Ankylosing spondylitis - part 2

  1. Ankylosing spondylitis is a chronic inflammatory disease primarily affecting the spine and sacroiliac joints. It is more common in men than in women, and its onset is typically between 30 and 50 years.
  2. Variation in prevalence of ankylosing spondylitis is thought to occur because of the presence of the human leucocyte antigen (HLA)-B27 gene within different populations. Although the effect of the gene is unclear, more than 90% of people with ankylosing spondylitis have the HLA-B27 gene.
  3. Ankylosing spondylitis causes pain and stiffness in the back, eventually resulting in joint damage and fusion predominantly of the sacroiliac joints, and ankylosing of the vertebrae leading to a classic bamboo spine, although this does not always occur.
  4. There are several extra-articular (non-skeletal) features that may be associated with ankylosing spondylitis, such as acute anterior uveitis, valve disorders and aortic incompetence.
  5. Ankylosing spondylitis can have a significant effect on patients’ lives, affecting work, family and social activities, and increasing the risk of depression. Therefore, early disease management is essential to prevent damage and disability.
In individuals where ankylosing spondylitis starts at a young age, there may be increased comorbidities such as cardiovascular disease, amyloidosis and infection, and complications of a fused spine such as spinal fractures, resulting in increased mortality (Bakland et al 2011). According to Peters et al (2010), the risk of myocardial infarction is almost four times higher in people with the disease compared with the general population – this is similar to other inflammatory disorders. It is, therefore, important that people with the disease are monitored carefully and that developing comorbidities such as hypertension or hypercholesterolaemia are managed appropriately.

Diagnosis

A better understanding of ankylosing spondylitis and developments in diagnostic techniques have led to changes in the diagnostic criteria for the disease. Initially, ankylosing spondylitis was thought to be a variation of rheumatoid arthritis, however it was not until the advent of diagnostic tests such as that for the HLA-B27 gene that ankylosing spondylitis was recognised as being different from rheumatoid arthritis. The modified New York criteria can be used to diagnose ankylosing spondylitis (van der Linden et al 1984) (Box 1).

Box 1. Modified New York criteria for diagnosis of ankylosing spondylitis

Clinical criteria:
  • Low back pain for more than three months, which is improved by exercise and not relieved by rest.
  • Limitation of lumbar spine motion in both the sagittal and frontal planes.
  • Limitation of chest expansion relative to normal values for age and sex.
Radiological criterion:
  • Sacroiliitis grade 2 or above. Grade 2 is bilateral sacroiliitis, grade 3-4 can be unilateral or bilateral depending on the degree of fusion.
Diagnosis:
  • Definite ankylosing spondylitis if radiological criterion is present, including at least one clinical criterion.
  • Probable ankylosing spondylitis if three clinical criteria are present, or if the radiological criterion is present, but there are no clinical signs of disease.
(van der Linden et al 1984)
One of the difficulties associated with using the modified New York criteria is that it can take up to five years before there is radiological evidence of sacroiliitis, by which time there might be significant joint destruction. Use of more advanced imaging technology such as magnetic resonance imaging reduces the time to diagnosis, with images showing inflammatory changes years before bony damage occurs. In 2009, the Assessment of SpondyloArthritis International Society (ASAS) published a consensus statement on the classification of axial spondyloarthritis (Rudwaleit et al 2009) (Figure 2).

Symptoms

There are a variety of symptoms of ankylosing spondylitis, and these include:
  • Early morning stiffness – this can take from a few minutes to many hours to ease, and it can take up to two or more hours for a person to get going in the morning. Sitting down for any length of time can cause the spine to stiffen up again (Khan 2003).
  • Pain – tends to develop gradually over weeks or months rather than days, and occurs mainly in the spine. Pain is worse at rest and is eased by exercise (Sieper et al 2009a).
  • Enthesitis – this is pain and swelling where ligaments and tendons attach to bone. A common site is the heel and pain on walking can be significant, particularly in the morning when the heel has been rested overnight (Gossec and Dougados 2004).
  • Fatigue – constant exhaustion not relieved by sleep (Mengshoel 2010).
  • Feverishness or night sweats – these are commonly reported symptoms in people with ankylosing spondylitis. However, these symptoms are also associated with other inflammatory and autoimmune disorders and there is a lack of evidence about the cause of feverishness or night sweats (Mold et al 2012).
  • Shortness of breath – as the disease progresses it can cause fusion of the thoracic vertebrae and also the attached ribs, limiting expansion of the chest. If the spine becomes fully ankylosed it can lead to a stoop, which will also limit chest expansion (Khan 2003).
  • Flares – individuals can go through periods where ankylosing spondylitis is dormant and then flares up. Cooksey et al (2009)suggested that 70% of people with the disease experience flares in any one week.
Learning Points:
  1. Symptoms of ankylosing spondylitis include: early morning stiffness; pain that gradually develop over weeks or months rather than day, and occurs mainly in the spine; enthesitis (commonly in the heel); fatigue; feverishness or night sweats; shortness of breath and flare-ups.
  2. When ankylosing spondylitis starts at a young age, there may be increased comorbidities such as cardiovascular disease, amyloidosis and infection, and complications of a fused spine such as spinal fractures, resulting in increased mortality.
  3. Ankylosing spondylitis was thought to be a variation of rheumatoid arthritis until the advent of diagnostic tests such as that for the HLA-B27 gene.
  4. The modified New York criteria can be used to diagnose ankylosing spondylitis but it can take up to five years before there is radiological evidence of sacroiliitis, by which time there might be significant joint destruction.
  5. Use of more advanced imaging technology such as magnetic resonance imaging (MRI) reduces the time to diagnosis, with images showing inflammatory changes years before bony damage occurs.

Assessment

There is an internationally recognised set of outcome measures for use with patients who have ankylosing spondylitis known as the Bath indices. These were designed to provide comprehensive information relating to an individual’s disease and its effect on their life and health (Irons and Jeffries 2004). The indices were devised in the 1990s and include (Irons and Jeffries 2004):
  • Bath ankylosing spondylitis disease activity index (BASDAI).
  • Bath ankylosing spondylitis functional index (BASFI).
  • Bath ankylosing spondylitis global score (BAS-G).
  • Bath ankylosing spondylitis metrology index (BASMI).
The Bath indices are patient-reported outcome measures and use visual analogue scales (VASs). These scales are designed to gauge an individual’s level of agreement with a statement. Scores range from 0-10, with 0 being good and 10 being bad. The use of VASs has limitations in that patients’ experiences, perceptions and life issues can affect how they perceive their disease and scores can be influenced by other events that may act as additional stressors. It is, therefore, possible that the patient’s physical, objective score, such as inflammatory markers and metrology, indicate improvement, while subjective measures, such as pain, may worsen (Kievit et al 2010). The Bath indices, in particular the BASDAI, are used as part of the screening process to assess the patient’s suitability for anti-tumour necrosis factor (TNF) therapy (Irons and Jeffries 2004).
Alternatives to the Bath indices have been suggested, such as the ASDAS (Gossec and Dougados 2004). This scoring system attempts to make assessment a more objective process, with the use of inflammatory markers such as C-reactive protein or erythrocyte sedimentation rate. However, it is important to note that as many as 50% of individuals with ankylosing spondylitis will not have raised inflammatory markers, thereby reducing the reliability of the ASDAS (Gossec and Dougados 2004).
  1. The Bath indices are patient-reported outcome measures that were designed to provide comprehensive information relating to an individual’s disease and the effects of ankylosing spondylitis on their life and health.
  2. The Bath indices use visual analogue scales (VASs) designed to gauge an individual’s level of agreement with a statement. The use of VASs has limitations in that patients’ experiences, perceptions and life issues can affect how they perceive their disease and scores can be influenced by other events.
  3. The ankylosing spondylitis disease activity score (ASDAS) is used as a more objective alternative to Bath indices and attempts to make assessment a more objective process, with the use of inflammatory markers such as C-reactive protein or erythrocyte sedimentation rat

Treatment

For many patients, ankylosing spondylitis remains a mild disease that has minimal effects on their activities of daily living and can be self-managed. The treatment options for patients with ankylosing spondylitis are outlined in Figure 3.
Exercise
Daily exercise might be helpful in the treatment of ankylosing spondylitis, and should include stretching and strengthening activities.
Dagfinrud et al (2008) found sufficient evidence to support the conclusion that any form of exercise is better than no exercise in the management of ankylosing spondylitis, and that supervised group therapy is preferable to group physiotherapy alone.

Conclusions

  • Ankylosing spondylitis can cause severe pain, fatigue and disability, thereby affecting all aspects of an individual’s life.
  • Identifying the disease early is crucial to limit the physical and psychological effects on patients.
  • Involvement of the multidisciplinary team and local support groups can help the individual to maintain a normal life with minimal disruption. It has been shown that regular exercise can reduce pain and maintain mobility. This, combined with appropriate pain management, can reduce the effect of fatigue and the incidence of depression.
  • NSAIDs and analgesics are the mainstay of treatment for many people and may limit disease progression. However, for those with more advanced or debilitating disease, newer biologics have been developed and are associated with reduced pain and disability.

Acronyms

Anti-TNF: anti-tumour necrosis factor
ASAS: Assessment of SpondyloArthritis International Society
ASDAS: ankylosing spondylitis disease activity score
BAS-G: Bath ankylosing spondylitis global score
BASDAI: Bath ankylosing spondylitis disease activity index
BASFI: Bath ankylosing spondylitis functional index
BASMI: Bath ankylosing spondylitis metrology index
HLA: human leucocyte antigen
NASS: National Ankylosing Spondylitis Society
NICE: National Institute for Health and Care Excellence
NSAIDs: non-steroidal anti-inflammatory drugs
RCN: Royal College of Nursing
VASs: visual analogue scales

Glossary

Acute anterior uveitis: inflammation of the front part of the uveal tract. It includes inflammation of the iris (iritis) and inflammation of the iris and the ciliary body (iridocyclitis).
Ankylosing spondylitis: a chronic inflammatory disease primarily affecting the spine and sacroiliac joints. Symptoms may include early morning stiffness, pain (mainly in the spine), enthesitis (commonly in the heel), fatigue, feverishness or night sweats, shortness of breath and flare-ups.
Arthritis: any inflammatory condition of the joints. It is characterised by pain, swelling, heat, redness and limitation of movement.
Bath indices: a set of outcome measures for use with patients who have ankylosing spondylitis. They are designed to provide comprehensive information relating to an individual’s disease and its effect on their life and health.
Enthesitis: an inflammation of the insertion of a muscle, with a strong tendency toward fibrosis and calcification.
HLA-B27: a protein on the surface of white blood cells. The presence of the HLA-B27 gene has been linked with ankylosing spondylitis.
Rheumatoid arthritis: a chronic, inflammatory, destructive, and sometimes deforming collagen disease that has an autoimmune component.
Sacroiliitis: an inflammation of the sacroiliac joint.
Spondylitis: an inflammation of any of the vertebrae, usually characterised by stiffness and pain.
Spondyloarthritides (SpA): an umbrella term for inflammatory diseases that involve the joints and the entheses (sites where the ligaments and tendons attach to the bones). There are five subtypes, the most frequent of which is ankylosing spondylitis.
Visual analogue scales (VASs): scales designed to gauge an individual’s level of agreement with a statement. Scores range from 0-10, with 0 being good and 10 being bad.

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