What is the ankylosing spondylitis (AS)?

Answer

Ankylosing spondylitis (AS) is a chronic, multisystem inflammatory disorder primarily involving the sacroiliac (SI) joints and the axial skeleton. Other clinical manifestations include peripheral arthritis, enthesitis, and extra-articular organ involvement.^{[15, 16, 17, 18]}AS has been designated by various names, including rheumatoid spondylitis in the American literature, spondyloarthrite rhizomegalique in the French literature, and the eponyms Marie-Strümpell disease and von Bechterew disease.

AS is the prototype of the spondyloarthropathies, a family of related disorders that also includes non-radioraphic axial spondyloarthritis (nr-axSpA), reactive arthritis (ReA), psoriatic arthritis (PsA), inflammatory bowel disease–associated spondyloarthropathy (IBD-SpA), undifferentiated spondyloarthropathy (USpA), and possibly Whipple disease and Behçet disease (see the image below). The spondyloarthropathies are linked by common genetics (the human leukocyte antigen [HLA] class-I gene HLA-B27) and a common pathology (enthesitis).

Family of spondyloarthropathies and HLA-B27 associated diseases

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AS is classified as a spondyloarthropathy. The disorder is often found in association with other spondyloarthropathies, including ReA, PsA, ulcerative colitis (UC), and Crohn disease. Patients often have a family history of either AS or another spondyloarthropathy.

The etiology of AS is not understood completely; however, a strong genetic predisposition exists.^{[19, 6]}A direct relationship between AS and the HLA-B27 gene has been determined.^{[20, 21, 22, 23]}The precise role of HLA-B27 in precipitating AS remains unknown; however, one hypothesis is molecular mimicry in which HLA-B27 preferentially presents a bacterial (arthritogenic) antigen that induces an immune response to a self-antigen and autoimmunity.

The diagnosis of AS is generally made by combining clinical criteria of inflammatory back pain and enthesitis or arthritis with radiological findings. Early diagnosis is important because early medical and physical therapy may improve functional outcome. As with any chronic disease, patient education is vital to familiarize the patient with the symptoms, course, and treatment of the disease. Treatment measures include pharmacologic, physical therapy, and surgical.

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Media Gallery

This 15-year-old female patient presented with recent onset of right-sided low back pain. Plain radiography findings were normal.
MRI of the same patient whose radiography findings were normal (previous image). She underwent further evaluation, including MRI. The MRI (short tau inversion recovery [STIR]) showed increased sinal intensity in the right sacroiliac joint, revealing sacroiliitis. Other laboratory study findings were essentially normal. The patient was started on indomethacin and rapidly improved.
Patient with ankylosing spondylitis affecting cervical and upper thoracic spine. The patient's spine has been fused in flexed position.
Posterior view of a patient with ankylosing spondylitis affecting cervical and upper thoracic spine. The patient's spine has been fused in a flexed position.
Anteroposterior radiograph of spine of a patient with AS. Ossification of annulus fibrosus can be observed at multiple levels, which has led to fusion of spine with abnormal curvature.
Anteroposterior radiograph of spine of a patient with AS.
Anteroposterior (left) and lateral (right) radiographs of a patient with AS.
Radiographs of hand (top) and arm (bottom) of a patient with peripheral involvement of AS. Fusion of joint spaces and deformity can be observed.
Sagittal MRI of thoracolumbar spine of a patient with AS. Syndesmophytes and anterior corner lesions can be seen.
Radiograph shows vertebral fracture in a patient with AS.
This radiograph of the lumbar spine of a patient with end-stage AS shows bridging syndesmophytes, resulting in bamboo spine.
This radiograph of the cervical spine of a patient with AS shows fusion of the vertebral bodies due to bridging syndesmophytes.
ASAS Classification Criteria for Axial Spondyloarthropathy.
ASAS Classification Criteria for Peripheral Spondyloarthropathy.
Family of spondyloarthropathies and HLA-B27 associated diseases
Bilateral symmetric sacroiliitis in a patient with AS showing blurring of the margins of the joints and pseudo-widening.
Bilateral symmetric sacroiliitis in a patient with AS with narrowing and sclerosis of the joints.
Bilateral sacroiliac joint fusion in a patient with AS.
Enthesitis at the site of the insertion of the annulus fibrosis on the corners of the vertebral bodies, shiny corner (Romanus) lesions.
CT scan of the pelvis in a patient with AS showing ankylosis of the sacroiliac joints.
CT scan of the L-spine in a patient with AS showing bony syndesmophytes.

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Author

Lawrence H Brent, MD Associate Professor of Medicine, Sidney Kimmel Medical College of Thomas Jefferson University; Chair, Program Director, Department of Medicine, Division of Rheumatology, Albert Einstein Medical Center

Lawrence H Brent, MD is a member of the following medical societies: American Association for the Advancement of Science, American Association of Immunologists, American College of Physicians, American College of Rheumatology

Disclosure: Stock ownership for: Johnson & Johnson.

Coauthor(s)

Anand Patel, MD Fellow, Department of Rheumatology, Temple University Hospital

Anand Patel, MD is a member of the following medical societies: American College of Rheumatology, Pennsylvania Rheumatology Society, Philadelphia Rheumatism Society

Disclosure: Nothing to disclose.

Ruchika Patel, MD Attending Physician, Division of Rheumatology, Einstein Medical Center

Ruchika Patel, MD is a member of the following medical societies: American College of Physicians, American College of Rheumatology, Philadelphia Rheumatism Society

Disclosure: Nothing to disclose.

Chief Editor

Herbert S Diamond, MD Visiting Professor of Medicine, Division of Rheumatology, State University of New York Downstate Medical Center; Chairman Emeritus, Department of Internal Medicine, Western Pennsylvania Hospital

Herbert S Diamond, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, American College of Rheumatology, American Medical Association, Phi Beta Kappa

Disclosure: Nothing to disclose.

Additional Contributors

Rajni Kalagate, MD Resident Physician, Department of Internal Medicine, Albert Einstein Medical Center

Rajni Kalagate, MD is a member of the following medical societies: American Academy of Pediatrics, Indian Medical Association

Disclosure: Nothing to disclose.

Acknowledgements

Jason C Eck, DO, MS Assistant Professor, Department of Orthopedics and Physical Rehabilitation, UMass Memorial Medical Center

Jason C Eck, DO, MS is a member of the following medical societies: American Osteopathic Academy of Orthopedics, American Osteopathic Association, International Society for the Study of the Lumbar Spine, and North American Spine Society

Disclosure: Medtronic Honoraria Speaking and teaching

Elliot Goldberg, MD Dean of the Western Pennsylvania Clinical Campus, Professor, Department of Medicine, Temple University School of Medicine

Elliot Goldberg, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, and American College of Rheumatology

Disclosure: Nothing to disclose.

Scott D Hodges, DO Consulting Surgeon, Department of Orthopedic Surgery, Center for Sports Medicine and Orthopedics

Scott D Hodges, DO is a member of the following medical societies: American Academy of Disability Evaluating Physicians, American Medical Association, American Osteopathic Association, American Spinal Injury Association, North American Spine Society, Southern Medical Association, Southern Orthopaedic Association, and Tennessee Medical Association

Disclosure: Medtronic Royalty Consulting; Biomet Spine Royalty Consulting

S Craig Humphreys, MD Orthopedic Spine Surgeon, Department of Orthopedic Surgery, Center for Sports Medicine and Orthopedics

S Craig Humphreys, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Orthopaedic Surgeons, American Medical Association, American Spinal Injury Association, North American Spine Society, Southern Medical Association, Southern Orthopaedic Association, and Tennessee Medical Association

Disclosure: Nothing to disclose.

James F Kellam, MD Vice-Chair, Department of Orthopedic Surgery, Director of Orthopedic Trauma and Education, Carolinas Medical Center

James F Kellam, MD is a member of the following medical societies: American Academy of Orthopaedic Surgeons, Orthopaedic Trauma Association, and Royal College of Physicians and Surgeons of Canada

Disclosure: Nothing to disclose.

Kristine M Lohr, MD, MS Professor, Department of Internal Medicine, Center for the Advancement of Women's Health and Division of Rheumatology, Director, Rheumatology Training Program, University of Kentucky College of Medicine

Kristine M Lohr, MD, MS is a member of the following medical societies: American College of Physicians and American College of Rheumatology

Disclosure: Nothing to disclose.

William O Shaffer, MD Professor, Vice-Chairman and Residency Program Director, Department of Orthopedic Surgery, University of Kentucky at Lexington

William O Shaffer, MD is a member of the following medical societies: American Academy of Orthopaedic Surgeons, American Orthopaedic Association, International Society for the Study of the Lumbar Spine, Kentucky Medical Association, Kentucky Orthopaedic Society, North American Spine Society, Southern Medical Association, and Southern Orthopaedic Association

Disclosure: DePuySpine 1997-2007 (not presently) Royalty Consulting; DePuySpine 2002-2007 (closed) Grant/research funds SacroPelvic Instrumentation Biomechanical Study; DePuyBiologics 2005-2008 (closed) Grant/research funds Healos study just closed; DePuySpine 2009 Consulting fee Design of Offset Modification of Expedium

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

What is the ankylosing spondylitis (AS)?

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