are living and coping with ankylosing spondylitis.

There are several organizations devoted to educating and supporting people with ankylosing spondylitis. One of these is the Spondylitis Association of America. In addition, to find a rheumatologist (physicians who specialize in treatment of spondylitis) or to learn what is new in the understanding or treatment of ankylosing spondylitis, contact the American College of Rheumatology.

Work

The majority of people with ankylosing spondylitis are able to continue a productive, active work schedule. Whether you work in or outside the home, the following suggestions may help. It may be helpful to discuss your work with your rheumatologist.

If your current job involves prolonged stooping or excessive strain on your back, you may want to contact a vocational rehabilitation agency in your state for guidance. The agency also may be able to help you if your experience, education, or training make it difficult for you to change jobs.

Family and friends

Most forms of arthritis do not limit one’s ability to enjoy romantic and sexual relationships. From time to time, however, problems such as pain and limited movement–especially of the hip joint–may get in the way of sexual enjoyment. Some extra planning may be all that’s needed.

One of the most important aspects of a good sexual relationship is good communication. If you and your partner can comfortably discuss each other’s needs, you probably can overcome almost any difficulty.

Adaptive aids

If some of your joints have fused or if you already have limited joint mobility, you may find it helpful to use some adaptive equipment or self-help aids. For instance, long-handled shoehorn or sock aids can help if your back or hips don’t bend easily.

When driving, always wear a seat belt with a shoulder harness and have the headrest in your car adjusted to support your neck. If a stiff neck or back makes backing into parking spaces difficult, try fitting your car with extra-wide mirrors.

Because your neck and spine may be hurt easily, avoid activities that could cause falling or produce a sudden impact. Talk to your doctor or occupational therapist about ways to avoid injury and ways to improve your ability to function.

There is no cure for ankylosing spondylitis. Treatment is aimed at providing education and relieving the symptoms.

Physical Therapy

Physical therapy may help prevent progression and worsening of symptoms. Treatment may include:

• Learning proper posture and the best positions for sleeping

• Exercise program that includes:

• Abdominal and back exercises (to decrease back stiffness and maintain good posture)

• Stretching exercises

• Water exercises

• Breathing exercises (in cases where the rib cage is affected)

•

Surgery

In severe cases, hip or joint replacement surgery may be needed. It will be done to relieve pain and help you move around easier. In some instances, spinal surgery is needed to allow an upright posture.

Prevention

There are no guidelines for preventing ankylosing spondylitis because the cause is unknown.

Typical advice includes:

• Learning correct body mechanics to maintain erect posture that counteracts the affects of kyphosis (the forward curve of the spine)

• Not using a pillow when you sleep, again to help prevent kyphosis

• Doing regular non-jarring exercises, such as swimming

• Maintaining high levels of activity

• Doing daily stretching exercises and deep breathing (for lung expansion)

• Avoiding prolonged bed rest and sitting