Ankylosing spondylitis (less commonly known as Bechterew disease and 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.
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 recent debate and research (females may be under-diagnosed). According to some research men tend towards 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.
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.
- anterior uveitis (25-40%)
- inflammatory bowel disease: ulcerative colitis/Crohn disease
- cardiovascular disease
- apical/upper lobe predominant interstitial lung disease with small cystic spaces (in ~1% of patients) 4
- aortic valve disease / aortitis
- secondary amyloidosis (rare)
- cauda equina syndrome (rare)
The axial skeleton is predominantly affected, although in ~20% of cases the peripheral joints are also involved.
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
- early spondylitis is characterized by small erosions at the corners of vertebral bodies with reactive sclerosis: Romanus lesions of the spine (shiny corner sign)
- vertebral body squaring
- noninfectious spondylodiskitis: Andersson lesion
- 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
- dural ectasia
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.
There can be bridging or fusion of the pubic symphysis.
Knees demonstrate uniform joint space narrowing with bony proliferation.
Hands are generally involved asymmetrically, with smaller, shallower erosions and marginal periostitis.
- Shoulder joint involvement is not uncommon and demonstrates a large erosion of the anterolateral aspect of the humeral head, producing a 'hatchet' deformity.
- Marrow edema of acromion process, at the site of origin of deltoid muscle has been described as a very specific sign of the disease.
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.
Plain radiograph may be normal, or may reveal cardiomegaly.
- 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
- 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
- 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
- superior to CT in the detection of cartilage inflammation and destruction
- useful in following treatment results in patients with active ankylosing spondylitis
- 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 focused 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.
- 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
- 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 spondylodiskitis 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)
It is a subjective assessment by the patient on a scale of 1- 10 (least to most severe) in the following five parameters 25
- How would you describe the overall level of fatigue/tiredness you have experienced?
- 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 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 long does your morning stiffness last from the time you wake up?
- 1. Riley MJ, Ansell BM, Bywaters EG. Radiological manifestations of ankylosing spondylitis according to age at onset. Ann. Rheum. Dis. 1971;30 (2): 138-48. doi:10.1136/ard.30.2.138 - Free text at pubmed - Pubmed citation
- 2. Resnick D, Niwayama G. Entheses and enthesopathy. Anatomical, pathological, and radiological correlation. Radiology. 1983;146 (1): 1-9. Radiology (abstract) - Pubmed citation
- 3. Wilkinson M, Bywaters EG. Clinical features and course of ankylosing spondylitis; as seen in a follow-up of 222 hospital referred cases. Ann. Rheum. Dis. 1958;17 (2): 209-28. Ann. Rheum. Dis. (citation) - Free text at pubmed - Pubmed citation
- 4. Mayberry JP, Primack SL, Müller NL. Thoracic manifestations of systemic autoimmune diseases: radiographic and high-resolution CT findings. Radiographics. 20 (6): 1623-35. Radiographics (full text) - Pubmed citation
- 5. Wang YF, Teng MM, Chang CY et-al. Imaging manifestations of spinal fractures in ankylosing spondylitis. AJNR Am J Neuroradiol. 2005;26 (8): 2067-76. AJNR Am J Neuroradiol (full text) - Pubmed citation
- 6. Jacobson JA, Girish G, Jiang Y et-al. Radiographic evaluation of arthritis: inflammatory conditions. Radiology. 2008;248 (2): 378-89. doi:10.1148/radiol.2482062110 - Pubmed citation
- 7. Baraliakos X, Landewé R, Hermann KG et-al. Inflammation in ankylosing spondylitis: a systematic description of the extent and frequency of acute spinal changes using magnetic resonance imaging. Ann. Rheum. Dis. 2005;64 (5): 730-4. Ann. Rheum. Dis. (full text) - doi:10.1136/ard.2004.029298 - Free text at pubmed - Pubmed citation
- 8. Bennett DL, Ohashi K, El-khoury GY. Spondyloarthropathies: ankylosing spondylitis and psoriatic arthritis. Radiol. Clin. North Am. 2004;42 (1): 121-34. doi:10.1016/S0033-8389(03)00156-8 - Pubmed citation
- 9. Cawley MI, Chalmers TM, Kellgren JH et-al. Destructive lesions of vertebral bodies in ankylosing spondylitis. Ann. Rheum. Dis. 1972;31 (5): 345-58. doi:10.1136/ard.31.5.345 - Free text at pubmed - Pubmed citation
- 10. Dihlmann W. Current radiodiagnostic concept of ankylosing spondylitis. Skeletal Radiol. 1979;4 (4): 179-88. - Pubmed citation
- 11. Fam AG, Rubenstein JD, Chin-sang H et-al. Computed tomography in the diagnosis of early ankylosing spondylitis. Arthritis Rheum. 1985;28 (8): 930-7. - Pubmed citation
- 12. Graham B, Van peteghem PK. Fractures of the spine in ankylosing spondylitis. Diagnosis, treatment, and complications. Spine. 1989;14 (8): 803-7. - Pubmed citation
- 13. Hanson JA, Mirza S. Predisposition for spinal fracture in ankylosing spondylitis. AJR Am J Roentgenol. 2000;174 (1): 150. AJR Am J Roentgenol (full text) - Pubmed citation
- 14. Burgener FA, Kormano M, Pudas T. Differential Diagnosis in Conventional Radiology. Thieme. (2008) ISBN:1588902757. Read it at Google Books - Find it at Amazon
- 15. Diekhoff T, Hermann KG, Greese J, Schwenke C, Poddubnyy D, Hamm B, Sieper J. Comparison of MRI with radiography for detecting structural lesions of the sacroiliac joint using CT as standard of reference: results from the SIMACT study. (2017) Annals of the rheumatic diseases. 76 (9): 1502-1508. doi:10.1136/annrheumdis-2016-210640 - Pubmed
- 16. Khmelinskii N, Regel A, Baraliakos X. The Role of Imaging in Diagnosing Axial Spondyloarthritis. (2018) Frontiers in medicine. 5: 106. doi:10.3389/fmed.2018.00106 - Pubmed
- 17. Puhakka KB, Jurik AG, Egund N, Schiottz-Christensen B, Stengaard-Pedersen K, van Overeem Hansen G, Christiansen JV. Imaging of sacroiliitis in early seronegative spondylarthropathy. Assessment of abnormalities by MR in comparison with radiography and CT. (2003) Acta radiologica (Stockholm, Sweden : 1987). 44 (2): 218-29. Pubmed
- 18. Hermann KG, Althoff CE, Schneider U, Zühlsdorf S, Lembcke A, Hamm B, Bollow M. Spinal changes in patients with spondyloarthritis: comparison of MR imaging and radiographic appearances. (2005) Radiographics : a review publication of the Radiological Society of North America, Inc. 25 (3): 559-69; discussion 569-70. doi:10.1148/rg.253045117 - Pubmed
- 19. Chittem L, Bhattacharjee S, Harshavardhan KR. Carrot stick fracture of cervical spine in ankylosing spondylitis. Neurol India. 2013;61 (3): 337. doi:10.4103/0028-3886.115102 - Pubmed citation
- 20. Oh DH, Jun JB, Kim HT et-al. Transverse myelitis in a patient with long-standing ankylosing spondylitis. Clin. Exp. Rheumatol. 2001;19 (2): 195-6. Pubmed citation
- 21. Lan HH, Chen DY, Chen CC et-al. Combination of transverse myelitis and arachnoiditis in cauda equina syndrome of long-standing ankylosing spondylitis: MRI features and its role in clinical management. Clin. Rheumatol. 2007;26 (11): 1963-7. doi:10.1007/s10067-007-0593-2 - Pubmed citation
- 22. The Australo-Anglo-American Spondyloarthritis Consortium (TASC), the Wellcome Trust Case Control Consortium 2 (WTCCC2), David M Evans, Chris C A Spencer, Jennifer J Pointon, Zhan Su, David Harvey, Grazyna Kochan, Udo Oppermann, Alexander Dilthey, Matti Pirinen, Millicent A Stone, Louise Appleton, Loukas Moutsianas, Stephen Leslie, Tom Wordsworth, Tony J Kenna, Tugce Karaderi, Gethin P Thomas, Michael M Ward, Michael H Weisman, Claire Farrar, Linda A Bradbury, Patrick Danoy, Robert D Inman, Walter Maksymowych, Dafna Gladman, Proton Rahman, Spondyloarthritis Research Consortium of Canada (SPARCC), Ann Morgan, Helena Marzo-Ortega, Paul Bowness, Karl Gaffney, J S Hill Gaston, Malcolm Smith, Jacome Bruges-Armas, Ana-Rita Couto, Rosa Sorrentino, Fabiana Paladini, Manuel A Ferreira, Huji Xu, Yu Liu, Lei Jiang, Carlos Lopez-Larrea, Roberto Díaz-Peña, Antonio López-Vázquez, Tetyana Zayats, Gavin Band, Céline Bellenguez, Hannah Blackburn, Jenefer M Blackwell, Elvira Bramon, Suzannah J Bumpstead, Juan P Casas, Aiden Corvin, Nicholas Craddock, Panos Deloukas, Serge Dronov, Audrey Duncanson, Sarah Edkins, Colin Freeman, Matthew Gillman, Emma Gray, Rhian Gwilliam, Naomi Hammond, Sarah E Hunt, Janusz Jankowski, Alagurevathi Jayakumar, Cordelia Langford, Jennifer Liddle, Hugh S Markus, Christopher G Mathew, Owen T McCann, Mark I McCarthy, Colin N A Palmer, Leena Peltonen, Robert Plomin, Simon C Potter, Anna Rautanen, Radhi Ravindrarajah, Michelle Ricketts, Nilesh Samani, Stephen J Sawcer, Amy Strange, Richard C Trembath, Ananth C Viswanathan, Matthew Waller, Paul Weston, Pamela Whittaker, Sara Widaa, Nicholas W Wood, Gilean McVean, John D Reveille, B Paul Wordsworth, Matthew A Brown, Peter Donnelly. Interaction between ERAP1 and HLA-B27 in ankylosing spondylitis implicates peptide handling in the mechanism for HLA-B27 in disease susceptibility. (2011) Nature Genetics. 43 (8): 761. doi:10.1038/ng.873 - Pubmed
- 23. Molnar C, Scherer A, Baraliakos X, de Hooge M, Micheroli R, Exer P, Kissling RO, Tamborrini G, Wildi LM, Nissen MJ, Zufferey P, Bernhard J, Weber U, Landewé RBM, van der Heijde D, Ciurea A. TNF blockers inhibit spinal radiographic progression in ankylosing spondylitis by reducing disease activity: results from the Swiss Clinical Quality Management cohort. (2018) Annals of the rheumatic diseases. 77 (1): 63-69. doi:10.1136/annrheumdis-2017-211544 - Pubmed
- 24. Sieper J, Poddubnyy D. Axial spondyloarthritis. (2017) Lancet (London, England). 390 (10089): 73-84. doi:10.1016/S0140-6736(16)31591-4 - Pubmed
- 25. Haywood KL, Garratt AM, Dawes PT. Patient-assessed health in ankylosing spondylitis: a structured review. (2005) Rheumatology (Oxford, England). 44 (5): 577-86. doi:10.1093/rheumatology/keh549 - Pubmed
- 26. Moser, Junie Lecours, Johan Michaud, Nathalie J. Bureau, Raphaël Guillin, Étienne Cardinal. The deltoid, a forgotten muscle of the shoulder. (2013) Skeletal Radiology. 42 (10): 1361. doi:10.1007/s00256-013-1667-7 - Pubmed
- 27. Robert G. W. Lambert, Sukhvinder S. Dhillon, Gian S. Jhangri, Juliette Sacks, Herbert Sacks, Benjamin Wong, Anthony S. Russell, Walter P. Maksymowych. High prevalence of symptomatic enthesopathy of the shoulder in ankylosing spondylitis: Deltoid origin involvement constitutes a hallmark of disease. (2004) Arthritis Care & Research. 51 (5): 681. doi:10.1002/art.20681 - Pubmed
- 28. Lee W, Reveille JD, Davis JC, Learch TJ, Ward MM, Weisman MH. Are there gender differences in severity of ankylosing spondylitis? Results from the PSOAS cohort. (2007) Annals of the rheumatic diseases. 66 (5): 633-8. doi:10.1136/ard.2006.060293 - Pubmed
Related Radiopaedia articles
- ankylosing spondylitis
- enteropathic arthritis
- psoriatic arthritis
- reactive arthritis (Reiter syndrome)
- undifferentiated spondyloarthritis
- juvenile idiopathic arthritis
- lyme arthritis
- rheumatoid arthritis
- robust rheumatoid arthritis
- systemic lupus erythematosus
- seronegative spondyloarthritides
- post-traumatic osteoarthritis
- erosive osteoarthritis
- rapidly destructive osteoarthritis of the hip
- osteoarthritis of the hand
- osteoarthritis of the knee
- scaphotrapeziotrapezoidal (STT) arthritis
- osteoarthritis of the vertebral column
- osteoarthritis of the TMJ
- primary cystic arthrosis of the hip
- secondary synovial osteochondromatosis
- osteoarthritis (mnemonic)
- miscellaneous disorders
- related articles