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Although amyloidosis of the respiratory tract is well recognized, pleural involvement is very rare with only two cases being reported in the past. We report a case of primary amyloidosis with pleural effusion and suggest that pleural involvement and pleural effusion be added to the classification of pulmonary amyloidosis, and that amyloidosis be added to the list of causes of a pleural effusion.
The echocardiographic abnormalities of nine patients with cardiac amyloidosis were correlated with their postmortem findings. All had congestive heart failure and autopsy-proven amyloidosis. M-mode echocardiographic features included (1) small or normal left ventricular (LV) dimensions; (2) thickened interventricular septa and posterior LV walls (89%); (3) left atrial enlargement (89%); and (4) reduced LV distensibility (78%, 100%) and contractility (22%, 44%). Serial M-mode echocardiography revealed that cardiac amyloidosis was initially manifested as diastolic LV dysfunction rather than systolic dysfunction. The final stage of this disease was characterized by severe impairment of both systolic and diastolic LV functions. Two-dimensional echocardiography provided additional features: (1) a more accurate diagnosis of pericardial effusion (67%) and (2) a characteristic "granular sparkling" appearance of the ventricular wall (55%). In four of five cases, these hyperrefractile myocardial echoes corresponded to scattered amyloid deposits histopathologically. There was no correlation between the types of amyloid deposits and the hyperrefractile myocardial echoes. Thus, cardiac involvement in systemic amyloidosis could be non-invasively assessed by M-mode and two-dimensional echocardiography.
Congo red screening of tissue blocks from 37 consecutive autopsies on leprosy patients revealed 7 cases of systemic amyloidosis, indicating a prevalence rate of 19%. 5 were males and 2 females. All were ethnic Chinese. Their ages ranged from 52 to 85 years with a mean of 69 years. Six had lepromatous leprosy while the remaining 1 had tuberculoid leprosy. In all 7 cases, the amyloid was AA in type, being permanganate-sensitive and immunoreactive with anti-human AA protein antiserum. Hepatic deposition was limited to blood vessels, a pattern typical of AA (secondary) amyloidosis. With regard to renal involvement, 4 showed a predominantly vascular pattern of infiltration while 3 exhibited the more ominous glomerular pattern. Three died of chronic renal failure and 2 of congestive cardiac failure attributable to renal and cardiac amyloidosis respectively. One patient succumbed to septicaemia and the remaining 1 to acute myocardial infarction. AA amyloidosis remains a serious and significant complication of leprosy among Malaysians.
At the instance of the variegated anamnesis of a patient with Crohn's disease the unusually rapid development of a secondary amyloidosis of the kidneys is described. Crohn's disease became manifest at the 17th year of age. The severe course of the disease of the last four years with relapsing intraabdominal abscesses and multiple formations of fistulae might be significant for the pathogenesis of the amyloidosis. If the local complications of Crohn's disease are controlled then the amyloidosis still threatens the patient's life. In case the inflammatory process could be cured the consequence would as expected be the alleviation of the amyloidosis. An additional colchicine therapy may then accelerate the process of healing. However, the nature of Crohn's disease is uncertain.
An analysis of cutaneous amyloidosis cases during a five year period, from January 1, 1983 to December 31, 1987, showed that of 78 cases suffering from lichen amyloidosis, 9 patients (11.5%) were male and 71 patients (80.5%) female. Macular skin amyloidosis was observed. Sixty patients (76.9%) were found in the age range of 20-50 years. Forty-nine patients (62.8%) suffered from the disease for 2 years or less. Skin changes were mainly located on the shin areas, the posterior part of the lower thighs and posterior part of the forearms. Treatment with strong topical corticosteroids and keratolytic agents (salicylic acid ointment in higher than 3%) proved to be unsatisfactory. The literature mentions higher incidence of lichen amyloidosis in Chinese, Malaysians and Indonesians than in Caucasians.
Thirty patients with primary amyloidosis in whom cardiac involvement was suspected underwent endomyocardial biopsies. Medical records and biopsies were reviewed and echocardiograms were interpreted by observers who were unaware of the recorded findings. Cardiac amyloidosis was documented by endomyocardial biopsy in 30 (100%) of the patients. In two patients, amyloid was present in only one of four specimens, suggesting that a minimum of four biopsy samples is necessary to eliminate the possibility of sampling error. Only 36 (55%) of 65 biopsy specimens of extracardiac tissues contained amyloid. Findings were positive for amyloidosis in 11 (58%) of 19 rectal biopsies and in 16 (52%) of 31 bone marrow biopsies. Of interest, only the endomyocardial biopsy tissues were positive for amyloid in eight (27%) of the 30 patients, among whom 12 extracardiac biopsies had been performed. Two-dimensional echocardiography was consistent with the diagnosis of amyloidosis in 19 (68%) of 28 patients and was abnormal but nonspecific in the remainder. Endomyocardial biopsy frequently provides information about cardiac involvement with amyloid when biopsy of other organs is negative or echocardiography is nonspecific.
Fifteen male BALB/c mice were given six intermittent oral doses of O.I. ml pristane (2, 6, 10, 14 tetramethylpentadecane) within a period of 9 weeks. Fifteen mice receiving tap water using the same schedule formed the control group. Amyloidosis was first detected in the spleen of a mouse which had died 33 weeks after the first dose and 24 weeks after the last. All six mice which were subsequently autopsied 34-51 weeks after the first dose also showed amyloidosis involving liver and spleen. The most extensive tissue deposits were seen at 37-38 weeks whereas the older mice showed predominantly chronic renal lesions with papillary necrosis, scars and cystic change. Electron microscopy confirmed the identity of the amyloid fibrils and the presence of globular stellate amyloid 'bodies' in liver and spleen. The amyloid deposits were shown to be made up of AA (amyloid associated) protein using an indirect immunoperoxidase method and a monoclonal rat anti-murine AA protein antibody. We did not find any plasmacytomas or increased numbers of plasma cells in the bone marrow. None of the control mice developed amyloidosis. This new experimental model promises to provide a means of studying several aspects of secondary amyloidosis which may be relevant to the clinical situation.
The paper is concerned with an analysis of a course of periodic disease (PD) without and with amyloidosis using also a method of the determination of survival rates in 437 patients followed up for 20 yrs. A course of PD without amyloidosis was benign and did not affect the patients' survival. In the development of amyloidosis the prognosis was unfavorable and determined by a degree of generalization of amyloidosis.
Two cases of amyloidosis were observed in a personal series of 42 patients with the adult from of Still's disease. Two other cases have been previously reported in the literature. In the first case, amyloidosis occurred 18 months after the onset of the disease and involved the kidneys and gastrointestinal tract with a fatal outcome in 6 months. In the second case, renal amyloidosis was observed 4 years after the onset of Still's disease and progressed to renal failure in 7 months, necessitating chronic haemodialysis. These cases show that the adult form of Still's disease may rarely be complicated by amyloidosis, the prognosis of which is particularly poor.
An adult patient with primary systemic amyloidosis associated with nephrotic syndrome and complete atrial-ventricular (A-V) block is described. This case was considered to be a slowly progressive primary amyloidosis because of a long time episode of poor appetite and nephrotic syndrome. Temporal pace making of the heart was not effective presumably due to heart muscle damage, pér sé. Autopsy specimens revealed typical features of primary amyloidosis in light microscopy and immunofluorescence. It is suggested that early detection of amyloid fibers in the heart muscles may improve prognosis in patients with primary amyloidosis.
In spite of an apparent decrease in its occurrence during the last 2-3 decades, amyloidosis associated with rheumatoid arthritis is a significant cause of increased morbidity and early death in both juvenile- and adult-onset rheumatoid arthritis (RA). Like other forms of reactive amyloidosis, the amyloid fibrils associated with rheumatoid disorders are of the protein AA type, and the acute phase reactant SAA, which is complexed to high density lipoprotein in serum, is its putative precursor. Raised SAA appears to be one of the prerequisites for the development of reactive, AA amyloid. Effective treatment of the underlying disorder i.e. rheumatoid arthritis, which lowers the levels of SAA is, therefore, recommended both as a prophylactic and therapeutic measure against reactive amyloidosis. As renal disease is the most severe and life-threatening consequence of reactive amyloidosis, both hemodialysis and renal transplantation are also to be considered in such patients.
In a series of 13 elderly patients with proven prealbumin-related senile systemic amyloidosis (SSA), depressed serum prealbumin values (110.7 +/- 14.1 micrograms/ml) were found as compared to an age-matched control group (175.1 +/- 20.3 micrograms/ml). As expected, there was a significant correlation between serum prealbumin and serum retinol-binding proteins in both groups of patients. Patients with reactive amyloid protein AA amyloidosis had slightly depressed serum prealbumin concentrations, whereas patients with prealbumin-related familial amyloidosis of Swedish type had prealbumin values within normal limits. Since the serum levels of the acute phase reactants, haptoglobin and amyloid-related serum protein AA, were higher in the group of patients with reactive amyloidosis than in patients with SSA, the depression of the prealbumin levels in SSA is not a result of inflammation. Since SSA is known to contain prealbumin, it is possible that a disturbed prealbumin metabolism in old age results in low prealbumin serum values and deposition of amyloid.
A case of amyloidosis revealed by a nephrotic syndrome in a 3 years old girl with juvenile chronic arthritis is reported. The child died from this amyloidosis at the age of 4 years. The main biochemical, clinical and therapeutics aspects of this form of secondary amyloidosis (amyloidosis AA) are discussed.
Amyloid fibrils can be dissolved by dimethylsulphoxide (DMSO) in vitro and in vivo. This prompted us to investigate the therapeutic value of DMSO in renal failure caused by amyloidosis. Two patients with renal failure caused by secondary amyloidosis due to rheumatoid arthritis, showed a remarkable improvement of renal function. The effect of DMSO in amyloidosis secondary to rheumatoid arthritis seems to depend on its anti-inflammatory action, resulting in a decrease in amyloid formation. No evidence was found for an increase in amyloid degradation. The effect of DMSO in primary amyloidosis was inconclusive.
Renal biopsy specimens of 15 patients with renal amyloidosis were studied by immunofluorescence microscopy. The amyloidosis was associated with chronic pulmonary disease in five, rheumatoid arthritis in one, chronic lymphocytic leukemia in one, and familial Mediterranean fever in five patients. In three patients no associated condition could be determined although the pattern of organ involvement resembled that of secondary amyloidosis. IgG and complement (C3) were demonstrated in the glomerular capillary walls and in the mesangium in all patients. The pattern of the deposits was neither granular nor linear. Ig and C3 appeared as large confluent masses or broad ribbon-like segments. In the six patients studied by electron microscopy the fibrillary formation of amyloid was seen in the mesangium and the glomerular capillary walls corresponding to the Ig deposits. No immunofluorescence or ultrastructural differences were observed among the patients with secondary, inherited and leukemia-associated amyloidosis included in this study.
Patients with reactive systemic amyloidosis have a reduced ability to degrade amyloid A protein fibrils in vitro. The amyloid A degrading activity in serum has been attributed to a neutral serine protease or proteases. Our results show that patients with reactive systemic (amyloid A) amyloidosis have low activities of two serum esterases, namely, arylesterase and paraoxonase, whereas the activity of a third esterase, cholinesterase, is normal. The combination of reduced arylesterase (less than 55 kU/L) plus reduced paraoxonase activity (less than 35 U/L) was found in 32% of patients with rheumatoid arthritis complicated by amyloidosis, but in only 5% of a control nonamyloid patient group, including patients with rheumatoid arthritis, liver disease, and hypoalbuminemia (p less than 0.001). A significant correlation between serum arylesterase and amyloid A degrading activity was found (patients with rheumatoid arthritis plus amyloidosis, n = 31, r = 0.51, p less than 0.01; all patients, n = 95, r = 0.34, p less than 0.001). Our results suggest that the amyloid A degrading activity may be closely related to the esterase activity in serum.
The needle aspirate of abdominal fat was investigated for its sensitivity in giving a tissue diagnosis in 32 consecutive patients with systemic amyloidosis. The fat tissue aspirate was stained with Congo red and examined with a polarizing microscope. Positive results were obtained in 95% (18/19) of patients with primary (AL) amyloidosis, 66% (4/6) of patients with secondary (AA) amyloidosis, and 86% (6/7) of patients with the heredofamilial (AF) form. The overall positive yield was 88% (28/32). Abdominal fat tissue aspiration is proposed as a simple, rapid, and effective technique for the diagnosis of amyloidosis. The prevalence of positive results in known amyloid disease are comparable with the rectal biopsy specimen and are more frequent than gingival or skin biopsy specimens.
We studied three patients with amyloidosis who had a significant hemorrhagic disorder secondary to increased fibrinolysis. In two of the three patients, bleeding was one of the major initial problems; in the other patient, bleeding occurred later in the course of the disease. All three patients were men with primary or myeloma-associated amyloidosis (ie, immunoglobulin-related or AL). None of the patients had significantly impaired liver function at the time of the diagnosis of increased fibrinolysis. At admission, fibrinogen levels were 60, 170, and 90 mg/dL in the three patients. All three patients had short euglobulin clot lysis times (less than 30 minutes) and evidence of in vitro clot lysis. One patient appeared to respond to aminocaproic acid (EACA). In patients with amyloidosis and bleeding, fibrinolytic tests should be done; in patients with increased fibrinolysis, a diagnosis of amyloidosis should be considered.