Spinal stenosis (SS) is a manifestation associated with cardiac amyloidosis (CA). However, there is a lack of studies assessing the prevalence of CA among patients with SS. We aimed to address the ...prevalence of CA among patients with SS and YLH.
We performed a cross-sectional study of consecutive patients older than 65 years with SS and yellow ligament hypertrophy (YLH). All the patients were assessed with an electrocardiogram, echocardiogram and biohumoral evaluation. Patients with CA red flags was further studied with cardiac magnetic resonance and 99mTc-DPD scintigraphy. A cohort of patients with confirmed CA and SS was used to assess clinical features associated with CA.
105 patients (75.0 ± 6.6 years old; 45.7% males) with SS and YLH 5.5 5–7 mm were screened. Prevalence of red flags of CA was high and 58 patients presented clinical suspicion of CA. One patient (0.95%) was finally diagnosed of CA.
Patients with confirmed CA presented a more expressive phenotype than the screened population.
Patients with suspected CA had greater YLH than patients without suspicion of CA (6.4 ± 1.3 vs. 5.0 ± 0.8 mm; p < 0.001) and patients with confirmed CA presented greater YLH than the screening population (6.7 ± 1.8 vs. 5.7 ± 1.2 mm; p = 0.018).
Despite red flags of CA are common among patients with SS, the prevalence of confirmed CA was low in our sample of screened patients.
•Red flags of cardiac amyloidosis were a common finding among patients with spinal stenosis and yellow ligament hypertrophy.•55% of patients with spinal stenosis and yellow ligament hypertrophy met current criteria for cardiac amyloidosis screening.•Nearly 2% of patients presenting with clinical suspicion of cardiac amyloidosis had a confirmed diagnosis of cardiac amyloidosis.•Yellow ligament thickness was greater in patients with confirmed cardiac amyloidosis than in patients without cardiac amyloidosis.
It is presented the phenotype of a new compound heterozygous mutation of the genes R384X and Q356X encoding the enzyme of 11-beta-hydroxylase.
Severe virilization, peripheral hypertension, and early ...puberty.
Managed with hormone replacement therapy (corticosteroid) and antihypertensive therapy (beta-blocker), resulting in the control of physical changes and levels of arterial tension.
According to the phenotypic characteristics of the patient, it is inferred that the
mutation carries an additional burden on the
mutation, with the latter previously described as a cause of 11-beta-hydroxylase deficiency. The description of a new genotype, as in this case, expands the understanding of the hereditary burden and deciphers the various factors that lead to this pathology as well as the other forms of congenital adrenal hyperplasia (CAH), presenting with a broad spectrum of clinical presentations. This study highlights the importance of a complete description of the patient's CAH genetic profile as well as their parents' genetic profile.
To determine the potential impact of extra-articular manifestations (EAMs) on disease characteristics and cardiovascular (CV) risk in patients with axial spondylarthritis (axSpA).
This is a ...cross-sectional study from the AtheSpAin cohort, a Spanish multicenter cohort to study atherosclerosis in axSpA. Data on the history of CV events, subclinical carotid atherosclerosis, and disease-related features, including EAMs, were collected.
888 axSpA patients were recruited. Concomitant acute anterior uveitis (AAU), psoriasis (PSO), and inflammatory bowel disease (IBD) were present in 177 (19.9%), 96 (10.8%), and 57 (6.4%) patients, respectively. When compared with axSpA patients without EAMs, a significant increase in past CV events was observed in patients with PSO (9% versus 4%, p = 0.048) and in those with at least one EAM (7% versus 4%, p = 0.032) or with more than one EAM (11% versus 4%, p = 0.022). The frequency of carotid plaques and the values of cIMT were higher in patients with EAMs than in those without EAMs, although only the univariable analysis for carotid plaques in patients with PSO (39% versus 30%, p = 0.038) and for cIMT in patients with AAU (665 ± 156 µm versus 637 ± 139 µm, p = 0.042) and those with at least one EAM (661 ± 155 µm versus 637 ± 139 µm, p = 0.024) showed significant results. In addition, patients with PSO or IBD were found to have specific disease-related features, such as higher ESR at diagnosis, and more frequent use of glucocorticoids and TNF inhibitors than those without EAMs. Also, PSO patients had more commonly peripheral involvement and those with AAU more severe radiographic damage than those without EAMs. The frequency of HLA B27 was higher in patients with AAU and lower in those with PSO or IBD compared to those without EAMs.
Patients with axSpA and EAMs, in addition to displaying their own disease-related features, are likely to have an increased CV risk that appears proportional to the number of EAMs and could be related to proatherogenic factors other than traditional CV risk factors, such as the inflammatory load and the use of glucocorticoids.
Display omitted
To determine the potential impact of sex-specific disease-related characteristics on cardiovascular (CV) disease in axial spondyloarthritis (axSpA).
Cross-sectional study of the Spanish AtheSpAin ...cohort to study CV disease in axSpA. Data on carotid ultrasound and CV disease and disease-related features were collected.
611 men and 301 women were recruited. Classic CV risk factors were significantly less prevalent in women, who also showed a lower frequency of carotid plaques (p = 0.001), lower carotid intima-media thickness (IMT) values (p<0.001) and CV events (p = 0.008). However, after adjustment for classic CV risk factors, only the differences with respect to carotid IMT remained statistically significant. Women showed higher ESR at diagnosis (p = 0.038), and more active disease (ASDAS, p = 0.012, and BASDAI, p<0.001). They had shorter disease duration (p<0.001), lower prevalence of psoriasis (p = 0.008), less structural damage (mSASSS, p<0.001), and less mobility limitation (BASMI, p = 0.033). To establish whether these findings could lead to sex differences in CV disease burden, we compared the prevalence of carotid plaques in men and women with the same level of CV risk stratified according to the Systematic Coronary Risk Evaluation (SCORE). Men included in the low-moderate CV risk SCORE category had more carotid plaques (p = 0.050), along with longer disease duration (p = 0.004), higher mSASSS (p = 0.001) and psoriasis (p = 0.023). In contrast, in the high-very high-risk SCORE category, carotid plaques were observed more frequently in women (p = 0.028), who were characterized as having worse BASFI (p = 0.011), BASDAI (p<0.001) and ASDAS (p = 0.027).
Disease-related features may influence the expression of atherosclerosis in patients with axSpA. This may be especially applicable to women at high CV risk, characterized by greater disease severity and more severe subclinical atherosclerosis than men, suggesting a stronger interaction between disease activity and atherosclerosis in women with axSpA.
•Identifying pro-atherogenic factors in rheumatic diseases can help prevent cardiovascular events.•Axial spondyloarthritis features associated with atherosclerosis remain unclear.•Inflammation and ...disease severity are associated with atherosclerosis in axial spondyloarthritis.
To identify disease-related factors associated with subclinical atherosclerosis and cardiovascular (CV) events in a large series of patients with axial spondyloarthritis (axSpA) and to identify possible differences in the effect of the potential pro-atherogenic factors between ankylosing spondylitis (AS) non-radiographic axSpA (nr-axSpA).
This is a cross-sectional observational study of the AtheSpAin cohort, a Spanish multicenter cohort to study atherosclerosis in axSpA. Subclinical atherosclerosis determined by carotid ultrasound included assessment of carotid intima-media thickness (cIMT) and plaque detection.
639 AS and 167 nr-axSpA patients were recruited. CV risk factors (CRF) and several disease-related factors showed a statistically significant association with subclinical atherosclerosis in the crude analysis. After adjustment for age, sex, and smoking (model 1), associations remained statistically significant for spinal mobility, inflammatory bowel disease, use of prednisone, and Disease-modifying antirheumatic drugs (DMARD) when assessing carotid plaques and for acute phase reactants (APR) at diagnosis, use of prednisone, DMARD, and TNF-inhibitors when measuring cIMT. In model 2, which also included classic CRF as confounding factors to identify axSpA features with a potential independent pro-atherogenic effect, the functional status was the only variable significantly associated with plaques and the use of prednisone and APR at diagnosis with cIMT. No association differences were found between both subtypes of patients. Besides, APR at diagnosis were also associated with subsequent development of CV events that had occurred in 33 patients.
Apart from CRF, atherosclerotic disease in AxSpA is associated with disease-related factors such as inflammatory response and disease severity, with no differences between AS and nr-axSpA.
Display omitted
La tendinopatía calcificada del hombro se caracteriza por el depósito de cristales de hidroxiapatita en uno o varios tendones del hombro. Dentro de los procesos que ocurren en esta entidad está la ...fase de reabsorción, en la que los depósitos podrían migrar hacia estructuras adyacentes. Una muy rara complicación es la migración hacia la unión miotendinosa del tendón correspondiente, la cual provoca una importante reacción inflamatoria muscular que puede objetivarse en pruebas complementarias específicas.
Presentamos un caso clínico de una tendinopatía calcificante del subescapular, con posterior migración hacia la unión miotendinosa causando una miositis del mismo.
Calcific tendinopathy of the shoulder is characterised by the deposit of hydroxyapatite crystals in one or more tendons of the shoulder. Within the processes that occur within this disorder, there is the resorption phase, in which the deposits could migrate towards adjacent structures. A very rare complication is the migration towards the myotendinous junction of the corresponding tendon, which causes a significant muscular inflammatory reaction that can be seen in specific complementary tests.
A clinical case is presented of a subscapular calcific tendinopathy, with subsequent migration to the myotendinous junction, causing myositis of the same.
PDF
