Ankylosing spondylitis (less commonly known as Bechterew disease or Marie-Strümpell disease) is a seronegative spondyloarthropathy, which results in fusion (ankylosis) of the spine and sacroiliac (SI) joints, although involvement is also seen in large and small joints.
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Epidemiology
Traditionally it was thought there was a male predilection of 3:1 or more, however, the gender predilection of the disease is a matter of ongoing research as females may be under-diagnosed. According to some research, men tend toward more severe disease 28. The disease usually manifests in young adults, with the first symptoms becoming evident in the third decade, although up to 18% of cases manifest in the second decade.
Associations
anterior uveitis: ~33% (range 25-40%) ref
inflammatory bowel disease: ulcerative colitis / Crohn disease
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cardiac disease: ~5% (range 2-10%) 31
conduction disorders
aortitis of the ascending aorta
mitral valve regurgitation (very rare) 31
apical/upper lobe predominant interstitial lung disease with small cystic spaces (in ~1% of patients) 4
secondary amyloidosis (rare)
cauda equina syndrome (rare)
Pathology
Genetics
Patients are rheumatoid factor (RF) negative, hence seronegative. HLA-B27 is the gene with the strongest association. Other possibly contributing genes include ERAP-1, IL23R and TNF-associated genes 22. Although approximately 90% of Caucasian individuals with ankylosing spondylitis have the HLA-B27 gene, it is important to note this gene is present in 8-9% of people of Northern European ancestry 5. Overall, ~5% of people positive for HLA-B27 develop ankylosing spondylitis.
Radiographic features
The axial skeleton is predominantly affected, although in ~20% of cases the peripheral joints are also involved.
Plain radiograph
Sacroiliac joints
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sacroiliitis is usually the first manifestation 5 and is symmetrical and bilateral
the sacroiliac joints first widen before they narrow
subchondral erosions, sclerosis, and proliferation on the iliac side of the SI joints
at end-stage, the SI joint may be seen as a thin line or not visible
Spine
early spondylitis is characterized by small erosions at the corners of vertebral bodies with reactive sclerosis: Romanus lesions of the spine (shiny corner sign)
noninfectious spondylodiscitis: Andersson lesion
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diffuse syndesmophytic ankylosis can give a "bamboo spine" appearance
syndesmophytes are classically described as paravertebral ossification running parallel to the spine
linear ossification along the central spine; representing interspinous ligament ossification can give a "dagger spine" appearance on frontal radiographs;
ossification of spinal ligaments, joints and discs (with fatty marrow within the ossified disc, best seen on MRI)
apophyseal and costovertebral arthritis and ankylosis
enthesophyte formation from enthesopathy
pseudoarthroses may form at fracture sites
Hips
Hip involvement is generally bilateral and symmetric, with uniform joint space narrowing, axial migration of the femoral head sometimes reaching a state of protrusio acetabuli, and a collar of osteophytes at the femoral head-neck junction.
Pelvis
Whiskering of the pelvic bones primarily affects the ischial tuberosities, resulting from ossification of the ligamentous origins.
There can be bridging or fusion of the pubic symphysis.
Knees
Knees demonstrate uniform joint space narrowing with bony proliferation.
Hands
Hands are generally involved asymmetrically, with smaller, shallower erosions and marginal periostitis.
Shoulders
glenohumeral joint involvement is not uncommon and demonstrates a large erosion of the anterolateral aspect of the humeral head, producing a 'hatchet' deformity ref
marrow edema of the acromion process, at the site of origin of deltoid muscle, has been described as a very specific sign of the disease ref
Chest
Radiographs of the lungs may demonstrate progressive fibrosis and bullous changes at the apices. These lesions may resemble tuberculosis infection and bullae may become infected.
See: thoracic manifestations of ankylosing spondylitis
Cardiac
Plain radiograph may be normal, or may reveal cardiomegaly.
See: cardiovascular manifestations of ankylosing spondylitis
CT
may be useful in selected patients with normal or equivocal findings on sacroiliac joint radiographs
chronic structural changes such as joint erosions, subchondral sclerosis, and bony ankylosis are better visualized on CT than on MRI or radiographs 15-17
some normal variants of the SI joints may mimic features of sacroiliitis
supplements scintigraphy in evaluating areas of increased uptake
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superior to radiographs and MRI in demonstrating injuries
imaging modality of choice in patients with advanced ankylosing spondylitis in whom there is suspicion of cervical spine fracture
sagittal reformats should be obtained as axial images poorly assess the transverse fracture plane
MRI
may have a role in early diagnosis of sacroiliitis; MRI is more sensitive than CT or plain radiography in detecting inflammatory changes (which precede structural changes) such as bone marrow edema (best demonstrated on STIR sequences), synovitis and capsulitis (on gadolinium enhanced T1 weighted sequences) 16,18
synovial enhancement on MR correlates with disease activity measured by inflammatory mediators
enhancement of the interspinous ligaments is indicative of enthesitis
increased T2 signal correlates with edema or vascularized fibrous tissue
periarticular fat metaplasia of the sacroiliac joints
superior to CT in the detection of cartilage inflammation and destruction
useful in following treatment results in patients with active ankylosing spondylitis
Bone scintigraphy
maybe helpful in selected patients with normal or equivocal findings on sacroiliac joint radiographs
qualitative assessment of accumulation of radionuclide in the SI joints may be difficult due to normal uptake in this location; thus, quantitative analysis may be more useful
ratios of SI joint to sacral uptake of 1.3:1 or higher is abnormal
Treatment and prognosis
First-line therapy is primarily focussed on NSAIDs and non-pharmacological measures including education, exercise, physiotherapy and group therapy. Together, these treatments can lead to substantial clinical improvement in 70-80% of patients. Local steroid injection and DMARDs (sulfasalazine and methotrexate) can also help with peripheral manifestations. Second-line therapy includes TNF-alpha blockers (etanercept, infliximab, adalimumab, certolizumab, golimumab) and IL17 inhibitors (secukinumab) 24. Whether TNF-alpha blockers can inhibit radiographic disease progression has been the subject of some debate and continues to be investigated 23.
Complications
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fracture
patients with ankylosing spondylitis have a 4x higher chance than the general population of spinal fracture; the overall risk of fracture is 5-15% 30
diffuse paraspinal ossification and inflammatory osteitis creates a fused, brittle spine, susceptible to fracture, even with minor trauma
more common at the thoracolumbar and cervicothoracic junctions
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recognition of minimally displaced fractures is difficult due to osteopenia and deformity, and it is important to specifically search for disc space widening and discontinuity of the ossified paraspinal ligaments
also known as "chalk stick" or "carrot stick fractures" 19
Andersson lesion: inflammatory spondylodiscitis that occurs in association with ankylosing spondylitis and results in a disc pseudarthrosis
rare neurological complications include transverse myelitis and/or cauda equina syndrome 20,21
Bath Ankylosing Spondylitis Disease Activity Index (BASDAI)
A subjective assessment by the patient on a scale of 1-10 (least to most severe) in the following six parameters 29:
How would you describe the overall level of fatigue/tiredness you have experienced?
How would you describe the overall level of AS neck, back, or hip pain you have had?
How would you describe the overall level of pain/swelling in joints other than neck, back, or hips you have had?
How would you describe the overall level of discomfort you have had from any areas tender to touch or pressure?
How would you describe the overall level of discomfort you have had from the time you wake up?
How long does your morning stiffness last from the time you wake up?
Differential diagnosis
general spine: enteropathic arthritis
cervical spine: juvenile rheumatoid arthritis
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