5.3 Deformity severity measures: 5.3.3 Deformity severity measures: ankylosing disorders



10.1055/b-0034-98152

5.3 Deformity severity measures: 5.3.3 Deformity severity measures: ankylosing disorders


Hossein K Elgafy, Daniel C Norvell, Jens R Chapman



1 Introduction to deformity severity measures for ankylosing spondylitis


Arthritis is a relatively loosely defined disorder affecting joints characterized by pain and swelling, progressive loss of function through destruction of the cartilage surfaces with resultant stiffness, loss of motion and eventual deformity. It is commonly identified as the leading cause of disability in the developed world. The human spinal column, with its average of 23 discs and 53 joints, is a prime target for manifestations of arthritic disorders. In general, arthritic disorders are differentiated into inflammatory “arthritic” diseases and degenerative “wear-and-tear” disorders, the latter generally being referred to as “arthrosis” outside of the English-speaking world. Despite its predilection towards arthritic diseases, the classification of its disease patterns has been almost entirely left to subspecialized Internal Medicine physicians with emphasis on inflammatory disorders. Within the field of “Rheumatology”, the main emphasis of providing structural analysis has mainly revolved around ankylosing spondylitis (AS), which had posed challenges in providing timely diagnosis due to lack of clear serologic or histologic diagnostic criteria. As any review of the literature will readily identify rheumatoid arthritis (RA) or juvenile rheumatoid arthritis (JRA) with their relatively clearly defined presentation and diagnostic pathways, have surprisingly not received well—publicized classification attempts in terms of disease severity or manifestation patterns. One notable exception of severity scores within Rheumatology is the Ranawat score, which was initially presented with the functional status of rheumatoid arthritis patients in mind. Even in the presence of atlanto-axial instability, which is a rather typical spinal affliction of Rheumatoid Arthritis, the prevailing scientific literature has used direct measurements of the atlas-dens interval (ADI), or has provided measurements for the space available for the cord (SAC), again as a continuum expressed in direct measurements of millimeters, rather than in a graded form [1]. There are also many well-delineated sub-entities within the inflammatory disorders, which have again not received further refinement in the form of severity scales or disease-specific classifications. From a surgeons’ perspective the absence of disease rating-systems for most inflammatory diseases is somewhat surprising, since increasingly complex and perhaps effective medical therapies have become available and results of treatment in terms of measurable joint disease regression would seem to benefit from an outcomes analysis based on severity scales.



2 Current classification systems for ankylosing spondylitis


In contrast to these sero-positive disorders, ankylosing spondylitis (AS) has been extensively covered with disease severity descriptors and classification systems, of which this chapter reviews a few pertinent examples. Many of these systems appeared to have arisen out of diagnostic uncertainty which historically had affected this particular disorder. In the process of developing diagnostic criteria, which were excluded for purposes of this book, the further evolution of assessment categories resulted in actual disease severity description concepts, which qualified as severity scales applicable to the spine, and are therefore listed in this chapter.


Diagnostic criteria for ankylosing spondylitis (AS) were first specified at a conference in Rome in 1963 [2] which focused on the anatomical and clinical components of the disease to include radiographic measures of the sacroiliac joints and pain, stiffness, and spinal motion. These criteria were later modified, resulting in the New York criteria formulated in 1966 which added grades of severity [3].


In 1984, van Der Linden et al proposed a modification of substituting the Rome pain criterion for the New York criterion [4]. In 1988, Mander et al created the Enthesis index (also known as the Newcastle Enthesis Index) which emphasized the clinical component of pressure applied to 66 different anatomical sites [5]. A 0 to 3 point pain scale was created and the total score was the sum of all sites. This measure was modified in 2003 and called the Maastricht Ankylosing Spondylitis Enthesitis Score (MASES) [6]. The 66 palpable enthesis were reduced to 13 and pain intensity was omitted. The total score was based on the sum of the individual enthesis.


In 1988, the Dougados Articular Index was published and is similar in nature to the Enthesis Index but focused on 10 joint sites using the same 0 to 3 point pain scale [7]. In 1991, Dougados et al as part of the European Spondylarthropathy Study Group (ESSG) developed criteria that included family history and other related comorbid conditions [8]. In the same year, the Stoke Ankylosing Spondylitis Spine Score (SASSS) was developed with a focus on grading the severity of lateral lumbar and pelvic x-rays [9]. This was modified in 2005 by adding a score for the cervical spine [10].


Beginning in 1994, a series of measures known as the Bath Ankylosing Spondylitis indices were developed including a radiology index (BASRI-s) and a metrology index (BASMI) which have also experienced modifications [1113].


In 2002 and 2003, the Ankylosing Spondylitis spine Magnetic Resonance Imaging (ASspiMRI) system and the Berlin MRI Spine Score were developed, respectively [1416]. Both systems use MRI to evaluate spinal lesions. The ASspiMRI has an activity score and a chronicity score covering the entire spine. The Berlin score modified the ASspiMRI by excluding the evaluating and scoring of erosions.


In 2005, the Spondyloarthritis Research Consortium of Canada (SPARCC) Magnetic Resonance Imaging (MRI) Spinal Inflammation Index was developed in a similar fashion to the other two MRI systems [17, 18]. This system also evaluates the entire spine, but only the 6 most severe vertebral units are scored.


In 2006, Maksymowych et al developed the Edmonton Ankylosing Spondylitis Metrology Index [19]. This index focused on cervical, chest, lumbar and hip mobility and severity is assigned based on the percentile rank of each.



Quality of the existing systems


On the whole, the AS measures reviewed in this book have done a fair job of including the important components of a disease severity measure. All of them include an anatomical component (A) and the majority include a clinical component (C). The anatomical component relies primarily on x-ray findings or painful palpation sites. Recently developed measures have depended more on MRI. The clinical components are primarily focused on pain with or without joint motion depending on the measure. The majority of the measures attempt to incorporate severity by applying relatively arbitrary scoring systems. None of the measures quantified severity based on the measures influence on treatment decision making and only two evaluated predictive validity. The BASRI-s was found to predict forward progression and the Berlin Score was found to predict the Bath Ankylosing Spondylitis Disease Activity Index (BASDAI).



Treatment implications


The primary goal of treatment for ankylosing spondylitis is to avoid serious deformity while maintaining a best possible overall functional status for the patient. Ideally the disease progression, which ends in complete loss of motion of its affected articulations, is delayed as long as possible, while keeping the patient in good comfort.


In regard to directing treatment, the currently available systems mainly serve diagnostic and epidemiologic interests, but hardly help in decision-making or analysis of the delivery of spinal care. From many aspects ankylosing spinal conditions, which include ankylosing spondylitis (AS), Forestier’s disease—more commonly referred to as disseminated idiopathic skeletal hyperostosis (DISH)—and end-stage degenerative spondylosis pose enormous challenges to spine practitioners. Atypical fractures, instability or stenosis of nonfused segments, aseptic discitis and many problems surrounding the common malalignment of the spine are just some of the typical problems encountered in this patient population. Aside from the geographic distribution of ankylosing joints, relevant clinical interests such as spinal alignment and its amount of deformity location of gibbus and sparing of joint fusions are not addressed in the available systems. Important adjuvant medical problems, such as presence of osteopenia, pulmonary function, swallowing capability, horizon gaze and comfort in sleeping and sitting readily impact treatment and outcomes, yet again are not reflected in the disease severity assessments. Recent studies have identified an increasing overlap of radiographic findings of AS with DISH. [20] These diseases have many distinctly different features, yet in an elderly patient may co-exist or actually overlap in their presentation to some degree.



3 Summary


Our ability to predict which patients will do best and respond to specific treatments is undoubtedly tied to the patients’ overall disease severity when they seek treatment. While clinical presentation is important in assessing severity measures that include additional factors such as functional capacity nutritional status, bone density or composite structural balance assessment may be more useful in predicting outcomes for spinal disorders in patients with ankylosing diseases than the present day systems allow for. For spine providers, review of the currently available systems will hopefully spur on the evolution of a new generation of more comprehensive systems of severity scales for the fascinating and perplexing field of spinal arthritis disorders.



4 References

1. Boden SD, Dodge LD, Bohlman HH, et al (1993) Rheumatoid arthritis of the cervical spine. A long-term analysis with predictors of paralysis and recovery. J Bone Joint Surg Am; 75:1282–1297. 2. Kellgren JH, Jeffrey MR, Ball J (1963) The Epidemiology of Chronic Rheumatism, Vol. I. Oxford: Blackwell Scientific Publications, 326–327. 3. Bennet PH, Wood PN (1968) Population Studies of Rheumatic Diseases. Amsterdam: Exerpta Medica Foundation, 456–457 4. van der Linden S, Valkenburg HA, Cats A (1984) Evaluation of diagnostic criteria for ankylosing spondylitis. A proposal for modification of the New York criteria. Arthritis Rheum; 27:361–368. 5. Mander M, Simpson JM, McLellan A, et al (1987) Studies with an enthesis index as a method of clinical assessment in ankylosing spondylitis. Ann Rheum Dis; 46:197–202. 6. Heuft-Dorenbosch L, Spoorenberg A, van Tubergen A, et al (2003) Assessment of enthesitis in ankylosing spondylitis. Ann Rheum Dis; 62:127–132. 7. Dougados M, Gueguen A, Nakache JP, et al (1988) Evaluation of a functional index and an articular index in ankylosing spondylitis. J Rheumatol; 15:302–307. 8. Dougados M, van der Linden S, Juhlin R, et al (1991) The European Spondylarthropathy Study Group preliminary criteria for the classification of spondylarthropathy. Arthritis Rheum; 34:1218–1227. 9. Taylor HG, Wardle T, Beswick EJ, et al (1991) The relationship of clinical and laboratory measurements to radiological change in ankylosing spondylitis. Br J Rheumatol; 30:330–335. 10. Creemers MC, Franssen MJ, van’t Hof MA, et al (2005) Assessment of outcome in ankylosing spondylitis: an extended radiographic scoring system. Ann Rheum Dis; 64:127–129. 11. Jenkinson TR, Mallorie PA, Whitelock HC, et al (1994) Defining spinal mobility in ankylosing spondylitis (AS). The Bath AS Metrology Index. J Rheumatol; 21:1694–1698. 12. Jones SD, Porter J, Garrett SL, et al (1995) A new scoring system for the Bath Ankylosing Spondylitis Metrology Index (BASMI). J Rheumatol; 22:1609. 13. MacKay K, Mack C, Brophy S, et al (1998) The Bath Ankylosing Spondylitis Radiology Index (BASRI): a new, validated approach to disease assessment. Arthritis Rheum; 41:2263–2270. 14. Braun J, Baraliakos X, Golder W, et al (2003) Magnetic resonance imaging examinations of the spine in patients with ankylosing spondylitis, before and after successful therapy with infliximab: evaluation of a new scoring system. Arthritis Rheum; 48:1126–1136. 15. Braun J, van der Heijde D (2002) Imaging and scoring in ankylosing spondylitis. Best Pract Res Clin Rheumatol; 16:573–604. 16. Rudwaleit M, Schwarzlose S, Listing J, et al (2003) Is there a place for magnetic resonance imaging (MRI) in predicting a major clinical response (BASDAI 50%) to TNF alpha blockers in ankylosing spondylitis? Arthritis Rheum; 50:S211. 17. Maksymowych WP, Dhillon SS, Park R, et al (2007) Validation of the spondyloarthritis research consortium of Canada magnetic resonance imaging spinal inflammation index: is it necessary to score the entire spine? Arthritis Rheum; 57:501–507. 18. Maksymowych WP, Inman RD, Salonen D, et al (2005) Spondyloarthritis research Consortium of Canada magnetic resonance imaging index for assessment of sacroiliac joint inflammation in ankylosing spondylitis. Arthritis Rheum; 53:703–709. 19. Maksymowych WP, Mallon C, Richardson R, et al (2006) Development and validation of the Edmonton Ankylosing Spondylitis Metrology Index. Arthritis Rheum; 55:575–582. 20. Caron T, Bransford R, Nguyen Q, et al Spine Fractures in Patients with Ankylosing Spinal Disorders. Spine (accepted for publication 2008).


1 Bennet New York Criteria*


Bennet PH, Wood PHN, (1968) Population studies of rheumatic diseases. Amsterdam: Exerpta Medica Foundation, 456–457



SCALE DESCRIPTION


The following clinical criteria for ankylosing spondylitis:


Diagnosis:




  • Limitation of lumbar spine motion



  • Pain at the dorso-lumbar junction or in the lumbar spine



  • Chest expansion ≤ 1 inch (2.5 cm)


Grading:




  • Definite ankylosing spondylitis:




  • Grade 3–4 bilateral sacroilitis with at least one clinical criterion



  • Grade 3–4 unilateral or Grade 2 bilateral sacroiliitis with Criterion 1 or with both Clinical criteria 2 and 3


Probable ankylosing spondylitis:




  • Grade 3–4 bilateral sacroiliitis with no clinical criteria


Interpretation:


Descriptive of criteria of diagnosis only.


* Modified New York Criteria


van der Linden S, Valkenburg HA, Cats A (1984) Evaluation of diagnostic criteria for ankylosing spondylitis. A proposal for modification of the New York criteria. Arthritis Rheum; 27:361–368.



METHODOLOGY


No predictive validity or reliability studies were identified.


Predictive validity


















Population tested in


Outcome


Predictive validity


Not tested




Reliability


















Population tested in


Interobserver reliability


Intraobserver reliability


Not tested





CONTENT



RATING



2 Braun Ankylosing Spondylitis spine Magnetic Resonance Imaging (ASspiMRI)


Braun J, van der Heijde D (2002) Imaging and scoring in ankylosing spondylitis. Best Pract Res Clin Rheumatol; 16:573–604.


Braun J, Baraliakos X, Golder W, et al (2003) Magnetic resonance imaging examinations of the spine in patients with ankylosing spondylitis, before and after successful therapy with infl iximab: evaluation of a new scoring system. Arthritis Rheum; 48:1126–1136.



SCALE DESCRIPTION


Scoring system using MRI to evaluate spinal lesions for acute and chronic changes in patients with ankylosing spondylitis:




  • Activity score (ASspiMRI-a):




  • 0 —Normal, no lesions



  • 1 —Minor bone marrow edema/enhancement < 25%



  • 2 —Moderate bone marrow edema/enhancement < 50%



  • 3 —Major bone marrow edema/enhancement >50%



  • 4 —Minor erosion (< 25%), with bone marrow edema/enhancement



  • 5 —Moderate erosion (> 25% but < 50%), with bone marrow edema/enhancement



  • 6 —Major erosion (> 50%), with bone marrow edema/enhancement




  • Chronicity score (ASspiMRI-c):




  • 0—Normal, no lesions



  • 1—Minor sclerosis/suspicion of relevant changes



  • 2—Sclerosis/vertebral squaring/possible syndesmophyte



  • 3—1–2 syndesmophytes/small erosions



  • 4—> 2 syndesmophytes/severe erosions



  • 5—Vertebral bridging



  • 6—Vertebral fusion


Each score includes all accessible vertebral units, from C2 to S1 (n = 23).


Each vertebral unit consists of lower half of vertebra above intervertebral space plus upper half of vertebra below intervertebral space.


Interpretation:


Both the activity and chronicity score are the sum of all vertebral units assessed.


Maximum score: 138 points


Minimum score: 0 points


The higher the score, the greater the severity.



SCALE ILLUSTRATION

Fig 5. 3. 3.2-1a–g a Vertebral unit (normal). Bone edema: b Grade 1. c Grade 2. d Grade 3. Erosion: e Grade 4. f Grade 5. g Grade 6.


METHODOLOGY


No predictive validity studies were identified.


Predictive validity


















Population tested in


Outcome


Predictive validity


Not tested




Reliability


























Population tested in


Interobserver reliability


Intraobserver reliability


MR images (N = 20) assessed twice by two experienced rater [1]


+


+


MR images (N = 30) assessed by five rheumatologists and four radiologists [2]


+


Not tested


X-rays and MR images (N = 39) assessed twice by two readers [3]


+


+


References:

1. Braun J, Baraliakos X, Golder W, et al (2003) Magnetic resonance imaging examinations of the spine in patients with ankylosing spondylitis, before and after successful therapy with infl iximab: evaluation of a new scoring system. Arthritis Rheum; 48:1126–1136. 2. Lukas C, Braun J, van der Heijde D, et al (2007) Scoring infl ammatory activity of the spine by magnetic resonance imaging in ankylosing spondylitis: a multireader experiment. J Rheumatol; 34:862–870. 3. Braun J, Baraliakos X, Golder W, et al (2004) Analysing chronic spinal changes in ankylosing spondylitis: a systematic comparison of conventional x rays with magnetic resonance imaging using established and new scoring systems. Ann Rheum Dis; 63:1046–1055.


CONTENT



RATING



3 Creemers Modified Stoke Ankylosing Spondylitis Spinal Score (mSASSS)


Creemers MC, Franssen MJ, van’t Hof MA, et al (2005) Assessment of outcome in ankylosing spondylitis: an extended radiographic scoring system. Ann Rheum Dis; 64:127–129.



SCALE DESCRIPTION


Modified the original Stoke Ankylosing Spondylitis Spinal Score by adding a score for the cervical spine and defining squaring.


The following two x-rays of the spine are used:




  • Lateral lumbar



  • Lateral cervical


Cervical is scored by examination of the lower border of C2 up to and including the upper border of T1. Lumbar examination and score is the same as the original SASSS.


All corners of each vertebrae are examined and scored:




  • 0—Normal



  • 1—Presence of erosion, squaring or sclerosis



  • 2—Presence of syndesmophyte formation



  • 3—Presence of total bony bridging


Each corner of each vertebra is scored on a 0 to 3 point scale.


Interpretation:


Sum of the total points of all corners of each vertebrae.


Maximum score: 72 points


Minimum score: 0 points


The higher the score, the greater the severity.



METHODOLOGY


No predictive validity studies were identified.


Predictive validity


















Population tested in


Outcome


Predictive validity


Not tested




Reliability






































Population tested in


Interobserver reliability


Intraobserver reliability


X-rays (N = 72) of the lumbar (n = 46) and cervical (n = 26) spine assessed twice by two observers 8 weeks apart [1]


+


Not tested


X-rays (N = 133) assessed by two observers at baseline, 1 year, 2 years, and 4 years [2]


Status scores + Progression scores +


Not tested


X-rays (N = 20) assessed by two observers at baseline, 1 year, 2 years, and 4 years [2]


Not tested


Status scores + Progression scores +


Cervical spine, lumbar spine, and SI joint x-rays (N = 217) assessed at baseline, 1 year, and 2 years by two observers [3]


+


+


X-rays (N = 95) assessed by two observers [4]


+


Not tested


X-rays and MR images (N = 39) assessed twice by two readers [5]


+


+


References:

1. Creemers MC, Franssen MJ, van’t Hof MA, et al (2005) Assessment of outcome in ankylosing spondylitis: an extended radiographic scoring system. Ann Rheum Dis; 64:127–129. 2. Wanders AJ, Landewe RB, Spoorenberg A, et al (2004) What is the most appropriate radiologic scoring method for ankylosing spondylitis? A comparison of the available methods based on the Outcome Measures in Rheumatology Clinical Trials filter. Arthritis Rheum; 50:2622–2632. 3. Spoorenberg A, de Vlam K, van der Linden S, et al (2004) Radiological scoring methods in ankylosing spondylitis. Reliability and change over 1 and 2 years. J Rheumatol; 31:125–132. 4. Salaffi F, Carotti M, Garofalo G, et al (2007) Radiological scoring methods for ankylosing spondylitis: a comparison between the Bath Ankylosing Spondylitis Radiology Index and the modified Stoke Ankylosing Spondylitis Spine Score. Clin Exp Rheumatol; 25:67–74. 5. Braun J, Baraliakos X, Golder W, et al (2004) Analysing chronic spinal changes in ankylosing spondylitis: a systematic comparison of conventional x rays with magnetic resonance imaging using established and new scoring systems. Ann Rheum Dis; 63:1046–1055.

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Jul 19, 2020 | Posted by in NEUROSURGERY | Comments Off on 5.3 Deformity severity measures: 5.3.3 Deformity severity measures: ankylosing disorders

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