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Query: UMLS:C0018801 (heart failure)
 72,216 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

From 1960 through 1972, 236 cases of amyloidosis with histologic proof were found. The amyloidosis was primary (without evidence of preceding or coexisting disease) in 132 cases (group 1) and associated with multiple myeloma in 61 (group 2). Secondary amyloidosis appeared in 19 cases (associated with rheumatoid arthritis or osteomyelitis in two-thirds of them). There were 22 patients with amyloid localized to a single organ (bladder, lung, skin, or larynx in more than half of them). Two patients had familial amyloidosis. In group 1 and group 2, the most common presenting symptoms were fatigue, weight loss, edema, dyspnea, light-headedness or syncope, and paresthesias. Symptoms of the carpal-tunnel syndrome were frequent. The liver was palpable in almost 50% of the series, but splenomegaly was an initial finding in less than 10%. Macroglossia was recorded in 26% of group 2 and in 12% of group 1. Enlargement of submandibular structures was noted in about 10% of cases; and purpura, particularly around the eyes, was a significant feature. Substantial numbers of the patients had carpal-tunnel syndrome, nephrotic syndrome, congestive heart failure, sprue, peripheral neuropathy, or orthostatic hypotension. Approximately 50% of patients had renal insufficiency at the time of diagnosis. Proteinuria was found in more than 90%. A monoclonal protein was found in the serum of 49% of group 1 and in 74% of group 2. Monoclonal proteins were found in the urine of 35% and 81%, respectively. Only 12% of patients in group 1 had no monoclonal protein when both serum and urine were analyzed, and all patients of group 2 had a monoclonal protein in the serum or urine when both were analyzed. Lambda light chains were more common than kappa. None of the patients in group 1 had more than 15% plasma cells in the marrow, whereas more than half of group 2 had more than 15% plasma cells. Roentgenograms showed no evidence of skeletal disease in 94% of group 1, but 50% of group 2 had skeletal abnormalities. Rectal biopsy was positive for amyloid in 84% of cases. Kidney, liver, and carpal-tunnel biopsies were positive in 90% or more. Follow-up of all 193 patients in groups 1 and 2 revealed that 80% of group 1 and 97% of group 2 had died. The median survival was 14.7 months in group 1 and 4 months in group 2. Cardiac failure was the most common cause of death, accounting for 30% of the fatalities. We also reclassified all cases by the method of Isobe and Osserman (105), which is based on clinical patterns: pattern I--principal involvement of tongue, heart, gastrointestinal tract, muscle, nerves, skin, and carpal ligaments; pattern II--principal involvement of liver, spleen, kidneys, and adrenals; and mixed pattern I and II. This analysis failed to reveal predictive value in the clinical pattern classification, and did not discern the survival differences between primary amyloidosis (group 1) and amyloidosis with myeloma (group 2). Consequently, for the present we prefer the classification used in this study.
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PMID:Amyloidosis: review of 236 cases. 115 71

We reviewed the autopsy material of 169 patients with rheumatoid arthritis (RA) and studied the cardiac changes is these patients. Systemic vasculitis was observed in 26 cases (15.38%) among 169 patients with RA. In 17 cases (10%) we found vasculitis of the subepicardial and/or intramural coronary vessels. Coronary arteritis or arteriolotis has led to multifocal small, and/or large myocardial infarctions in 10 cases (5.9%) and was the cause of progressive cardiac insufficiency, the direct cause of death. Multifocal circumscribed myocardial infarction reported in rheumatoid disease. In 8 cases rheumatoid nodules were found in the myocardium, 3 of them related to vasculitis. We suggest that rheumatoid nodules are the most severe form of necrotizing granulomatous vasculitis. Pathognomic nodular rheumatoid pericarditis was seen in 3 cases and diffuse rheumatoid pericarditis in another case. Pathognomic nodular valvulitis was found in 7 cases. Rheumatoid nodules localized to the epi-, myo-, or endocardium were observed on 9 patients. Generalized secondary amyloidosis was observed in 32 (18.93%) of the 169 patients with RA. Secondary amyloidosis was prevalent in the heart in 29 of 32 cases (relative frequency: 90.62%).
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PMID:Cardiac changes in rheumatoid arthritis. 136 61

Secondary amyloidosis is an important complication that may have a strong influence on the prognosis of patients with rheumatoid arthritis (RA). We studied 21 RA patients with secondary amyloidosis. The two major initial signs were gastrointestinal symptoms and renal involvement. When 15 of the 21 patients were diagnosed as having secondary amyloidosis, they displayed renal involvement including proteinuria, hematuria and hypercreatininemia. The 15 patients with amyloidosis were either subjected to dialysis or died within 35 months on the average. The causes of death in 13 patients were cardiac failure, gastrointestinal bleeding and infection, which were strongly implicated with renal failure. Dialysis was applied to seven patients. Three of them were maintained with chronic dialysis. We discussed the induction-time and the method of dialysis in patients with amyloidosis secondary to RA.
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PMID:[Clinical characteristics and prognosis of secondary amyloidosis in patients with rheumatoid arthritis--renal involvement and therapy]. 144 80

Secondary amyloidosis (SA) affects cardiac texture and function by interstitial fibrosis. Doppler tissue imaging (DTI) may quantify heart function through the assessment of myocardial velocities. Echocardiographic findings of early cardiac amyloidosis (CA) without heart failure (HF) caused by SA were determined both by standard methods and DTI. It was then determined whether DTI is superior to conventional echocardiography in documenting early CA due to SA. Twenty-five patients with SA who had CA without HF (group 1) were compared with 25 healthy control subjects (group 2). After standard echocardiography, systolic (s), early (e) and late diastolic (a) velocities of interventricular septum, anterolateral, and anterior and inferior walls were measured from mitral annulus by DTI. The averages were called (s(mean)), (e(mean)), and (a(mean)), respectively. Fractional shortening (FS) and ejection fraction (EF) values of groups 1 and 2 were similar. Standard Doppler echocardiographic values were not typical for a specific diastolic abnormality. The (s(mean)) and (e(mean)) for group 1 were lower but (a(mean)) was higher compared with group 2 (all P < .05). The group 1 (e(mean)/a(mean)) was lower (P < .0001) and (E/e(mean)) was higher (P = .003) than in group 2 (both P < .05). (E/e(mean)) and (E/e(lateral wall)) ratios were positively correlated (r = 0.74, P < .05). In patients with early CA due to SA without HF, by DTI, (s(mean)) and (e(mean)) velocities decrease and (a(mean)) velocity increases. These may be markers of subclinical CA of SA when standard echocardiography is not informative. (E/e(mean)) ratio may be an alternative index to (E/e(lateral wall)).
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PMID:Doppler tissue imaging of the heart in secondary amyloidosis. 1641 51

Involvement of the heart is a common finding in amyloidosis. The heart is usually infiltrated by amyloid fibrils in primary amyloidosis and age-related forms of amyloidosis, less commonly in transthyretin familial amyloidosis, and rarely in secondary amyloidosis. The most common clinical presentation is restrictive cardiomyopathy with right-sided heart failure. The second most frequent presentation is congestive heart failure due to systolic dysfunction, followed by arrhythmias and orthostatic hypotension. The diagnosis of amyloidosis requires tissue sample confirmation; at present, Congo red staining in polarized light is the diagnostic method of choice. The characterization of protein fibril type by immunohistochemistry or biochemistry is essential for patient prognosis and treatment. The therapeutic approach consists of specific treatment of amyloidosis and supportive treatment for cardiac-related symptoms. The treatment depends on the type of amyloidosis and the stage of disease. The mainstay of supportive treatment of cardiac failure is diuretic therapy. Primary amyloidosis treatment protocol includes melphalan and prednisone chemotherapy. Heart transplantation is only a palliative treatment. Stem cell transplantation is an emerging treatment alternative. Combination therapy of melphalan and stem cell transplantation has been shown to be a promising treatment strategy. Secondary amyloidosis requires aggressive treatment of the associated inflammatory and neoplastic process. Age-related (senile) amyloidosis benefits from supportive cardiac treatment when applicable. Transthyretin amyloidosis, the most common cardiac hereditary amyloidosis, is treated by liver or combined liver-heart transplantation. New therapies based on chemical and immunologic reaction with amyloid or its precursor are under intensive development.
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PMID:Current treatment in cardiac amyloidosis. 1707 11

Renal amyloid involvement results, especially, from AL (primary) or AA (secondary) amyloidosis. The extent of amyloid tissue deposits in the kidneys and the clinical course of amyloidosis not only depend on the type of basic process but also reflect the time of diagnosis and the ability to affect the underlying disease. We analyzed laboratory and clinical data from patients with bioptically proven renal amyloidosis. Renal amyloidosis was found in 99 patients (4.65%) from an overall number of 2,128 renal biopsies (RB) performed in our department during a period of 11 years (from 1995 to 2006). AA amyloidosis was diagnosed in 46 patients. Nephrotic syndrome was diagnosed in 27 patients (59%) with AA amyloidosis; all these patients had different degrees of proteinuria. Impaired renal function was discovered in 24 patients (52%); in three of these patients (6.5%) we had to start renal replacement therapy. Patients were treated with corticosteroids, disease-modifying antirheumatic drugs (DMARDs), and biological therapy in various regimens. Nine patients (19.5%) died during the one-year follow-up period; complications such as sepsis and cardiac failure were the leading causes of death. Median survival in the AA group was 54 months. Although for approximately half of patients different treatment regimens can lead to a partial remission or disease stabilization, the prognosis of patients with amyloidosis could be regarded as unsatisfactory.
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PMID:Renal AA amyloidosis: survey of epidemiologic and laboratory data from one nephrology centre. 1918 13