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Impact of sex on inheritance of ankylosing spondylitis: a cohort study.

BACKGROUND: Ankylosing spondylitis is a genetically determined and commonly familial disorder. Men and women differ in their susceptibility to ankylosing spondylitis, with about 2.5 men affected for every woman with the disease. We investigated the influence of the sex of the index case on disease penetrance within families. METHODS: The ages at which 50% and 75% of patients were diagnosed with ankylosing spondylitis were ascertained from a database of 4400 cases. Index patients with children or siblings who were old enough to have obtained a diagnosis (50% and 75% rates) were assessed for prevalence of disease among relatives. Confirmation of diagnosis for affected relatives was sought for all offspring and a random 25% selection of siblings. FINDINGS: Ankylosing spondylitis was more prevalent among children (odds ratio 1.9 [95% CI 1.2-3.0], p<0.005) and siblings (1.5 [1.2-1.9], p<0.0001) of female index cases than among those of male cases. Analyses restricted to index cases with a young age at onset (< or = 21 years) indicated that children of women had an even higher incidence of ankylosing spondylitis (7.2 [1.5-34], p=0.013) than did children of men at similar age at onset. 38% of children of female cases had disease compared with 8% of male cases. There was no difference in sex distribution among affected children or siblings of female patients with ankylosing spondylitis. By contrast, the sons and brothers of male patients had a higher prevalence of the disease (odds ratio 2.6 [1.4-5.2], p=0.003) than did daughters and sisters (1.7 [1.3-2.2], p<0.0001). INTERPRETATION: The influence of female sex is greater than that of male sex in determining increased susceptibility to ankylosing spondylitis in children. The striking maternal effect is greatest for women with young age at onset, which is not seen in men. The sex ratio of affected children depends on the sex of the affected parent.

Adolescent↗

Magnetic resonance imaging of tuberculous spondylitis.

STUDY DESIGN: This retrospective study analyzed the magnetic resonance imaging characteristics of tuberculous spondylitis. OBJECTIVE: To describe the magnetic resonance imaging characteristics of tuberculous spondylitis and compare the diagnostic yield of magnetic resonance imaging versus other modalities. SUMMARY OF BACKGROUND DATA: Tuberculous spondylitis is not an uncommon occurrence with extrapulmonary disease. It requires prompt diagnosis and management. In the pre-magnetic resonance imaging era, computed tomography was used to delineate the associated radiologic changes. Data are limited that describe the magnetic resonance imaging pattern of tuberculous spondylitis and the effect of post-contrast enhancement. METHODS: The magnetic resonance imaging characteristics of 28 vertebrae in 12 patients with tuberculous spondylitis were studied. RESULTS: The thoracic spine was the most commonly involved region, with involvement occurring in the thoracic spine alone in 12 vertebrae (43%) and with other areas of the spine in an additional five (18%). Partial involvement was detected in the majority of the vertebral lesions (24; 86%). Magnetic resonance imaging evidence of disc space involvement was apparent in only 46% of the lesions. Paraspinal abscess and epidural extension were documented by magnetic resonance imaging in 71% and 61% of lesions, respectively. Decreased signal intensity on T1-weighted images was demonstrated in 13 vertebrae (46%), with increased signal intensity on T2-weighted images seen only in five (18%). CONCLUSION: Magnetic resonance imaging is a useful diagnostic modality for patients with suspected tuberculous spondylitis. Partial vertebral involvement and paraspinal and epidural extension were delineated. Study of the signal intensity on T1- and T2-weighted images revealed a pattern that may be dissimilar to that commonly reported. Post-contrast enhancement adds more certainty to the diagnosis of tuberculous spondylitis.

Adult↗

Contained rupture of the aneurysm of common iliac artery associated with pyogenic vertebral spondylitis.

STUDY DESIGN: A case report. OBJECTIVES: To report and discuss a case of contained rupture of the aneurysm of common iliac artery associated with pyogenic vertebral spondylitis, so that investigators and practitioners may avoid the diagnostic and therapeutic pitfalls associated with pyogenic vertebral spondylitis and aortic disease. SUMMARY OF BACKGROUND DATA: Pyogenic vertebral spondylitis is a rare disorder that may have serious consequences, including death, if it is not diagnosed promptly and treated effectively. The association of pyogenic vertebral spondylitis with infection of the aorta is a rare but potentially fatal condition that requires prompt diagnosis and aggressive surgical and medical therapy. To our knowledge, this is the first report of a contained rupture of the aneurysm of common iliac artery case associated with pyogenic vertebral spondylitis resulting from an infection with Bacteroides fragilis,although Salmonellae infections are commonly associated with vertebral osteomyelitis and lesions of the contiguous aorta. METHODS: A 60-year-old man with chronic lower back pain began to experience a severe pain and had increased difficulty in walking. An MRI scan showed an increased signal in the L4-L5 disc space and an abscess extending into the spinal canal. The presumptive diagnosis was infective spondylitis. While performing a CT-guided needle biopsy, an unexpected contained rupture of the aneurysm of common iliac artery was discovered. RESULTS: A wide resection of all infected tissue, including the right common iliac artery and bony lesions, was performed in combination with antimicrobial therapy. A cryopreserved aortic allograft was used to reconstruct the artery, and an iliac strut graft was used to fill the debrided vertebral cavity. The patient's postoperative recovery was uneventful. CONCLUSION: The coexistence of pyogenic vertebral spondylitis and lesions of the aorta is rare, but may be lethal if not diagnosed promptly and treated effectively. Even if a patient's condition is stable and the hematocrit is normal, it is important to consider the possibility of a contained rupture of a mycotic abdominal aneurysm in all patients with vertebral osteomyelitis who have acute episodes of unusual severe back pain. CT is sometimes more beneficial than MRI in the identification and characterization of contained rupture of aneurysms.

Aneurysm, Ruptured↗

Spinal position sense in ankylosing spondylitis.

STUDY DESIGN: A cross-sectional comparison of spinal position sense in 50 patients with mild ankylosing spondylitis and 50 healthy controls. OBJECTIVES: To determine if spinal position sense is impaired in patients with mild ankylosing spondylitis. SUMMARY OF BACKGROUND DATA: Pathologic processes in ankylosing spondylitis target sites in the spine where joint capsules, ligaments, and tendons attach into bone. Because these sites contain receptors conveying position sense, proprioception may become impaired, and this may contribute to the spinal deformity that often develops in the more advanced stages of the disease. If deficits in proprioception are apparent then specialized exercise regimes aimed at improving proprioception in ankylosing spondylitis patients could prove beneficial. METHODS: Position sense was assessed in patients and controls by determining the absolute error in reproducing upright and flexed postures in the coronal and sagittal planes using an electromagnetic tracking device, the 3-Space Fastrak (Polhemus, Colchester, VT). Measurements were taken from sensors placed on the skin overlying T1, T7, L1, and S2. In patients, the association between position sense and validated radiographic, disease activity, and functional scores was explored. RESULTS: No deficits in spinal position sense were found in patients with mild ankylosing spondylitis. In these subjects, repositioning errors were < or = 3.88 degrees in flexed postures and < or = 2.76 in upright postures. Corresponding results for controls were < or = 4.86 degrees and < or = 3.42 degrees, respectively. Weak correlations were found between position sense and other ankylosing spondylitis outcome measures, which included indexes of function, disease activity, and posture and mobility, but in all cases, the overall trend was for position sense acuity to improve slightly with more advanced disease. CONCLUSION: Spinal position sense is unaffected in patients with mild ankylosing spondylitis and is therefore unlikely to be a useful outcome measure in this condition.

Adult↗

Failure of standard imaging to detect a cervical fracture in a patient with ankylosing spondylitis.

STUDY DESIGN: Retrospective case study of 38-year-old male with ankylosing spondylitis who presented with a Brown-Séquard syndrome following a fall and an occult fracture on initial spinal imaging studies. OBJECTIVE: To review the recommended imaging protocol in a patient with ankylosing spondylitis and a suspected cervical spine fracture. SUMMARY OF BACKGROUND DATA: Plain radiographic imaging using orthogonal views can detect the majority of spinal fractures. However, fracture identification is particularly difficult in patients with ankylosing spondylitis. Of the various contemporary imaging methods, [magnetic resonance imaging (MRI), computed tomography (CT), nuclear scanning] high-definition multidetector CT scanning is the most useful in identifying subtle fractures, but in metabolic bone diseases the utility of these methods is unknown. METHODS: Retrospective radiographic evaluation of imaging studies of ankylosing spondylitis patient with occult fracture by five spine surgeons and neuroradiologists, followed with detailed review and interpretation of literature concerning present radiology techniques and methods to isolate occult fractures. RESULTS: Initial imaging studies, including plain radiographs of the spinal axis, cervical and thoracic single-detector CT with reconstructed images, and MRI, were unremarkable in detecting an occult cervical spine fracture in a patient with ankylosing spondylitis and a spinal cord injury following a fall. The patient was placed empirically in a halo orthosis and a high definition multidetector CT scan was obtained. This study demonstrated a transverse fracture through the fused C6-C7 spinal level. CONCLUSION: Occult fractures in ankylosing spondylitis may not be apparent on routine plain radiographic and MRI studies. In the setting of ankylosing spondylitis, a high index of suspicion must be maintained in all patients presenting with spinal pain following even minor trauma. High-resolution multidetector CT imaging appears to be more sensitive and accurate in the diagnosis of fractures in this patient subgroup than other contemporary imaging methods.

Adult↗

One-year follow-up of two exercise interventions for the management of patients with ankylosing spondylitis: a randomized controlled trial.

OBJECTIVE: To assess the long-term effects on functional and mobility outcomes of two exercise interventions for the management of patients with ankylosing spondylitis. DESIGN: In an extended 12-mo follow-up of a randomized controlled trial, 40 patients who were diagnosed with ankylosing spondylitis according to the modified criteria of New York, allocated to control or experimental groups using a random numbers table, and who performed their respective exercise program at least three times per month, were included in this long-term study. The control group was treated during 15 sessions with a conventional exercise regimen in ankylosing spondylitis, whereas the experimental group received 15 sessions of exercises based on the treatment of the shortened muscle chains following the guidelines described by the Global Posture Reeducation method. These patients were followed up and assessed again 1 yr after entering the study, re-applying the same validated indexes: BASMI (Bath Ankylosing Spondylitis Metrology Index [tragus to wall distance, modified Schober test, cervical rotation, lumbar side flexion, and intermalleolar distance]), BASDAI (Bath Ankylosing Spondylitis Disease Activity Index), and BASFI (Bath Ankylosing Spondylitis Functional Index). RESULTS: The intragroup comparison between follow-up and post-intervention data showed that both groups decrease their clinical and functional measures during the follow-up period. This decrease was only significant for lumbar side flexion and intermalleolar distance measurements, being more significant in the control group (P = 0.001 and P = 0.002, respectively). Intragroup differences between follow-up and pre-intervention assessments revealed that improvements in all mobility measures of the BASMI index and in the BASFI index were partially maintained at the 12-mo follow-up in the experimental group but not in the control group. The intergroup comparison (unpaired t test analysis) between changes on each outcome during the long-term follow-up (post-follow-up; and pre-follow-up) showed no significant differences in the decrease between post-intervention and follow-up data between the study groups. On the other hand, the intergroup comparison between pre-intervention and follow-up data revealed significant differences in almost all mobility measures of the BASMI index (except for cervical rotation) and in the BASFI index, in favor of the experimental group. CONCLUSIONS: An exercise regimen based on the Global Posture Reeducation method and focusing on specific strengthening and flexibility exercises of the shortened muscle chains offers promising short- and long-term results in the management of patients who have ankylosing spondylitis.

Adult↗

Hypothalamic-pituitary-adrenal axis function in ankylosing spondylitis.

OBJECTIVE: To assess basal function and responsiveness of the hypothalamic-pituitary-adrenal (HPA) axis in patients with ankylosing spondylitis during dynamic testing. METHODS: Insulin induced hypoglycaemia (IIH) (Actrapid HM 0.1 IU/kg, as intravenous bolus) was induced in 17 patients and 11 healthy controls matched for age, sex, and body mass index. Concentrations of glucose, adrenocorticotrophic hormone (ACTH), cortisol, insulin, dehydroepiandrosterone sulphate (DHEAS), 17alpha-hydroxyprogesterone, interleukin 6 (IL-6), and tumour necrosis factor alpha (TNFalpha) were determined in plasma. RESULTS: Comparable basal cortisol levels were found in the two groups, with a trend to be lower in ankylosing spondylitis. In the ankylosing spondylitis group, there were higher concentrations of IL-6 (mean (SEM): 16.6 (2.8) pg/ml v 1.41 (0.66) pg/ml in controls; p<0.001) and TNFalpha (8.5 (1.74) pg/ml v 4.08 (0.42) pg/ml in controls; p<0.01). Glucose, insulin, ACTH, DHEAS, and 17alpha-hydroxyprogesterone did not differ significantly from control. The IIH test was carried out successfully in 11 of the 17 patients with ankylosing spondylitis, and the ACTH and cortisol responses were comparable with control. General linear modelling showed a different course of glycaemia (p = 0.041) in the ankylosing spondylitis patients who met the criteria for a successful IIH test compared with the controls. CONCLUSIONS: The results suggest there is no difference in basal HPA axis activity and completely preserved responsiveness of the HPA axis in patients with ankylosing spondylitis. The interpretation of the different course of glycaemia during IIH in ankylosing spondylitis requires further investigation.

17-alpha-Hydroxyprogesterone↗

Finnish HLA studies confirm the increased risk conferred by HLA-B27 homozygosity in ankylosing spondylitis.

OBJECTIVE: To determine the influence of HLA-B27 homozygosity and HLA-DRB1 alleles in the susceptibility to, and severity of, ankylosing spondylitis in a Finnish population. METHODS: 673 individuals from 261 families with ankylosing spondylitis were genotyped for HLA-DRB1 alleles and HLA-B27 heterozygosity/homozygosity. The frequencies of HLA-B27 homozygotes in probands from these families were compared with the expected number of HLA-B27 homozygotes in controls under Hardy-Weinberg equilibrium (HWE). The effect of HLA-DRB1 alleles was assessed using a logistic regression procedure conditioned on HLA-B27 and case-control analysis. RESULTS: HLA-B27 was detected in 93% of cases of ankylosing spondylitis. An overrepresentation of HLA-B27 homozygotes was noted in ankylosing spondylitis (11%) compared with the expected number of HLA-B27 homozygotes under HWE (4%) (odds ratio (OR) = 3.3 (95% confidence interval, 1.6 to 6.8), p = 0.002). HLA-B27 homozygosity was marginally associated with reduced BASDAI (HLA-B27 homozygotes, 4.5 (1.6); HLA-B27 heterozygotes, 5.4 (1.8) (mean (SD)), p = 0.05). Acute anterior uveitis (AAU) was present in significantly more HLA-B27 positive cases (50%) than HLA-B27 negative cases (16%) (OR = 5.4 (1.7 to 17), p<0.004). HLA-B27 positive cases had a lower average age of symptom onset (26.7 (8.0) years) compared with HLA-B27 negative cases (35.7 (11.2) years) (p<0.0001). CONCLUSIONS: HLA-B27 homozygosity is associated with a moderately increased risk of ankylosing spondylitis compared with HLA-B27 heterozygosity. HLA-B27 positive cases had an earlier age of onset of ankylosing spondylitis than HLA-B27 negative cases and were more likely to develop AAU. HLA-DRB1 alleles may influence the age of symptom onset of ankylosing spondylitis.

Adult↗

Increased disease activity is associated with a deteriorated lipid profile in patients with ankylosing spondylitis.

BACKGROUND: Cardiovascular mortality is increased in patients with ankylosing spondylitis. A possible explanation might be a more prevalent atherogenic lipid profile in patients with ankylosing spondylitis than in the general population. It has been postulated that inflammation deteriorates the lipid profile, thereby increasing cardiovascular risk. OBJECTIVE: To explore the association between disease activity and lipid profile in patients with ankylosing spondylitis. METHODS: Disease activity parameters for ankylosing spondylitis and lipid levels (total cholesterol, high-density lipoprotein cholesterol (HDLc) and triglycerides) were measured in 45 patients with ankylosing spondylitis for 6 months after starting treatment with leflunomide or placebo. Findings in this treatment group were compared with those in 10 patients with ankylosing spondylitis treated with etanercept. A specialised regression model, adjusting for repeated measurements, age and sex, was used to assess the influence of the disease activity variables on the lipid levels. RESULTS: Multilevel regression analyses showed significant associations between disease activity parameters and lipid levels-for instance, an increase of 30 mm at the end of the first hour in erythrocyte sedimentation rate was associated with a decrease of about 6% in total cholesterol level and a decrease of about 11% in HDLc levels. Similar significant associations were found between other disease activity parameters and lipid levels. CONCLUSION: Increase in disease activity was associated with decreases in lipid levels. The decrease in HDLc levels tended to be almost twice as large as the decrease in total cholesterol levels, resulting in a more atherogenic lipid profile. Hence, effective treatment of disease activity in patients with ankylosing spondylitis may lower the cardiovascular risk by improving the lipid profile.

Adult↗

Raised titres of anti-klebsiella IgA in ankylosing spondylitis, rheumatoid arthritis, and inflammatory bowel disease.

Serum titres of IgA are raised in ankylosing spondylitis and increased titres of antibodies to klebsiella have also been reported. The humoral response was investigated in ankylosing spondylitis and other inflammatory disorders. IgA antibodies to klebsiella pneumoniae K43 were measured in patients with ankylosing spondylitis, Crohn's disease, ulcerative colitis, and rheumatoid arthritis and in controls. Significantly raised median titres of anti-klebsiella IgA, measured as optical density at 405 nm with an enzyme linked immunosorbent assay (ELISA), were seen among the patients with ankylosing spondylitis (0.7), Crohn's disease (0.8), rheumatoid arthritis (0.6), and ulcerative colitis (0.8) compared with controls (0.4). Activity of disease in ankylosing spondylitis and titres of anti-klebsiella IgA were not correlated. In contrast, titres of anti-klebsiella IgM were significantly lower in patients with ankylosing spondylitis and ulcerative colitis. The increase in the titres of anti-klebsiella IgA may be due to increased permeability of the gut to bacterial antigens, leading to an increased IgA response in the gut mucosa and permitting the release of IgA into the circulation. As the increased antibody titres were seen in Crohn's disease and rheumatoid arthritis as well as in ankylosing spondylitis the response may be nonspecific, occurring because of possible underlying inflammatory bowel disease in these conditions.

Antibodies, Bacterial↗

Brucellar and tuberculous spondylitis: comparative imaging features.

Radiographs, scintigrams, computed tomographic scans, and magnetic resonance (MR) images of 17 patients with brucellar spondylitis and 15 with tuberculous spondylitis were analyzed to identify distinguishing features. Characteristic findings of brucellar spondylitis included predilection for the lower lumbar spine (68% of lesions), bone destruction limited to the end plates, disk collapse (16 of 19 disks), and granulation tissue or localized soft-tissue edema (17 of 19 sites). MR imaging showed diffuse increased signal in vertebrae and disks on long repetition time (TR)/echo time (TE) images in four patients and focal increased signal with normal disks in one. Epidural extension was best seen on short TR/TE images in four. Tuberculous spondylitis was characterized by predilection for the midthoracic spine (73%), vertebral destruction with gibbus deformity (60%), disk collapse, and paraspinal abscesses (14 of 15). On MR images signal intensity of affected vertebrae was similar to but more severe than findings in patients with brucellar spondylitis. Scintigraphy was the least helpful in differentiating the two infections. Lesions of tuberculous spondylitis affecting the lower lumbar spine were difficult to differentiate from those of brucellar spondylitis.

Adult↗

Reactive bone marrow changes in infectious spondylitis: quantitative assessment with MR imaging.

PURPOSE: To evaluate diffuse, reactive bone marrow changes in unaffected vertebrae on magnetic resonance (MR) images in patients with proved infectious spondylitis. MATERIALS AND METHODS: Percentage signal intensity increase of the unaffected bone marrow on contrast material-enhanced MR images (percentage enhancement) was calculated retrospectively in 22 cases of infectious spondylitis and 86 cases without bone marrow disease. Multiple regression analysis and Student t test statistics were performed. RESULTS: Multiple regression analysis showed a significant influence of age and the presence of spondylitis on the values of percentage enhancement (P: <.001). For those aged 35 years or younger, the mean percentage enhancement was 43.2% +/- 4.0 for patients with infectious spondylitis (n = 3) and was 26.4% +/- 8.6 for the control group (n = 23). For those older than 35 years, the mean percentage enhancement was 28.2% +/- 12.2 for patients with infectious spondylitis (n = 19) and 17.5% +/- 7.9 (P: <.001) for the control group (n = 63). Six (27%) of 22 patients with infectious spondylitis showed abnormal percentage enhancement values in unaffected bone marrow when the upper limit of the normal value was 2 SDs above the mean of the control group. CONCLUSION: On MR images, reactive bone marrow changes can be found in unaffected vertebrae in patients with infectious spondylitis. The signal intensity changes and increased percentage enhancement associated with this disease are similar to those of myeloproliferative and diffuse neoplastic disorders and bone marrow stimulation in hemolytic anemia.

Adult↗

Uveitis screening: HLAB27 antigen and ankylosing spondylitis in a New Zealand population.

AIMS: To establish rates of HLAB27-positive uveitis and ankylosing spondylitis in a New Zealand population and to examine characteristics of patients with ankylosing spondylitis that present with uveitis. METHODS: A retrospective audit of 124 consecutive patients presenting to the Acute Eye Clinic at Auckland City Hospital with a diagnosis of either bilateral uveitis or their second episode of uveitis was undertaken. All patients underwent full blood count, erythrocyte sedimentation rate, HLAB27 typing, and radiographs of the sacroiliac joints and lumbar spine. RESULTS: The HLAB27 antigen was detected in 36% of patients undergoing uveitis screening tests. Ankylosing spondylitis was diagnosed on lumbar spine and sacroiliac joint X-ray in 41% of HLAB27-positive patients and in none of the HLAB27-negative patients. No correlation was identified between age at first presentation and diagnosis of ankylosing spondylitis. Ankylosing spondylitis was not associated with elevated white cell count or erythrocyte sedimentation rate. Male gender and detection of the HLAB27 antigen were strongly associated with a diagnosis of ankylosing spondylitis (p<0.001). CONCLUSIONS: Ankylosing spondylitis was detected in 41% of the HLAB27-positive patients and in 0% of the HLAB27-negative patients. Routine lumbar spine and sacroiliac joint X-rays are only recommended for HLAB27-positive patients.

Adolescent↗

[Spondylitis--spondylodiscitis. Pathologico-anatomical morphology and diagnostic problems].

Inflammatory disorders of the spine usually develop in the medullary space of the vertebral bodies, and this clinical picture is identified as spondylitis. If the inflammation involves the intervertebral disk as well as adjacent vertebrae, this lesion is defined as spondylodiscitis. Spondylitis may be brought about by several causes which should be diagnostically clearly defined in order to introduce a suitable therapy. In many cases, no conclusions can be drawn from the radiological structures alone with respect to the underlying disease, an additional biopsy investigation (e.g., by a needle puncture) is therefore required. Most infections conditions of spondylitis represent histologically either an acute, purulent or a chronic, unspecific osteomyelitis. The causative germs are determined by simultaneous bacteriological investigation. The biopsy material of specific spondylitis shows typical histological granulomas which, together with the bacteriological findings, will allow a precise diagnosis to be established. Both spondylitis and spondylodiscitis may also be produced by fungi that can be histologically identified. Parasites (e.g., Echinococci) may also be recognized histologically. In spondylitis of unknown etiology the histological structures do not have a pathognomonic appearance, and therefore clinical and radiological findings should be included in the diagnosis. Diagnostic problems will only be solved by considering a synthesis of all findings.

Candidiasis↗

Is disease severity in ankylosing spondylitis genetically determined?

OBJECTIVE: To assess the role of genes and the environment in determining the severity of ankylosing spondylitis. METHODS: One hundred seventy-three families with >1 case of ankylosing spondylitis were recruited (120 affected sibling pairs, 26 affected parent-child pairs, 20 families with both first- and second-degree relatives affected, and 7 families with only second-degree relatives affected), comprising a total of 384 affected individuals. Disease severity was assessed by the Bath Ankylosing Spondylitis Disease Activity Index (BASDAI) and functional impairment was determined using the Bath Ankylosing Spondylitis Functional Index (BASFI). Disease duration and age at onset were also studied. Variance-components modeling was used to determine the genetic and environmental components contributing to familiality of the traits examined, and complex segregation analysis was performed to assess different disease models. RESULTS: Both the disease activity and functional capacity as assessed by the BASDAI and the BASFI, respectively, were found to be highly familial (BASDAI familiality 0.51 [P = 10(-4)], BASFI familiality 0.68 [P = 3 x 10(-7)]). No significant shared environmental component was demonstrated to be associated with either the BASDAI or the BASFI. Including age at disease onset and duration of disease as covariates made no difference in the heritability assessments. A strong correlation was noted between the BASDAI and the BASFI (genetic correlation 0.9), suggesting the presence of shared determinants of these 2 measures. However, there was significant residual heritability for each measure independent of the other (BASFI residual heritability 0.48, BASDAI 0.36), perhaps indicating that not all genes influencing disease activity influence chronicity. No significant heritability of age at disease onset was found (heritability 0.18; P = 0.2). Segregation studies suggested the presence of a single major gene influencing the BASDAI and the BASFI. CONCLUSION: This study demonstrates a major genetic contribution to disease severity in ankylosing spondylitis. As with susceptibility to ankylosing spondylitis, shared environmental factors play little role in determining the disease severity.

Adolescent↗

Genetic differences between B27 positive patients with ankylosing spondylitis and B27 positive healthy controls.

In a controlled study of the 499 available first degree relatives of 79 consecutive HLA-B27 positive patients with ankylosing spondylitis and 69 HLA-B27 positive healthy blood donors, 19 cases of ankylosing spondylitis were found: 16 (15 B27 positive) among the 282 relatives of the patients with ankylosing spondylitis, and 3 (1 B27 positive, 1 B27 negative, 1 unknown) among the 217 relatives of healthy donors (chi c2 = 5.11; P less than 0.025). However, if all cases of possible spondylarthritis are included, 48 cases of ankylosing spondylitis were found: 37 of 282 relatives of patients with ankylosing spondylitis and 11 of 217 relatives of healthy donors (chi c2 = 8.29; P less than 0.01). Assuming that 50% of relatives of B27 positive individuals carry this antigen, 15 of 142 (10.6%) B27 positive relatives of patients and 2 of 108 (1.9%) B27 positive relatives of healthy subjects (chi c2 = 5.91; P less than 0.025) have ankylosing spondylitis. The relative risk of spondylarthropathy for B27 positive relatives of B27 positive patients compared with relatives of B27 positive healthy subjects is 5.6. Assuming all subjects were evaluated in a similar manner, analysis of these data suggests genetic differences between B27 positive diseased individuals and B27 positive healthy subjects.

Adult↗

Clinical course and prognosis of Brucella spondylitis.

A multicentre prospective study of 593 patients with brucellosis, of whom 58 (9.7%) had spondylitis, was performed in order to evaluate the possible clinical, radiological and evolutionary differences in the different segments of the spinal column. Five of the patients with cervical spondylitis (71%) had compression of the medulla or roots, versus just two (11%) in the dorsal group and nine (21%) in the lumbar group (p less than 0.05). There were no other clinical, haematological or biochemical differences between the three spinal segments, and both the serological response and the percentage of positive blood cultures were also similar in the three groups. The patients with cervical and dorsal spondylitis had a significantly higher number of paravertebral and/or epidural masses than those with lumbar spondylitis (p less than 0.05). Seventy-one percent of the patients with cervical spondylitis made unsatisfactory progress, versus 11% and 5% of those in the dorsal and lumbar groups, respectively (p less than 0.05 and p less than 0.001). In conclusion, given the high incidence of paravertebral and/or epidural masses, the neurological involvement, and the high rate of important functional disabilities, cervical spondylitis should be considered to be a very severe complication of brucellosis, and its treatment and follow-up must therefore be energetic and rigorous in order to detect and correct as early as possible compressions of the neural axis and its roots.

Adolescent↗

[Candida spondylitis. Case report and review of the literature].

Candida species have emerged as important pathogens in human infection. Although a variety of deep-seated candidal infections have been reported, Candida spondylitis has rarely been described. One patient with candida tropicalis spondylitis L I and L II in combination with candida coxitis is presented, and the 31 adult cases with vertebral involvement previously reported are reviewed. Candida spondylitis is noted as a simultaneous occurrence or late manifestation of hematogenously disseminated candidiasis. Spondylitis may not be prevented by a course of Amphotericin B adequate to control the acute episode of disseminated candidiasis, particularly in immune suppressed patients. Spondylitis does not present as a postoperative wound infection. The insidious progression of infection, the nonspecificity of laboratory data, and the failure to recognise Candida as a potential pathogen may lead to diagnostic delay. Diagnosis can be made by either open biopsy or CT controlled needle aspiration. Successful therapeutic regimes have employed combinations of antifungal therapy (Amphotericin B or fluconazole) with radical surgical debridement. Ventral and facultatively dorsal instrumentation is required to stabilize the spine. It is anticipated that the spondylitis will become a more commonly recognised manifestation of hematogenously disseminated candidiasis. A increasing significance of candida species as etiologic agents of infection immune compromised humans has been recognised in the recent years. In those patients whom an antecedent Candida septicaemia was documented, a striking delay of 3.3 months was found between the septicaemia and the onset of symptoms as well as the time of diagnosis.

Adult↗