University of Iowa Family Practice Handbook, Fourth Edition, Chapter 7

Rheumatology: Spondyloarthropathies

Characterized by involvement of spine and entheses (insertions of tendons and ligaments). Associated with HLA-B27. The target organs are not only the joint, but also the axial skeleton, the enthesis, the eye, the gut, the urogenital tract, the skin, and sometimes the heart. The prevalence of this entity in the general population is estimated at 1%, equal to the prevalence of rheumatoid arthritis. Diseases included within this concept are ankylosing spondylitis, reactive arthritis/Reiter's disease, undifferentiated spondyloarthropathies, some forms of psoriatic arthritis, juvenile chronic arthritis, acute anterior uveitis, and arthritis associated with inflammatory bowel diseases such as Crohn's disease and ulcerative colitis, as well as infectious diarrhea such as Yersinia.

1. Ankylosing Spondylitis (Marie-Strumpell disease).
   1. Clinical features. A disease primarily affecting the sacroiliac joints with varying involvement of the spine and less so the appendicular skeleton.
      1. Clinically. Onset is usually insidious, generally between 10 and 40 years of age. Patients generally have back pain that is worse after rest and improves with exercise. Patients may notice morning back stiffness that improves during the day; the pain may be so severe at night that it keeps the patient awake or prompts the patient to get up and become mobile to reduce the symptoms. By definition, the back is always involved. However, peripheral joints are involved in up to 25% of cases. Proximal, large joints, such as hips, knees, shoulders, and ankles, are preferentially affected. TMJ involvement is common. Involvement is usually asymmetric but not always. May have mild systemic manifestations such as fever, malaise, or anorexia.
      2. Associated with HLA-B27 in >90% of cases. The diagnosis is made according to clinical and radiologic criteria. Offspring of those with the disease have a 10% to 20% risk of having disease.
      3. Enthesopathy. Involvement of the sites of insertion of ligaments and tendons (entheses) is manifested clinically as Achilles tendinitis, plantar fasciitis, and costochondritis.
      4. Extraarticular manifestations. May include uveitis (25%), cardiac involvement in 10%, especially aortic insufficiency, cardiomegaly, conduction defects, heart block, and renal and neurologic complications. Pulmonary involvement consists principally of upper lobe fibrocystic changes and chest wall restriction but may include Aspergillus infection in the pulmonary cavities.
      5. Late complications secondary to bone involvement can include cord compression caused by spinal fractures; cauda equina syndrome (neurogenic bladder, fecal incontinence, leg pain); and severe chest wall restriction.
   2. Diagnosis.
      1. Physical examination.
         1. Flexion test. Mark two points on the back, at the lumbosacral junction and 10 cm above, with patient standing erect. Have the patient bend forward and measure the distance between the two points. The normal spine should have an increase of greater than 5 cm. Less than 5 cm is suggestive of decreased spinal mobility.
         2. Chest expansion. Normally, chest circumference increases by 5 cm with full inspiration. This will be decreased in those with ankylosing spondylitis.
      2. Radiographic findings. Sacroiliitis with sclerosis and fusion of the sacroiliac joints. May have an asymmetric erosive arthropathy. Spine involvement may be manifested by squaring of superior and inferior margins of vertebral body, syndesmophytes, and "bamboo spine."
      3. Lab tests. The sedimentation rate is elevated in 75% of patients, as is the CRP. However, they do not always correlate with the activity of the disease. Immunofluorescence will show IgM deposition in the superficial vessels of the skin. HLA-B27 is present not only in 95% of whites with ankylosing spondylitis but also 6% to 8% of normal population. HLA-B27 should not be obtained as a screening test; the diagnosis is made on clinical and radiographic findings.
   3. Treatment.
      1. NSAIDs. Indomethacin is the drug of choice, with a starting dose of 25 mg PO TID. This may be increased to 50 mg TID. Side effects include nausea, gastric discomfort and diarrhea, headache, vertigo, and depression common in elderly. Other NSAIDs, such as naproxen and sulindac, are also effective.
      2. Aspirin. For unknown reasons generally not effective.
      3. Sulfasalazine 2 to 3 g PO QD. May be helpful, especially for peripheral joint disease. The effect starts within the first 2 to 3 months, and the drug continues to be effective acutely. The effect seems to be best in patients with high peak of disease activity and early disease. In very chronic cases, the response has not been different from the placebo. Monitoring includes CBC and liver enzymes every 2 weeks during the first 3 months.
      4. Methotrexate and cyclophosphamide have also been used in recalcitrant cases.
      5. Education about good posture. Exercise to promote extension of the back. Stop smoking. Avoiding pillows at night and encouraging sleep in the prone position are important.
      6. Physical therapy exercises (especially swimming) and attention to posture are critical. Range-of-motion exercises, especially of the back, are important to maintain flexibility.
      7. Genetic counseling should be recommended.
2. Reiter's Syndrome.
   1. Clinical features.
      1. Reiter's syndrome. A seronegative arthropathy (reactive arthritis) that preferentially involves the lower extremities. Reiter's syndrome is the most common type of polyarthritis in young men. It may manifest as insidious joint pain or acutely with fever (as high as 39° C), swollen, hot joints, severe weight loss, and diffuse poly- articular involvement. When synovitis is limited to a few joints, low-grade fever or no fever at all is the rule. The onset may temporally be related to urethritis, diarrhea, or other infection with organisms such as Yersinia, Salmonella, Shigella, Campylobacter, or Chlamydia. The triad of arthritis, conjunctivitis, and urethritis should be suggestive of a diagnosis of Reiter's syndrome. However, the manifestation of these symptoms may be separated by months. Relapses begin 3 to 4 years after the first episode and can consist of recurrence of peripheral arthritis or enthesopathic pain of pelviaxial symptoms, or of iritis or other extraarticular symptoms. These symptoms can be isolated or associated. Radiographic changes may now be observed.
      2. Other associated findings. Skin disorders (balanitis, oral ulcerations, or keratoderma blennorrhagicum), conjunctivitis, and urethritis. In chronic disease, heart block or aortic regurgitation may occur. Diarrhea may precede the development of Reiter's syndrome. There is an association of Reiter's disease with HIV infection, and arthritis may be present before symptoms or signs related to the HIV infection appear.
      3. Lab tests. Findings may include an elevated ESR/CRP and anemia of chronic disease; 80% of patients are HLA-B27 positive. Stool cultures and urethral cultures should be done. Offer HIV testing in the appropriate population.
   2. Treatment.
      1. NSAIDs. Indomethacin 25 mg PO QID (can be increased to 50 mg PO QID).
      2. Antibiotics. Treatment of the underlying bacterial infection may hasten resolution. A 3-month course of tetracycline has been shown to hasten resolution of symptoms in those with disease related to Chlamydia organisms.
      3. Immunosuppressive drugs, such as methotrexate or azathioprine, may be effective.
      4. Steroids. Patients with large-joint involvement may benefit from intra-articular corticosteroid injection.
3. Arthritis Associated with Inflammatory Bowel Disease (Enteropathic Arthritis). There are two types of arthritis associated with ulcerative colitis and Crohn's disease: a nondestructive oligoarthritis of peripheral joints and ankylosing spondylitis.
4. Psoriatic Arthritis.
   1. Clinical features. Occurs in up to 7% of patients with psoriasis and is strongly associated with the presence of nail pitting. Most patients (95%) have involvement of multiple small joints of the hands and feet. Others have solely spine involvement or, more commonly (35% to 50%), a combination of spine involvement and peripheral joint involvement. There is a high prevalence (10% to 30%) of atlantoaxial subluxation in severe, chronic disease. The inflamed joints in patients with psoriatic arthritis often have a purplish discoloration, which is not commonly seen in other forms of arthritis.
   2. Treatment. Basic management uses NSAIDs, exercise, physical therapy, and education; control of psoriasis is important. Other possible therapies include methotrexate, antimalarials, sulfasalazine, and other DMARDs.

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See related Provider Topics Ankylosing Spondylitis or Bones, Joints and Muscles.

See related Patient Topics or Bones, Joints and Muscles.

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