A new scoring system for the Bath Ankylosing Spondylitis Metrology Index (BASMI)
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Biomedical subjects
Publications and source records attributed to A Calin.
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OBJECTIVES: The evaluation of the role of polymorphism within the class II encoded antigen processing genes, LMP2 and TAP, in susceptibility to ankylosing spondylitis (AS). METHODS: Eighty five patients with ankylosing spondylitis, 35 B27 positive healthy controls, and 55 unrelated healthy controls were studied. TAP1 and TAP2 alleles were assigned by ARMS PCR, and LMP2 alleles were assigned by restriction enzyme digestion of a PCR product. RESULTS: The TAP1C allele was increased in the AS group (6%) compared with random controls (1%), p = 0.03 and TAP2E was increased in AS (3.5%) compared with random controls (0%), p = 0.05. However, the frequencies of these alleles were also increased in B27 matched controls. There were no differences in LMP2 allele or genotype frequencies between AS and either of the control groups. Partitioning of patients according to presence or absence of uveitis did not reveal any significant associations. CONCLUSIONS: Increases of the minor TAP alleles, 1C and 2E, in AS reflect linkage disequilibrium between these alleles and HLA-B27. Polymorphism of the class I antigen processing pathway does not contribute significantly to AS susceptibility nor to the development of anterior uveitis associated with AS.
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OBJECTIVE: After pain and stiffness, one of the most important complaints of patients with ankylosing spondylitis (AS) is disability. The main aims of treatment are to control pain but also to improve function. Various methods of assessing function exist but are either not specific for the disease or have not been adequately validated. As a result of this deficiency we developed the Bath Ankylosing Spondylitis Functional Index (BASFI) as a new approach to defining and monitoring functional ability in patients with AS. METHODS: This self-assessment instrument was designed by a team of medical professionals in conjunction with patients, and consists of 8 specific questions regarding function in AS and 2 questions reflecting the patient's ability to cope with everyday life. Each question is answered on a 10 cm horizontal visual analog scale, the mean of which gives the BASFI score (0-10). The questionnaire was completed 257 times in total: once by 116 outpatients and by 47 inpatients on 3 occasions over a 3-week intensive physiotherapy course. In addition, the instrument was compared with the Dougados functional index. RESULTS: Patients scores covered 95% of the BASFI range, giving a normal distribution of results. In contrast only 65% of the Dougados functional index scale was used. Furthermore, over the 3 week period of inpatient treatment, the BASFI revealed a significant improvement in function (20%, p = 0.004) while there was a less impressive change in the Dougados functional index (6%, p = 0.03). This demonstrates the superior sensitivity of the BASFI: Consistency was good for both indices (p < 0.001), as was the relationship between patient perception of function and function as assessed by an external observer (p < 0.001). CONCLUSION: The BASFI satisfies the criteria required of a functional index: it is quick and easy to complete, is reliable and is sensitive to change across the whole spectrum of disease.
OBJECTIVE: Disease status, in terms of disease activity, disease progression and prognosis is difficult to define in ankylosing spondylitis (AS). No gold standard exists. Therefore, the Bath Ankylosing Spondylitis Disease Activity Index (BASDAI), a self-administered instrument, has been developed as a new approach to defining disease activity in patients with AS. METHODS: The index, designed by a multidisciplinary team with input from patients, consists of six 10 cm horizontal visual analog scales to measure severity of fatigue, spinal and peripheral joint pain, localized tenderness and morning stiffness (both qualitative and quantitative). The final BASDAI score has a range of 0 to 10. The index was distributed to a cross section of patients, including inpatients receiving 3 weeks of intensive physiotherapy treatment and hospital outpatients. BASDAI was completed by a total of 154 patients. Validation of the new instrument was achieved through analysis of user friendliness, reliability (consistency), score distribution and sensitivity to change. Comparisons were made with a previous Bath disease activity index (DAI) and the Newcastle Enthesis Index. RESULTS: The BASDAI was found by patients to be quick and simple to complete (mean: 67 s). Test-retest reliability was good (r = 0.93; p < 0.001), as was the distribution of scores across the scale (score range: 0.5-10; mean: 4.31). BASDAI was sensitive to change, reflecting a 16% (mean) improvement in inpatient scores after 3 weeks of treatment. It is superior to the DAI in terms of construct and content validity and to the Enthesis Index in all aspects. CONCLUSION: In summary, BASDAI is user friendly, reliability, sensitive to change and reflects the entire spectrum of disease. It is a comprehensive self-administered instrument for assessing disease activity in AS.
OBJECTIVE: To determine the most appropriate clinical measurements for the assessment of ankylosing spondylitis (AS) to develop the new metrology index. METHODS: One hundred and ninety-three individuals with AS were studied. The patients reflected the entire spectrum of cases of AS. Metrology was performed on 327 occasions. First the metrology (20 measurements) of 43 patients was analyzed. From this, 5 simple clinical measurements were defined which most accurately reflect axial status: cervical rotation, tragus to wall distance, lateral flexion, modified Schober's, and intermalleolar distance. These measurements were assessed for reliability, speed and both inter and intraobserver variability in another 40 patients. RESULTS: Analysis of the first group of 43 patients and a subsequent group of 54 patients, using the 5 measurements that constitute this new Bath AS Metrology Index (BASMI), demonstrated that they accurately and reliably mirror the 20 clinical measurements assessed previously (r = 0.92, p < 0.001). In a new group of 40 patients the measurements were demonstrated to be accurate and reproducible for both intraobserver variability (r = 0.99, p < 0.001) and interobserver variability (r = 0.97, p < 0.001). In a further 56 patients, admitted for inpatient therapy, an improvement in the BASMI from 3.34 (SD 2.71) to 2.16 (SD 2.42) was noted over a period of 3 weeks (regardless of disease severity) which indicates a sensitivity to change (chi 2 = 6.55, p < 0.01). The mean improvement over baseline was about 30%. CONCLUSION: Five clinical measurements provide a composite index (BASMI) and define disease status in AS. The BASMI is quick (7 min), reproducible and sensitive to change across the disease spectrum.
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OBJECTIVE: To define potential differences and the possible contribution of susceptibility or severity genes in familial versus sporadic ankylosing spondylitis (AS). METHODS: Three hundred twenty patients with AS were studied: 160 who had first-degree relatives with AS (familial) and 160 age- and sex-matched controls who had no first-degree relative with the disease (sporadic). Disease expression in the two groups was evaluated using an index of physical, psychological, and social functioning (the Arthritis Impact Measurement Scales [AIMS]) and an assessment of spinal mobility. RESULTS: Familial disease was significantly milder than sporadic disease as assessed by all measures, e.g., spinal mobility score (mean 4.08 versus 4.65, P < 0.038), AIMS overall impact score (mean 2.63 versus 3.59, P = 0.002), AIMS physical activity score (4.19 versus 5.10 [P = 0.004]), AIMS social function score (4.02 versus 4.60, P = 0.023), and AIMS pain score (4.15 versus 5.33, P = 0.002). CONCLUSION: The greater prevalence of AS in at-risk families may be explained by the occurrence of more AS "susceptibility" genes in those families, whereas the more severe disease, seen in patients with sporadic AS, is conferred by the presence of more "severity" genes than "susceptibility" genes.
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A 69-year-old lady with RA developed porphyria cutanea tarda 2 weeks following initiation of methotrexate therapy. The clinical and laboratory features are described.
OBJECTIVE: To investigate the interrelated effect of phenotypic expression [i.e., primary ankylosing spondylitis (1 degree AS) or disease secondary to psoriasis (Ps) AS or inflammatory bowel disease (IBD)AS], age at onset, sex and inheritance of responsible genes in AS. METHODS: Three studies were performed to evaluate 1949 subjects with AS. Subgroups of the patients were formed for each study depending on disease type (1 degree AS = 1695; Ps AS = 173; IBD AS = 81), nature of inheritance or age at onset of AS symptoms. These groups were further subdivided to assess the effect of sex. RESULTS: The sex ratio of the entire group was 2.6:1 in favor of men. However, IBD AS had an equal sex distribution as does IBD alone. By contrast, Ps, which has an equal sex ratio as a lone event or in association with arthritis, resulted in a male dominance of 4.1:1 when it occurred as Ps AS. Women with IBD AS had a significantly younger onset compared to women with 1 degree AS [mean onset 21.7 years (SD 6.65) vs mean onset 24.4 years (SD 9.79), respectively; p = 0.019]. A younger age at onset was found in women with familial disease [mean 22.2 years (SD 7.55)] compared with the mean onset of sporadic disease in women [24.5 years (SD 10.0); p = 0.0059]. There was a progressive fall in the sex ratio as the age at onset increased (p = 0.053). For example: M:F ratio of < 20 years old was 3:1 compared to 1.8:1 for those with an onset of > 40 years. CONCLUSION: Sex ratio and age at onset are influenced both by each other and such factors as disease type and familial versus sporadic occurrence. These data help provide a predictable pattern of disease in spondyloarthropathy.
OBJECTIVE: Fatigue in ankylosing spondylitis (AS) is largely ignored by physicians and classical texts. By contrast, patients frequently allude to it as a major complaint. METHODS: To address the situation, 3 studies were performed: (1) Symptoms were defined in a cross sectional evaluation of 1950 consecutive patients with AS. (2) From each of the 3 groups who specified a particular main symptom (pain, stiffness or fatigue), a random cohort of 20 was selected and all 60 were prospectively followed over a 14-day period. (3) An additional 100 patients [50 randomly selected with AS and 50 with rheumatoid arthritis (RA)] took part in a comparative prospective study. RESULTS: In the first study for those with a definitive major symptom, 34% (n = 670) described pain while stiffness and fatigue were reported by 25% (n = 492) and 6% (n = 124), respectively. Thirty-two percent (n = 616) could not distinguish between the 3. Strikingly, when prospectively studied over a 2-week period, over 50% of the patients revealed that fatigue was the main symptom. Moreover, in the cohort which expressed pain as their major problem initially, fatigue had the highest prevalence (mean fatigue value versus mean stiffness, p = 0.009; fatigue versus pain, p < 0.001). In the direct comparison between patients with RA and those with AS, the RA cohort had statistically more fatigue and pain than the AS cohort (p = 0.002, p = 0.007, respectively) with a similar amount of stiffness expressed by both groups (n = 0.149). In both subsets, pain had the least impact on the patients (mean 2.60 and 1.87, respectively). CONCLUSION: Our data reveal that fatigue should be considered a major problem for patients with AS, worthy of further exploration in terms of both etiology and therapy.
Initially, 1,492 patients with ankylosing spondylitis (AS) were assessed by a new disease activity index. This addressed the degree of pain, severity and overall disease activity (scale 3-26). As expected, there was a normal distribution with a median of 12. Sixty-five patients, selected from the extremes of the disease activity scale, (< 5, > 22), were prospectively followed over a 2-year period: 30 with high activity mean score 24.1 (SD 1.2) and 35 with low mean score 3.4 (SD 0.5). At followup, subjects were assessed with this new index and a validated functional index. Although at followup the high activity mean score was significantly reduced to 21.1 (p < 0.001) and the low activity mean score was significantly increased to 6.1 (p = 0.002), the majority of the patients had remained in their original quartiles i.e., 63 and 77%, respectively. Disease status at followup was independent of disease duration; i.e., high activity group mean 27.1 (SD 5.3) and remission group 26.9 (SD 13.77) years, respectively (NS). When the 2 disease indices were compared, an excellent correlation existed: r = 0.788, p < 0.001. Our data suggest (1) < 1% of patients with AS who present to a rheumatologist enter longterm remission ("burn out"). (2) Some 20% of patients in remission will develop active disease 2 years later. (3) The prognosis over 2 years for those with active disease is poor.
An analysis of the age at first presentation was undertaken in patients with ankylosing spondylitis and mechanical back pain seen at the London Hospital department of rheumatology between 1952 and 1983. There was a significant positive correlation with the calendar year of presentation in the patients with ankylosing spondylitis but a negative correlation in those with mechanical back pain. An increasing age at presentation in ankylosing spondylitis is likely to be due to an increasing age at disease onset--all anticipated biases would act in the opposite direction. This observation in a prospective study supports the findings of other studies using different epidemiological techniques.
Classification criteria for most of the disorders belonging to the spondylarthropathy group already exist. However, the spectrum of spondylarthropathy is wider than the sum of these disorders suggests. Seronegative oligoarthritis, dactylitis or polyarthritis of the lower extremities, heel pain due to enthesitis, and other undifferentiated cases of spondylarthropathy have been ignored in epidemiologic studies because of the inadequacy of existing criteria. In order to define classification criteria that also encompass patients with undifferentiated spondylarthropathy, we studied 403 patients with all forms of spondylarthropathy and 674 control patients with other rheumatic diseases. The diagnoses were based on the local clinical expert's opinion. The 403 patients included 168 with ankylosing spondylitis, 68 with psoriatic arthritis, 41 with reactive arthritis, 17 with inflammatory bowel disease and arthritis, and 109 with unclassified spondylarthropathy. Based on statistical analysis and clinical reasoning, we propose the following classification criteria for spondylarthropathy: inflammatory spinal pain or synovitis (asymmetric or predominantly in the lower limbs), together with at least 1 of the following: positive family history, psoriasis, inflammatory bowel disease, urethritis, or acute diarrhea, alternating buttock pain, enthesopathy, or sacroiliitis as determined from radiography of the pelvic region. These criteria resulted in a sensitivity of 87% and a specificity of 87%. The proposed classification criteria are easy to apply in clinical practice and performed well in all 7 participating centers. However, we regard them as preliminary until they have been further evaluated in other settings.