Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UNIPROT:P01889 (ankylosing spondylitis)
 5,717 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The case of a 50 year old male with the Fiessinger-Leroy-Reiter syndrome, ankylosing spondylitis and generalised pustular psoriasis is reported. This condition wax complicated by non-obstructive cardiomyopathy, congestive cardiac failure and first-degree atrioventricular block, the site of which was localised by electrophysiological studies (nodal block with an infrahisian conduction defect). After failure of several therapeutic regimes, a spectacular improvement was obtained with Methotrexate associated with a diuretic; the signs of heart failure regressed and the cardiomyopathy stablised. A parallel improvement was seen in the skin, cardiac and articular lesions and has been maintained with an 18 months follow-up. Left ventricular performance was studied by echocardiography. The mechanism of the beneficial effect of Methotrexate is unclear; this therapeutic trial is to be extended to include other cases of primary cardiomyopathy without obstruction.
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PMID:[Fiessinger-Leroy-Reiter syndrome with non-obstructive cardiomyopathy treated with methotrexate]. 11 79

Echocardiography has been useful in the evaluation of congestive and hypertrophic cardiomyopathies. We present echocardiographic findings in seven patients with infiltrative cardiomyopathy due to amyloid. Cardiac amyloidosis was documented at autopsy in two patients, and the diagnosis was suggested by clinical, echocardiographic, tissue, or hemodynamic findings in the other five. Hemodynamic findings in three patients mimicked constrictive pericarditis; and autopsy was performed on one of the three and showed a normal pericardium. Underlying disorders were multiple myeloma (five patients), ankylosing spondylitis (one patient), and an unknown disorder (one patient). The basic echocardiographic findings in infiltrative cardiomyopathy due to amyloid were (1) symmetrically increased left ventricular wall thickness (in the absence of hypertension or aortic valvular disease), (2) hypokinesia and decreased systolic thickening of the interventricular septum and left ventricular posterior wall, and (3) small to normal size of the left ventricular cavity. Two patients also had small pericardial effusions. Thus, in a patient with congestive heart failure, these echocardiographic findings should suggest infiltrative cardiomyopathy.
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PMID:Echocardiographic manifestations of infiltrative cardiomyopathy. A report of seven cases due to amyloid. 100 Oct 49

Reiter's syndrome was found in three men who presented with cardiac conduction disturbances. In two patients, Reiter's syndrome had been present for more than 30 years and had been previously unrecognized. These patients included a 67 year old man with complete heart block of 13 years' duration, and his son, who had left bundle branch block and chronic generalized cardiomyopathy. A chart review of 19 other patients with Reiter's syndrome who were seen at this institution disclosed five patients with conduction abnormalities. Transient first-degree heart block was the most common disturbance detected and was usually associated with active Reiter's syndrome. Some conduction abnormalities appeared after a long latent period at a time when other manifestations of Reiter's syndrome were inactive. An association with this disorder was therefore not obvious. In all five patients with Reiter's syndrome and conduction disturbances, testing for B27 antigen gave positive results. Both clinical and histopathologic changes in the heart in Reiter's syndrome are analogous to those in ankylosing spondylitis, also associated with B27 antigen. We suggest that the heart, like the joints and iris, may be a target organ for B27-associated disease by a mechanism that remains to be defined.
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PMID:Cardiac conduction abnormalities in Reiter's syndrome. 712 60

The authors' objective was to determine by 2-dimensional echo Doppler (2DECHO) the cardiac abnormalities in juvenile onset ankylosing spondylitis (JOAS) and adult onset ankylosing spondylitis (AOAS) in male patients with long-term disease. Twenty patients with JOAS, 31 with AOAS, and 20 healthy controls of the same age and gender without cardiopulmonary symptoms were studied. Using 2DECHO, the heart dimensions were determined according to American Society of Echocardiography guidelines. The left ventricle ejection fraction (LVEF) was calculated by Teichholz's formula. Cardiomyopathy was established when 2DECHO had diminished LVEF. Statistics used were the Student t and Fisher test, chi2, and ANOVA. Ninety percent of JOAS and 51% of AOAS patients were B27+ (p=0.005). The disease duration was 19.3 +/- 8.8 years in JOAS and 14.8 +/- 12.8 years in AOAS (p=NS). Age at the time of the study was 30.7 +/- 9.9 years in JOAS vs 40.3 +/- 12.7 in AOAS (p=0.003), and vs 40.2 +/- 17 years in controls (p=NS). There was a higher frequency of cardiomyopathy in AOAS (32.2%) than in JOAS (25%) and the controls (0%) (p=0.01). Patients with JOAS had a higher mitral valve gradient (25%) than AOAS patients (19%, p=NS) and controls (0%, p=0.04). Abnormal aortic ring reflectance was shown in 19% of AOAS vs 0% abnormalities in JOAS and controls (p=0.01). The aortic root diameter was increased in 58% of AOAS, 30% of JOAS, and 0% of controls (p=0.001). The frequency of 2DECHO abnormalities was increased in cardiopulmonary asymptomatic spondylitis patients. Despite the high frequency of B27+, JOAS had a lower frequency of aortic abnormalities than AOAS. Mitral valve gradient was found in JOAS and in AOAS that could contribute to a decreased ejection fraction and to left ventricular dysfunction.
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PMID:Two-dimensional echo Doppler findings in juvenile and adult onset ankylosing spondylitis with long-term disease. 1151 93

Seronegative spondylarthritis are frequently characterised by extra-articular manifestations. They are frequently in recurrent uveitis. Between the cutaneous manifestations should be mentioned erythema nodosum, typical of inflammatory bowel diseases, and keratoderma blenorrhagicum, in the Reiter's syndrome. Cardiac complications in ankylosing spondylitis (AS) include aortic valvular regurgitation and arrhythmia and, more rarely, mitral valvulopathy, cardiomyopathy and pericarditis. Pulmonary involvement in AS includes ventilatory restrictive syndrome and fibro-bullous disease of the apex. Vertebral osteoporosis is a very important extra-articular manifestation because of the possibility of spontaneous fractures of the vertebrae. Central neurological manifestations include medullary compression from cervical sub-luxation while the most important peripheral involvements are lumbar stenosis and the cauda equina syndrome. Type AA amyloidosis is a rare late complication of the AS, possible cause of death especially in patients with aggressive disease. Kidney complications can be observed as consequences of prolonged anti-inflammatory therapy, but the most frequent renal complications are amyloidosis and mesangial IgA segmental and focal glomerulonephritis.
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PMID:[Extra-articular manifestations of seronegative spondylarthritis]. 1683 61

Ankylosing spondylitis presents challenges for the obstetric anesthesiologist in administering neuraxial anesthesia or managing the airway. A pregnant patient with ankylosing spondylitis, cardiomyopathy and preeclampsia requiring cesarean delivery was managed with an awake nasotracheal fiberoptic intubation. The use of topical cocaine, epinephrine, phenylephrine, and oxymetazoline to produce nasal vasoconstriction is discussed. Selective alpha-2 agonists that can potentially provide nasal mucosa vasoconstriction and placental vasculature vasodilation are also discussed.
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PMID:Topical vasoconstrictor use for nasal intubation during pregnancy complicated by cardiomyopathy and preeclampsia. 2131 77

Ankylosing spondylitis is a chronic inflammatory condition that usually affects young men. Cardiac dysfunction and pulmonary disease are well-known and commonly reported extra-articular manifestation, associated with ankylosing spondylitis (AS). AS has also been reported to be specifically associated with aortitis, aortic valve diseases, conduction disturbances, cardiomyopathy and ischemic heart disease. The pulmonary manifestations of the disease include fibrosis of the upper lobes, interstitial lung disease, ventilatory impairment due to chest wall restriction, sleep apnea, and spontaneous pneumothorax. They are many reports detailing pathophysiology, hypothesized mechanisms leading to these derangements, and estimated prevalence of such findings in the AS populations. At this time, there are no clear guidelines regarding a stepwise approach to screen these patients for cardiovascular and pulmonary complications.
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PMID:Cardiopulmonary manifestations of ankylosing spondylitis. 2154 38

Ankylosing spondylitis often involves a heart, such as aortic or mitral regurgitation, conduction disorder, or cardiomyopathy. We present a 34-year-old male patient with ankylosing spondylitis who has severe aortic regurgitation, mild mitral stenosis, and a conduction disturbance of the left bundle branch block, identified using multimodal images.
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PMID:Full-blown cardiac manifestations in ankylosing spondylitis. 2753 79

A 58 years old male with a long-standing history of HLA-B27 positive ankylosing spondylitis presented with increasing fatigue and dyspnea on exertion. He had left ventricular dysfunction and enlargement, flail right coronary leaflet of aortic valve with severe eccentric aortic insufficiency along with left ventricular non-compaction in echocardiography. The most common cardiac manifestations of ankylosing spondylitis are aortic insufficiency and conduction disturbances. Involvement of myocardium, in the form of dilated cardiomyopathy and restrictive cardiomyopathy, has also been reported. This case presents a very rare association of ankylosing spondylitis with non-compaction cardiomyopathy.
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PMID:Left ventricular non-compaction in a patient with ankylosing. 2821 Apr 76