1. Approach to Diagnosis of Ankylosing Spondylitis Iraj Salehi-Abari MD
Approach to Diagnosis of Ankylosing Spondylitis Iraj Salehi-Abari MD., Internist Rheumatologist

2. In the name of God the merciful the compassionate

3. Iraj Salehi Abari salehiabari@sina.tums.ac.ir

4. Iraj Salehi-Abari
Definition:
Ankylosing Spondylitis (AS) is a chronic inflammatory disease of the axial skeleton with sacroiliitis and spondylitis, manifested by inflammatory LBP and progressive stiffness of the spine accompanied by enthesitis
Amir Alam Hosp.

5. Previous names: Marrie Strumble disease Bechtereve disease
Iraj Salehi-Abari
Previous names:
Marrie Strumble disease
Bechtereve disease
Amir Alam Hosp.

6. Iraj Salehi-Abari
Introduction:
AS is the prototype member of the Spondyloarthritis (SpA) family of disorders
SpA are characterized by:
Spondylitis: Inflammatory LBP
Sacroiliitis
Enthesitis
Arthritis
HLA-B27 positivity
Usually RF negativity
Amir Alam Hosp.

7. SpondyloArthritis (SpA):
Iraj Salehi-Abari
SpondyloArthritis (SpA):
Ankylosing Spondylitis
Reactive arthritis (Reiter’s Synd.)
Psoriatic Arthritis
Enteropathic Arthritis (IBD. R. A.)
Juvenile Spodyloarthropathy
Undifferentiated SpA
Amir Alam Hosp.

8. Epidemiology: Chronic LBP is a common symptom
Iraj Salehi-Abari
Epidemiology:
Chronic LBP is a common symptom
It occurs in > 80% of people
Less than 5% of chronic LBP is inflammatory
Prevalence of AS: %
Amir Alam Hosp.

9. Definite inflammatory LBP:
Iraj Salehi-Abari
Definite inflammatory LBP:
LBP lasting for > 3 months and at least 4 out of 5 below parameters:
Age at onset < 40 years
Insidious onset
Improvement with exercise
No improvement with rest
Pain at night (with improvement upon getting up)
Amir Alam Hosp.

10. Probable inflammatory LBP type I*:
Iraj Salehi-Abari
Probable inflammatory LBP type I*:
LBP lasting for < 3 months and at least 4 out of 5 below parameters:
Age at onset < 40 years
Insidious onset
Improvement with exercise
No improvement with rest
Pain at night (with improvement upon getting up)
* [Defined by Iraj Salehi-Abari, Rheumatol Int, 2012]
Amir Alam Hosp.

11. Probable inflammatory LBP type II*:
Iraj Salehi-Abari
Probable inflammatory LBP type II*:
LBP lasting for > 3 months and 2–3 out of 5 below parameters:
Age at onset < 40 years
Insidious onset
Improvement with exercise
No improvement with rest
Pain at night (with improvement upon getting up)
* [Defined by Iraj Salehi-Abari, Rheumatol Int, 2012]
Amir Alam Hosp.

12. Iraj Salehi-Abari
Epidemiology*:
Mean age at diagnosis: years
Male; 75%, Female: 25%
Definite inflammatory LBP: #65%
Probable inflammatory LBP: 25%
Positive family history of AS:
First-degree relatives: 8.5%
Second-degree relatives: 1%
HLA-B27 positivity in Iranian AS: 45%
*Iraj Salehi-Abari, Early diagnosis of AS, Rheumatol Int. 2012, table 3
Amir Alam Hosp.

13. Initial presentation*:
Iraj Salehi-Abari
Initial presentation*:
History:
A male (75%) with age of years
Definite inflammatory LBP: #65%
Probable inflammatory LBP: 25%
Buttock pain
(+) Family history (FH) of AS #10%
*Iraj Salehi-Abari, Early diagnosis of AS, Rheumatol Int. 2012, table 3
Amir Alam Hosp.

14. Initial presentation*:
Iraj Salehi-Abari
Initial presentation*:
Physical examination:
Lumbar LOM in all direction: 75%
Positive sacral push test: > 20%
Enthesitis: #30%
Arthritis: 40%
Limited chest expansion: < 2%
No systemic manifestations
*Iraj Salehi-Abari, Early diagnosis of AS, Rheumatol Int. 2012, table 3
Amir Alam Hosp.

15. Spinal limitation of motion:
Iraj Salehi-Abari
Spinal limitation of motion:
Schober sign: 10 cm above S1 (5. 1-2)
Ott sign: 30 cm below C7 (2-4, 1-2)
Fingertips-to-floor distance test
Occiput to wall test
Chest expansion test
Amir Alam Hosp.

16. Positive sacral push test:
Iraj Salehi-Abari
Positive sacral push test:
Clinical sacroiliitis
> 2+ tenderness on sacroiliac joints or the buttock elicited by direct vertical pressure over the centre of sacrum in prone position
Amir Alam Hosp.

17. Enthesitis: Inflammation of Enthesis Achilles tendonitis
Iraj Salehi-Abari
Enthesitis:
Inflammation of Enthesis
Achilles tendonitis
Plantar fasciitis
Costochondritis
Others
Amir Alam Hosp.

18. Imaging and Sacroiliitis:
Iraj Salehi-Abari
Imaging and Sacroiliitis:
Standard AP plain X-ray of the pelvis: It may show sacroiliitis with a delay of 8-10 years
MRI of the pelvis; the most sensitive
Whole Body Bone Scan (WBS) or Scintigraphy of Bones
Amir Alam Hosp.

19. Radiological Sacroiliitis:
Iraj Salehi-Abari
Radiological Sacroiliitis:
Grade 0: Normal SI joints
Grade 1: Suspicious changes of SI joints
Grade 2: Minimal erosions or sclerosis of SI joints without altration in the joint width
Grade 3: Moderate to significant erosions, sclerosis, Widening, narrowing, or Partial ankylosis of SI joints
Grade 4: Total ankylosis of SI joints
Amir Alam Hosp.

20. Radiological Sacroiliitis:
Iraj Salehi-Abari
Radiological Sacroiliitis:
In AS:
Bilateral sacroiliitis is more common than Unilateral
Symmetric sacroiliitis is a Hallmark feature
In other SpA:
Unilateral or Asymmetric sacroiliitis is a compatible feature
Amir Alam Hosp.

21. HLA-B27 positivity: 10-fold increase in chance of AS In USA:
Iraj Salehi-Abari
HLA-B27 positivity:
10-fold increase in chance of AS
In USA:
In North American whites: 7%
In white AS: 90%
In black AS: 45%
In Iranian AS: 45% [ Iraj Salehi-Abari, Rheumatol Int, 2012]
Amir Alam Hosp.

22. Extra-Articular (Systemic) features:
Iraj Salehi-Abari
Extra-Articular (Systemic) features:
Usually no initial presentation
Eyes:
Acute anterior asymmetric uveitis (A3U)
25-40%
Renal:
IgA nephropathy
NSAIDs nephropathy
Amyloidosis
Urinary stones
Amir Alam Hosp.

23. Extra-Articular (Systemic) features:
Iraj Salehi-Abari
Extra-Articular (Systemic) features:
Heart:
Aortic regurgitation (AR) due to Aortitis
Heart block (CHB)
Lungs:
Apical pulmonary fibrosis
Bowel:
Subclinical Ileo-colitis: 50%
Amir Alam Hosp.

24. Extra-Articular (Systemic) features:
Iraj Salehi-Abari
Extra-Articular (Systemic) features:
Nervous system:
Cervical myelopathy
Atlantoaxial subluxation
Fractures of C5-C6
Spinal canal stenosis
Cauda equina syndrome
Amir Alam Hosp.

25. Iraj Salehi-Abari
1984 Modified New York Classification Criteria for Ankylosing Spondylitis:
Clinical criteria:
LBP and stiffness for > 3 months that improves with exercise but is not relieved by rest
Lumbar LOM (sagittal & frontal)
Limitation of chest expansion
Radiological criteria:
Sacroiliitis grade > 2 bilaterally
Sacroiliitis grade 3-4 unilaterally
Amir Alam Hosp.

26. Iraj Salehi-Abari
1984 Modified New York Classification Criteria for Ankylosing Spondylitis:
A patient is regarded as having definite AS if he or she fulfills at least one radiological criteria plus at least one clinical criteria
It is Moderately specific and
It has a low degree of sensitivity
Amir Alam Hosp.

27. New York criteria is low sensitive and moderately specific because:
Iraj Salehi-Abari
New York criteria is low sensitive and moderately specific because:
Radiologic changes in pelvis X-ray appear with at least 8 years delay in most cases and MRI is not used for detecting Sacroiliitis
Limited chest expansion is an uncommon and delayed finding
Amir Alam Hosp.

28. New York criteria is low sensitive and moderately specific because:
Iraj Salehi-Abari
New York criteria is low sensitive and moderately specific because:
Inflammatory LBP is a leading symptom with sensitivity of 75% and it is typical in about 70-80% of patients with LBP
It is not included FH of AS
It is not included Enthesitis
It is not included HLA-B27 positivity
Amir Alam Hosp.

29. ASAS classification criteria for Axial SpA:
Iraj Salehi-Abari
ASAS classification criteria for Axial SpA:
ASAS: Assessment of SpondyloArthritis international Society
It is for all Axial SpA
Step I (Entry criteria): LBP for > 3 months in an age of onset of < 45 years
Step II: HLA-B27 positivity or Sacroiliitis on imaging
Amir Alam Hosp.

30. ASAS classification criteria for Axial SpA:
Iraj Salehi-Abari
ASAS classification criteria for Axial SpA:
Step III:
HLA-B27 positivity with at least 2 features of SpA or
Sacroiliitis with at least one feature of SpA
SpA features: 1. Inflammatory LBP, 2. Arthritis, 3. Heel enthesitis, 4. Uveitis, 5. Dactylitis, 6. Psoriasis, 7. IBD, 8. Good response to NSAIDs within hours, 9. FH of SpA, 10. Elevated CRP
Amir Alam Hosp.

33. Iraj Salehi-Abari
Amir Alam Hosp.

34. Iran criteria for early diagnosis of AS:
Iraj Salehi-Abari
Iran criteria for early diagnosis of AS:
Entry criteria:
No other prominent diagnosis such as other SpA (ReA, PsA, IBDrA) and Brucellosis is proposed according to the patient’s Hx. and Ph. Exam.
Amir Alam Hosp.

35. Iran criteria for early diagnosis of AS:
Iraj Salehi-Abari
Iran criteria for early diagnosis of AS:
Clinical criteria: Up to 8 P.
Inflammatory LBP:
Definite P.
Probable P.
Positive family history of AS Up to 2 P.
First-degree P.
Second-degree P.
Lumbar LOM in all directions P.
Positive sacral push test P.
Enthesitis &/or arthritis P.
Amir Alam Hosp.

36. Iran criteria for early diagnosis of AS:
Iraj Salehi-Abari
Iran criteria for early diagnosis of AS:
Imaging criteria: Up to 3 P.
AP X-ray or MRI of pelvis:
Unilateral sacroiliitis (grade >2) P.
Bilateral sacroiliitis (grade > 2) P.
Whole body bone scan (WBS):
Enthesitis &/or arthritis P.
Spondylitis P.
Sacroiliitis P.
HLA-B27 positivity (+1)
Amir Alam Hosp.

37. Iran criteria for early diagnosis of AS:
Iraj Salehi-Abari
Iran criteria for early diagnosis of AS:
Clinical criteria points
Imaging criteria points
HLA-B27 positivity point
Amir Alam Hosp.

38. AS is the diagnosis if there are:
Iraj Salehi-Abari
AS is the diagnosis if there are:
Six clinical points or
Five clinical and imaging points or
If HLA-B27 is positive:
Five clinical points or
Four clinical and imaging points.
Amir Alam Hosp.

39. “Amir Alam Hospital Guideline” approaching towards diagnosis of AS:
Iraj Salehi-Abari
“Amir Alam Hospital Guideline” approaching towards diagnosis of AS:
Step I: History and Physical examination
Step II: AP X-ray of pelvis and HLA-B27
Step III: MRI of pelvis
Step IV: Whole body bone scan (WBS)
Amir Alam Hosp.

40. “Amir Alam Hospital Guideline” approaching towards diagnosis of AS:
Iraj Salehi-Abari
“Amir Alam Hospital Guideline” approaching towards diagnosis of AS:
The physician must go through the steps one by one and if Iran criteria for AS are not yet satisfied in each step, go through the next.
Amir Alam Hosp.

41. “Amir Alam Hospital Guideline” approaching towards diagnosis of AS:
Iraj Salehi-Abari
“Amir Alam Hospital Guideline” approaching towards diagnosis of AS:
However, if the patients fulfil the criteria in the first step, we suggest the investigations be necessarily continued by the second step as in routine practice, a pelvic X-ray and HLA-B27 testing are beneficial for documentation and prognosis of AS patients
Amir Alam Hosp.

42. Iran criteria versus New York criteria for diagnosis of AS:
Iraj Salehi-Abari
Iran criteria versus New York criteria for diagnosis of AS:
Sensitivity of Iran criteria is 100% from the initial presentation of disease
Sensitivity of New York criteria:
Two years after initial presentation: 48.4%
Five years after initial presentation: 74.2%
Ten years after initial presentation: 80%
After 10 years: 92.1%
Mean sensitivity: 74.2%
Amir Alam Hosp.

43. Iran criteria versus New York criteria for diagnosis of AS:
Iraj Salehi-Abari
Iran criteria versus New York criteria for diagnosis of AS:
Specificity of Iran criteria is more than New York criteria?
Iran criteria is a diagnostic criteria for AS but New York criteria has been made for classification of AS
Amir Alam Hosp.

44. Iraj Salehi-Abari
Conclusion:
Iran criteria for AS is a highly sensitive instrument to detect AS in its early and late, clinical and subclinical, radiographic and pre-radiographic stages as well as atypical forms
Amir Alam Hosp.