Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0018801 (heart failure)
 72,216 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

An anti Wa antibody was reported as a new t-RNA related protein antibody in 1986. This autoantibody is now considered specific for the diagnosis of progressive systemic sclerosis (PSS). Up to date only 5 cases with anti Wa antibody have been identified. We report here an autopsied case of PSS with this antibody. A 53 years old female was admitted to our hospital because of dry cough and dyspnea in Sep 1987. There were fine crackles and chest X ray revealed interstitial pneumonia. The progressive respiratory failure was treated by steroid pulse therapy effectively. Sclerotic skin changes of hand began to appear in Sep 1988 and rapidly progressed to arms, chest and forehead by Dec 1988. A skin biopsy confirmed PSS changes. An anti Wa antibody was detected by double immunodiffusion and the protein antigen was associated with t-RNA when immunoprecipitation was conducted. She died of heart failure in July 1989. An autopsy revealed the diffuse fibrotic change of the heart and the lung. Cases with anti Wa antibody were shortly reviewed from the literature.
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PMID:[An autopsied case of progressive systemic sclerosis with anti Wa antibody who showed a rapid progression]. 144 86

Aortic valve lesions in progressive systemic sclerosis (PSS) are very uncommon. To our knowledge, aortic regurgitation (AR) associated with PSS has not been reported previously. We would like to report the case of a 58-year-old woman who had PSS with AR due to Raynaud's symptom, fever, positive ANA, accelerated ESR, and diastolic blowing murmur along the left sternal border. After treatment with adreno-cortico steroid and an immunosuppressive agent, the patient improved serologically and symptomatically. However, she was later admitted to our hospital again due to heart failure with progressive AR. She died of refractory heart failure with severe AR and tricuspid regurgitation (TR). The former was caused by aortic cusp lesions and the latter by pulmonary hypertension. An autopsy confirmed the diagnosis of PSS, which was found to have involved the heart, lungs and pancreas. Vasculitis with infiltration and fibrotic changes were noted in these organs. Moreover, there were fibrotic thickenings and shortenings in the aortic cusps with cell infiltration. There were no indications of rheumatic disease. These results suggest that the cause of our patient's aortic valve disease may have been PSS vasculitis.
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PMID:[Report of a case with aortic regurgitation in progressive systemic sclerosis]. 151 78

Myositis and myocarditis have been reported in progressive systemic sclerosis, and these patients have had favorable therapeutic responses to intravenous pulse methylprednisolone. Thus far, premortem biopsy documentation of myocarditis and myocardial fibrosis has not been reported in such patients. We report the case of a patient with subacute congestive heart failure six months after she developed Raynaud's phenomenon. Clinical examination was typical of scleroderma but there was no proximal muscle weakness. She had elevated creatine kinase and MB-creatine kinase and laboratory evidence of hypothyroidism. Echocardiogram demonstrated four-chamber dilatation and severe left ventricular dysfunction. Cardiac catheterization revealed normal epicardial coronary arteries and severely decreased cardiac index. A skin biopsy specimen of the forearm was consistent with diffuse systemic sclerosis, and an endomyocardial biopsy specimen demonstrated mild fibrosis and lymphocytic infiltrate. Her heart failure initially improved with digoxin, furosemide, and enalapril. She also received L-thyroxine and intravenous methylprednisolone. The heart failure progressed over the next six weeks and she died. Patients with scleroderma and new-onset heart failure may have acute myocarditis.
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PMID:Acute myocarditis in fulminant systemic sclerosis. 154 Nov 69

Systemic scleroderma is a general disease of the connective tissue which may affect practically any organ. The authors describe the case of a man where in the course of the disease affection of the heart with symptoms of severe cardiac failure became the dominating symptom.
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PMID:[Heart involvement in systemic scleroderma]. 156 79

We have characterized a clinical significance of anti-U3RNP, anti-7-2RNP, anti-RNA polymerase I and anti-PM-Scl antibody, autoantibodies to nucleolar proteins detected by immunoprecipitation method in patients with systemic sclerosis (SSc). In 248 patients with SSc, anti-U3RNP antibody was positive in 9 (3.6%), anti-7-2RNP antibody was positive in 7 (2.8%) and anti-RNA polymerase I antibody was positive in 3 (1.2%). But none of 248 patients was positive for anti-PM-Scl antibody. Anti-U3RNP antibody positive SSc patients showed significantly lower frequency of joint and lung involvements, compared with anti-U3RNP antibody negative SSc patients. Anti-7-2RNP antibody was found only in patients with limited scleroderma. The anti-7-2RNP antibody could be detected before appearance of skin thickening, so this indicate the usefulness of detecting anti-7-2RNP antibody in the early stage of SSc. Two of 3 anti-RNA polymerase I antibody positive patients were classified as diffuse scleroderma. All anti-RNA polymerase I antibody positive patients had high incidence of internal organ involvements including lung, heart and kidney, so two of these patients died of heart failure. These data showed the close clinical association of antigenic specificities of anti-nucleolar antibodies analysed by immunoprecipitation method, and indicated the usefulness of detecting these anti-nucleolar antibodies in subgrouping of patients with SSc.
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PMID:[Clinical significance of anti-nucleolar antibodies detected by immunoprecipitation method in patients with systemic sclerosis]. 160 15

A 21-month-old infant presented with simultaneous localized scleroderma and severe cardiomyopathy with heart failure. Cardiac abnormalities and serological changes (positive rheumatoid factor assay, elevated IgM and IgG levels, and elevated erythrocyte sedimentation rate) reverted to normal with prednisone therapy, and there was substantial, though incomplete, resolution of her skin changes during the same period. To our knowledge, this is the first patient with definite, clinically significant cardiac involvement associated with focal scleroderma. The possibility of internal organ involvement, including cardiac involvement, must be considered with focal scleroderma as well as with progressive systemic sclerosis.
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PMID:Focal scleroderma and severe cardiomyopathy. Patient report and brief review. 199 94

We have studied the cardiac manifestations of connective tissue diseases. In 213 files of patients with connective tissue disease of the Department of Medicina I, Hospital Santa Maria, during 21 years. Cardiac manifestations were observed in 63 (90%) SLE. Pericarditis was the most frequent manifestation and occurred in 33 patients (43%). The cardiac manifestations were observed in 40 (41%) RA. Pericarditis appeared in 11 patients, valvulopathy in 12 patients and coronaropathy in 11 patients. In 10 of PD diagnosed patients, ECG abnormalities were the only findings. Arrhythmias, conduction disturbances, cardiac failure and coronaropathy were the cardiac manifestations of PSS in 11 patients. Polyarteritis Nodosa patients had myocardial ischemia and another had a malignant hypertension diagnosis. We found pericardial effusion in one patient and angina in another one with MCTD diagnosis. We did'nt find any cardiac manifestation in AS. Cardiac manifestations are frequent in connective tissue diseases. The ECG, ECO and pathology show abnormal findings. Although there is not clinical cardiological expression of the disease we suggest the use of ECG. ECO Holter electrocardiography and isotopic myocardial perfusion scan technics in the clinical evaluation of such patients.
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PMID:[Cardiac manifestations of connective tissue diseases]. 269 91

In conclusion, systemic sclerosis is both a fascinating and frustrating affliction. It is a systemic disease of multiple stages. Prognosis is dependent on the site and extent of visceral involvement. There is evidence to implicate the vascular system as the primary target organ of the disease. The cardiovascular manifestations include myocardial fibrosis, pericarditis, and a variety of arrhythmias and conduction abnormalities. Intractable heart failure or sudden cardiac death can ensue. Cardiac involvement in systemic sclerosis portends an ominous prognosis, and is probably most directly related to the extent of myocardial fibrosis which is present. The pathogenesis of myocardial fibrosis has not been determined, but it appears to be a result of an impairment of myocardial perfusion at both the small artery and microvasculature level. Obstructive, vasospastic, and devascularization factors all may be playing a role.
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PMID:The cardiovascular manifestations of systemic sclerosis (scleroderma). 348 17

A 27-year-old black woman with cardiac failure, angina pectoris and Raynaud's syndrome is presented. Skin biopsy and barium studies established the diagnosis of scleroderma (progressive systemic sclerosis (PSS)). Systemic lupus erythematosus (SLE) was strongly suggested by the results of immunological studies and increasing severity of renal failure. Because of the possibility of a cardiomyopathy, cardiac catheterization, selective coronary angiography and right ventricular endomyocardial biopsy were carried out but failed to show any histological features of either SLE or PSS. The patient went into progressive renal failure despite immunosuppressive therapy and plasmapheresis and died; consent for autopsy was refused. A final diagnosis of mixed connective tissue disease (MCTD) was made. The salient features of cardiac involvement in SLE, PSS and MCTD are outlined.
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PMID:Cardiac involvement in mixed connective tissue disease. A fatal case of scleroderma combined with systemic lupus erythematosus. 406 33

Scintigraphic examination of the myocardium, using 99mTc-labelled pyrophosphate, was carried out in 17 patients suffering from systemic sclerosis. This connective tissue disorder very often affects the myocardium secondarily. The results of the cardiac scan were compared with the information obtained from the electrocardiogram of systolic time intervals. In addition, spirometry was undertaken to detect a potential relation between cardiac and pulmonary involvement. The scan was found to be positive in seven patients and electrocardiographic findings were pathological in five patients only. The systolic time intervals were abnormal in three patients only. A ventilation disturbance was recorded in 10 patients. No clear relation was found between the results of the individual examinations. It is concluded that pyrophosphate heart scintigraphy may detect myocardial impairment in some cases of systemic sclerosis before it is manifested by heart failure. Examination of systolic time intervals is of little importance.
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PMID:Pyrophosphate heart scan in patients with progressive systemic sclerosis. 627 70


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