II. Epidemiology

  1. Strong Association with HLA-B27
    1. HLA-B27 positive in up to 95% of AS cases
    2. Only 2% of HLA-B27 develop Ankylosing Spondylitis
    3. Populations with higher IncidenceHLA-B27 positive
      1. Native Americans
      2. Asian populations (except Japanese)
      3. European and United States Caucasian
  2. Men more often affected by ration of 3:1
  3. Onset between Puberty and age 40 years
  4. Low Prevalence Groups
    1. South American Indians
    2. Japanese
    3. African-Americans

III. Diagnosis: Criteria

  1. Back pain
    1. Starts with dull low back radiating to gluteal area
    2. Progresses up spine to ultimately involve neck
  2. Onset before age 40 years (may occur as early as 13)
  3. Insidious onset
  4. Duration longer than 3 months
  5. Pain worse in the morning
  6. Morning stiffness lasts longer than 30 minutes
  7. Pain decreases with Exercise or activity
  8. Pain provoked by prolonged inactivity or lying down
  9. Pain accompanied with constitutional Symptoms
    1. Anorexia
    2. Malaise
    3. Low grade fever

IV. Articular Symptoms and Signs

  1. Monoarticular Arthritis or Oligoarticular Arthritis
  2. Asymmetric and nonerosive Arthritis
  3. Common joint involvement
    1. Inflammatory low back (esp. Sacroiliitis)
    2. Large joints:
      1. Shoulders
      2. Hips (Hip Flexion contractures with rigid gait)
      3. Peripheral joint involvement more common in women
        1. Women have less axial skeleton involvement
  4. Costosternal Pleuritic Chest Pain
  5. Heel Pain
    1. Achilles tendon insertion at Calcaneus
    2. Plantar fascia insertion at Calcaneus

V. Systemic Signs

  1. Acute Anterior Uveitis (Nongranulomatous)
    1. Occurs in 20-40% of Ankylosing Spondylitis
  2. Microscopic Colitis (often asymptomatic)
    1. Occurs in 25-40% of Ankylosing Spondylitis
  3. Cardiac involvement rare
    1. Aortic Insufficiency
    2. Aortitis
    3. Conduction defects
    4. Arrhythmias
  4. Pulmonary Involvement
    1. Restrictive Lung Disease
      1. Restricted costovertebral mobility
    2. Apical lobe fibrosis
  5. Neurologic Involvement
    1. Spine Fractures or dislocations
    2. Cauda Equina Syndrome
    3. Atlantoaxial subluxation

VI. Complications of Late Spondyloarthropathy

  1. Spondylodiscitis
  2. Cauda Equina Syndrome
  3. Pseudoarthrosis with spinal cord compression
    1. Resultant neurologic deficits

VII. Exam

  1. Observation of back
    1. Lumbar lordosis flattened
    2. Thoracic kyphosis exaggerated
    3. Cervical Spine hyperextended
  2. Tests for Sacroiliac Joint Inflammation
    1. Gaenslen's Test
    2. Patrick's Test
  3. Tests for range of motion loss at Lumbar Spine
    1. Schober's Test
    2. Decreased lateral bending and lumbar extension

VIII. Radiology

  1. Anteroposterior Pelvis XRay
    1. Usually sufficient as only XRay confirmation
    2. Reveals bilateral and symmetric Sacroiliitis
      1. Sclerosis may be present (usually not in children)
      2. Later findings include erosions or SI joint fusion
  2. Spine XRay other findings
    1. Initial
      1. Bony sclerosis appears as squaring of Vertebrae
    2. Next
      1. Osteitis of Vertebral margins
    3. Late
      1. Annulus fibrosus ossifies
      2. Syndesmophytes between Vertebrae
        1. Classic "Bamboo" spine (<10%) appearance
        2. Progresses up spine
  3. Special XRay views
    1. Ferguson's View (specialized sacroiliac view)
  4. Other studies with limited indications
    1. Bone Scan
    2. CT or MRI spine

IX. Labs

  1. HLA-B27 Assay
    1. Not recommended for routine testing
    2. Nonspecific: Present in up to 10% of Caucasians
  2. Acute phase reactants
    1. General
      1. Increased in up to 70% of Ankylosing Spondylitis
      2. Not correlated with disease activity or severity
    2. Markers
      1. C-Reactive Protein (CRP)
      2. Erythrocyte Sedimentation Rate (ESR)

X. Management: Non-pharmacologic

  1. Regular therapeutic Exercise
  2. Erect Posture
  3. Firm mattress (without a pillow)
  4. Deep breathing Exercises
    1. Maintain normal chest expansion
  5. Spinal extension Exercises
  6. Range of Motion Exercises
    1. Cervical Spine
    2. Shoulders
    3. Hips
    4. Knees
  7. Consider physical therapy

XI. Management: Medications

  1. First Line: NSAIDS
    1. Indomethacin (up to maximum of 50 mg PO tid)
    2. Tolmetin 400 mg PO tid-qid
  2. Second Line: NSAID refractory cases or NSAID Adjuncts
    1. Sulfasalazine 2-4g/day divided doses
      1. Effective peripheral Arthritis
      2. Less effective for axial skeleton symptoms
    2. Methotrexate
      1. Effective for peripheral but not axial Arthritis
    3. Local Corticosteroids injection
      1. For persistent synovitis and enthesopathy
  3. Other agents potential benefit
    1. Pamidronate (Aredia) IV
    2. Tumor Necrosis Factor alpha agents
      1. Etanercept (Enbrel)
      2. Infliximab (Remicade)
      3. Braun (2003) Ann Rheum Dis 62:817-24 [PubMed]
  4. Medications to avoid
    1. Avoid long term Systemic Corticosteroids
      1. Not generally effective in Ankylosing Spondylitis
    2. Avoid gold and Penicillamine

XII. References

  1. Inman in Klippel (1997) Primer Rheumatic, p. 189-93
  2. van der Linden in Ruddy (2001) Kelley's Rheum, p. 1039
  3. Dougados (2002) Ann Rheum Dis 61 [PubMed]
  4. Kataria (2004) Am Fam Physician 69:2853-60 [PubMed]

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Ontology: Ankylosing spondylitis (C0038013)

Definition (MSH) A chronic inflammatory condition affecting the axial joints, such as the SACROILIAC JOINT and other intervertebral or costovertebral joints. It occurs predominantly in young males and is characterized by pain and stiffness of joints (ANKYLOSIS) with inflammation at tendon insertions.
Definition (CHV) chronic inflammation of the spine and the low back bone joint
Definition (CHV) chronic inflammation of the spine and the low back bone joint
Definition (CHV) chronic inflammation of the spine and the low back bone joint
Definition (CHV) chronic inflammation of the spine and the low back bone joint
Definition (CHV) chronic inflammation of the spine and the low back bone joint
Definition (CHV) chronic inflammation of the spine and the low back bone joint
Definition (CHV) chronic inflammation of the spine and the low back bone joint
Definition (CHV) chronic inflammation of the spine and the low back bone joint
Definition (CHV) chronic inflammation of the spine and the low back bone joint
Definition (CHV) chronic inflammation of the spine and the low back bone joint
Definition (MEDLINEPLUS)

Ankylosing spondylitis is a type of arthritis of the spine. It causes swelling between your vertebrae, which are the disks that make up your spine, and in the joints between your spine and pelvis. Ankylosing spondylitis is an immune disease. The disease is more common and more severe in men. It often runs in families.

Early symptoms include back pain and stiffness. These problems often start in late adolescence or early adulthood. Over time, ankylosing spondylitis can fuse your vertebrae together, limiting movement. Symptoms can worsen or improve or stop altogether. The disease has no cure, but medicines can relieve the pain, swelling and other symptoms. Exercise can also help.

NIH: National Institute of Arthritis and Musculoskeletal and Skin Disease

Definition (MSHCZE) Zánětlivé onemocnění kloubů postihující zejm. klouby páteře, křížové (sakroiliakální) klouby a některé větší klouby končetin (ramena, kyčle). Onemocnění se vyskytuje ve svých těžších formách převážně u mužů. Předcházejí mu někdy záněty oka (iridocyklitida) a bolesti pat (Achillovy šlachy). Později se objevuje bolest v kříži a v zádech, omezení hybnosti páteře a v neléčených případech může dojít až k jejímu naprostému ztuhnutí (ankylózy páteřních kloubů). V léčbě se používají antirevmatika, nesmírně důležitá je rehabilitace. Příčina nemoci není známa, existuje vztah k některým HLA antigenům. (cit. Velký lékařský slovník online, 2013 http://lekarske.slovniky.cz/ )
Definition (NCI) An autoimmune chronic inflammatory disorder characterized by inflammation in the vertebral joints of the spine and sacroiliac joints. It predominantly affects young males. Patients present with stiffness and pain in the spine.
Concepts Disease or Syndrome (T047)
MSH D013167
ICD9 720.0
ICD10 M45 , M45.0
SnomedCT 156619005, 9631008
English Bechterew Disease, Bechterew's Disease, Bechterews Disease, Marie Struempell Disease, Marie-Struempell Disease, Spondylarthritis Ankylopoietica, Spondylitis, Ankylosing, Rheumatoid Spondylitis, ANKYLOSING SPONDYLITIS, Spondylitis, Rheumatoid, Idiopathic ankylos spondylit, SPONDYLITIS ANKYLOSING, BECHTEREW DIS, MARIE STRUEMPELL DIS, BECHTEREWS DIS, Ankylosing Spondylitis, ankylosing spondylitis, ankylosing spondylitis (diagnosis), Ank spond, Spondylitis ankylopoietica, Spondylitis ankylosing, Ankylosing Spondyloarthritis, Spondyloarthritides, Ankylosing, Ankylosing Spondyloarthritides, Ankylosing Spondylarthritis, Spondylarthritides, Ankylosing, Spondylarthritis, Ankylosing, Spondyloarthritis, Ankylosing, Ankylosing Spondylarthritides, Spondylitis, Ankylosing [Disease/Finding], bechterew disease, Disease;Bechterews, spine rheumatoid arthritis, arthritis rheumatoid spine, bechterew's disease, marie strumpell disease, spondylarthritis ankylopoietica, spondylitis ankylosing, marie-strumpell disease, rheumatoid spondylitis, Bechterew's disease, Spondyloarthritis Ankylopoietica, Spondylitis Ankylopoietica, Bekhterev's disease, Rheumatoid spondylitis, AS - Ankylosing spondylitis, Idiopathic ankylosing spondylitis, Marie-Strumpell spondylitis, Marie Strümpell spondylitis, Ankylosing spondylitis (disorder), Rheumatoid arthritis of spine, arthritis; spine or vertebra, Marie-Strümpell, arthritis; spine or vertebra, ankylosing, Marie-Strümpell; spondylitis, Marie-Strümpell, Strümpell-Marie, Von Bechterew, Bechterew, rheumatoid; arthritis, spine, rheumatoid; spondylitis, spine or vertebra; arthritis, Marie-Strümpell, spine or vertebra; arthritis, ankylosing, spondylitis; Marie-Strümpell, spondylitis; ankylopoietica, spondylitis; ankylosing, spondylitis; rheumatoid, ankylopoietica; spondylitis, ankylosing; spondylitis, arthritis; rheumatoid, spine, Ankylosing spondylitis, NOS, Marie Strumpell spondylitis, Rheumatoid arthritis of spine, NOS, Ankylosing spondylitis, Spondylitis, rheumatoid, Spondylitis, Marie-Strumpell, Rheumatioid arthritis of spine NOS, Bechterews disease
French SPONDYLARTHRITE ANKYLOSANTE, Spond des chevilles, Spondylite ankylopoïétique, Spondylarthrite rhumatoïde ankylosante, Pelvi-spondylite rhumatismale, Spondylarthrite ankylosante, Pelvispondylite rhumatismale, Cyphose hérédotraumatique, Maladie de Bechterew, Maladie de Marie-Strümpell
Portuguese ESPONDILITE ANQUILOSANTE, Espondilite anquilopoética, Espondilite reumatóide, Espondilite Reumatoide, Espondilite anquilosante, Doença de Bechterew, Doença de Marie-Struempell, Espondilartrite Anquilopoiética, Espondilite Anquilosante
Dutch reumatoïde spondylitis, spondylitis ankyloserend, spondylitis ankylopoetica, ziekte van Bekhterew, ziekte van Bechterew, Marie-Strümpell; spondylitis, ankylopoietica; spondylitis, ankyloserend; spondylitis, artritis; reumatoïd, wervelkolom, artritis; wervel{kolom}, Marie-Strümpell, artritis; wervel{kolom}, ankyloserend, reumatoïd; artritis, wervelkolom, reumatoïd; spondylitis, spondylitis; Marie-Strümpell, spondylitis; ankylopoietica, spondylitis; ankyloserend, spondylitis; reumatoïd, wervel{kolom}; artritis, Marie-Strümpell, wervel{kolom}; artritis, ankyloserend, Spondylitis ankylopoetica [Bechterew], ankyloserende spondylitis, Bechterew, ziekte van, Spondylarthritis ankylopoetica, Spondylitis ankylopoetica, Ziekte van Bechterew, Marie-Strümpell, ziekte van, Reumatoïde spondylitis, Ziekte van Marie-Strümpell
German rheumatoide Spondylitis, Bechterew Krankheit, Spond Ank, SPONDYLOSIS ANKYLOSANS, Bechterew-Krankheit, Morbus Bechterew, Spondylarthritis ankylosans, Spondylitis ankylosans, Rheumatoide Spondylitis, Marie-Strümpell-Krankheit
Italian Malattia di Bekhterev, Spondilosi della caviglia, Malattia di Bechterew, Spondilite anchilopoietica, Spondilite reumatoide, Spondiloartrite anchilopoietica, Malattia di Marie Struempell, Morbo di Bechterew, Spondilite anchilosante
Spanish Espondilitis reumatoide, Espondilitis con anquilosis, Espondilitis anquilopoyética, Enfermedad de Bekhterev, artritis reumatoidea vertebral, enfermedad de Bekhterev, espondilitis anquilosante (trastorno), espondilitis anquilosante idiopática, espondilitis anquilosante, espondilitis de Marie Strümpell, espondilitis reumatoidea, espondilosis deformante, Espondilitis anquilosante, Enfermedad de Bechterew, Enfermedad de Marie-Struempell, Espondilitis Anquilosante, Espondilitis Reumatoide, Espondiloartritis Anquilopoyética
Swedish Spondylit, ankyloserande
Japanese キョウチョクセイセキツイエン, リウマチセイセキツイエン, ベヒテレフビョウ, ゴウチョクセイセキツイエン, ストリュンペル・マリー病, マリー・シュトリュンペル病, Bechterew病, 脊椎炎-強直性, Marie-Struempell病, マリー-シュトリュンペル病, リウマチ性脊椎炎, 強直性脊椎炎, 強直性脊椎関節炎, 癒着性椎骨炎, ストリュンペル-マリー病, ベヒテレフ病, 椎骨炎-癒着性, 脊椎炎-リウマチ性, 脊椎関節炎-強直性
Czech Bechtěrevova nemoc, Revmatoidní spondylitida, Ankylozující spondylitida, ankylózující spondylitida, spondylitida ankylózující, ankylózující spondylartritida
Finnish Selkärankareuma
Russian SPONDILIT ANKILOZIRUIUSHCHII, SPONDILOARTRIT, VYZVANNYI ANKILOZOM, BEKHTEREVA BOLEZN', MARI-SHTRIUMPELIA BOLEZN', БЕХТЕРЕВА БОЛЕЗНЬ, МАРИ-ШТРЮМПЕЛЯ БОЛЕЗНЬ, СПОНДИЛИТ АНКИЛОЗИРУЮЩИЙ, СПОНДИЛОАРТРИТ, ВЫЗВАННЫЙ АНКИЛОЗОМ
Korean 강직성 척추염
Croatian SPONDILITIS, ANKILOZANTNI
Polish Zapalenie stawów kręgosłupa zesztywniające, Choroba Bechterewa
Hungarian Rheumatoid spondylitis, Bechterew-kór, Ankylotizáló spondylitis, SPA, Bechterew betegség, Spondylitis ankylopoetica
Norwegian Bekhterev-sykdom, Spondylarthritis ancylopoietica, Ankyloserende spondylitt, Bechterew-sykdom, Bechterews sykdom, Bekhterevs sykdom

Ontology: Juvenile ankylosing spondylitis (C0409675)

Concepts Disease or Syndrome (T047)
ICD10 M08.1
SnomedCT 201802002, 239805001
English Juvenile ankylosin spondylitis, AS-Juvenile ankylos spondylit, juvenile ankylosing spondylitis (diagnosis), Juvenile ankylosing spondylitis, AS - Juvenile ankylosing spondylitis, Juvenile ankylosing spondylitis (disorder), juvenile ankylosing; spondylitis, spondylitis; ankylosing, juvenile, spondylitis; juvenile ankylosing, ankylopoietica; spondylitis, juvenile
German Juvenile Spondylitis ankylosans
Korean 청소년성 강직성 척추염
Spanish espondilitis anqulilosante juvenil, espondilitis anqulilosante juvenil (trastorno), espondilitis anquilosante juvenil, espondilitis anquilosante juvenil (trastorno)
Dutch ankylopoetica; spondylitis, juveniel, juveniel ankyloserend; spondylitis, spondylitis; ankylopoetica, juveniel, spondylitis; juveniel ankyloserend, Juveniele spondylitis ankylopoetica