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PubMed1985

Senile amyloidosis and neuron binding antibody in the aging Syrian hamster.

The effects of age, sex, and irradiation on the genesis of amyloidosis, neuron-binding antibody (NBA), and the concomitant appearance of these two phenomena were studied in a colony of Syrian hamsters. In nonirradiated controls amyloidosis increased in prevalence with age after 12 months, and prevalence was higher in females than in males. Irradiation had the effect of advancing the appearance of amyloidosis to the 7-12 months group but did not intensify the amyloidotic process. IgG binding to the nucleus or cytoplasm of neurons was rare, and, despite the fact that IgM and IgA binding to these structures was present in about one-third of the animals, there was neither an aging nor an irradiation effect. The only statistically significant findings with respect to the concomitant occurrence of amyloid and NBA were negative correlations between nuclear IgM and IgA binding and amyloidosis. Of the various species thus far studied, the hamster is the first in which there has been no aging effect in respect to NBA.

Aging↗
PubMed1996

Diagnostic aspects of beta 2-microglobulin amyloidosis.

Osteoarticular symptoms and signs, such as carpal tunnel syndrome, periarthritis humeroscapularis, synovial and joint inflammation, lead to the clinical diagnosis of beta 2-microglobulin (beta 2-M) amyloidosis. Radiologically, destructive arthropathy, spondylarthropathy and periarticular cystic bone radiolucencies can be demonstrated by plain X-ray and conventional and computed tomography. Magnetic resonance is used to visualize amyloid masses in special locations such as the cervical spine. Joint ultrasonography demonstrating thickening of synoviae and tendons has become a useful non-invasive diagnostic tool, although it is not specific for beta 2-M amyloidosis, and results depend on observer experience. Efforts to develop more specific methods for demonstration of amyloid deposits have led to two scintigraphic techniques based on the injection of radiolabelled proteins which are incorporated into the amyloid tissue. One method uses serum amyloid P component, a non-specific constituent of all types of amyloid. An alternative imaging technique based on radiolabelled beta 2-M as the specific precursor molecule of beta 2-M amyloidosis appears to be a more sensitive diagnostic method. Both tracer techniques have been used so far only in small single-centre clinical studies, and a more widespread application will require the introduction of recombinant material as the protein source for labelling. The 'gold standard' for the definitive diagnosis of beta 2-M amyloidosis, and also for reference in the evaluation of new diagnostic techniques, remains the histomorphological demonstration of beta 2-M amyloid by Congo red staining, green birefringence under polarized light, positive immunostaining with anti-beta 2-M antibodies, and electronoptic visualization of amyloid fibrils in tissue obtained invasively by biopsy, surgery or autopsy.

Amyloidosis↗
PubMed1996

Clinical and therapeutic aspects of renal amyloidosis.

Renal amyloidosis is not a frequent disease: its annual frequency is approximately 2.5-2.8% of all renal biopsies. Individual cases are normally rare and for this reason clinical findings and therapeutic results obtained by clinical multicentre studies are well considered. This review describes the clinical and therapeutic aspects of renal amyloidosis and refers particularly to the results obtained by the Italian Study Group for renal amyloidosis and by the multicentre Italian study protocol for AL amyloidosis.

Amyloidosis↗
PubMed2000

Clinical manifestations of AB-amyloidosis: effects of biocompatibility and flux.

BACKGROUND: Highly permeable biocompatible dialysis membranes may postpone the development of AB-amyloidosis, but the relative contribution of enhanced flux or reduced inflammation by highly biocompatible membranes and sterile dialysis fluid remains unknown. METHODS: In this retrospective investigation, 89 patients with end-stage renal disease maintained on regular haemodialysis for at least 10 years and treated with one type of dialysis membrane exclusively were selected for analysis. They were divided into three groups: low-flux, bioincompatible cellulose (I), low-flux, intermediately biocompatible polysulphone or PMMA (II), or high-flux, highly biocompatible polysulphone or AN69 (III). In addition, the patients were analysed according to the microbiological quality of the dialysis fluid, which had been tested regularly and was classified either as standard or as intermittently contaminated. The clinical manifestations indicative of AB-amyloidosis, namely, carpal tunnel syndrome, arthropathy and bone cysts, were diagnosed after recruitment. RESULTS: Clinical symptoms were most pronounced in group I, intermediate in group II, and lowest in group III. Patients treated with intermittently contaminated dialysis fluid showed a higher prevalence of AB-amyloidosis than patients with less contaminated dialysis fluid. Logistic regression analysis demonstrated that the flux characteristics of the dialyser and the microbiological quality of the dialysis fluid as well as the biocompatibility of the dialyser were independent determinants of AB-amyloidosis. CONCLUSION: It would be prudent clinical practice to employ high-flux biocompatible membranes in conjunction with ultrapure dialysis fluid for the treatment of end-stage renal disease patients who need to remain on long-term haemodialysis.

Acrylic Resins↗
PubMed2005

Clinicopathological and epidemiological analysis of amyloidosis in Turkish patients.

BACKGROUND: The aim of the present study was to assess the correlation of immunohistochemical subtyping with clinical diagnosis in order to achieve useful epidemiological data regarding amyloidosis in Turkish patients. METHOD: We carried out immunohistochemical studies on 128 biopsies from various sites of 111 patients with biopsy-proven amyloidosis and, based on the results, classified the patients. We assessed the correlation of immunohistochemical subtype with clinical diagnosis and gathered epidemiological data. RESULTS: The sites most biopsied were kidney and rectum, followed by the testicle, liver, small intestine and bladder. Amyloid deposits showed positive staining with a single antibody in 120 biopsies. Pure amyloid A (AA) positivity was seen in 113 biopsies; six biopsies were positive for amyloid lambda (AL) and one for beta2-microglobulin (beta2MG). The clinical diagnoses of 81 patients (98 biopsies all AA positive) were suggestive of familial Mediterranean fever (FMF). Also AA positive were eight patients with tuberculosis, seven patients with rheumatoid arthritis, four patients with bronchiectasis and one patient with Crohn's disease. The biopsies from seven patients clinically suspected to have plasma cell dyscrasias were AL positive. One patient undergoing haemodialysis was beta2MG positive. Two patients without definite diagnoses showed double or triple positivity, which could not be interpreted and classified immunohistochemically. CONCLUSIONS: This study demonstrates that the predominant association of AA amyloidosis is with FMF. It also suggests that the routine immunohistochemical study of patients with amyloidosis who are of certain ethnic backgrounds suffices for classifying the subtype of amyloid fibril protein and the related disease.

Adolescent↗
PubMed2002

Unicentric Castleman's disease complicated by systemic AA amyloidosis: a curable disease.

BACKGROUND: Castleman's disease (angiofollicular lymph node hyperplasia) is a group of rare lymphoproliferative disorders sharing characteristic clinical and histological features, and usually accompanied by a marked systemic inflammatory response. All types may be complicated by acquired systemic amyloidosis, usually of AA type, but occasionally of AL type associated with monoclonal gammopathy. DESIGN: Descriptive study of five patients with unicentric Castleman's disease complicated by systemic AA amyloidosis. METHODS: A diagnosis of amyloidosis was confirmed by microscopy and immunohistochemical staining. Serum concentrations of C-reactive protein (CRP) and serum amyloid A protein (SAA) were measured by immunoassays. Radiolabelled serum amyloid P component scintigraphy was used to monitor the progress of amyloid deposition. RESULTS: In four patients the primary diagnosis was made only after years of investigation of systemic symptoms. The tumours were resected in all cases, leading to remission of the systemic inflammatory state. Long-term follow-up in four patients, including scintigraphy, showed regression of amyloid deposits. DISCUSSION: This rare but usually fatal condition can be cured surgically even in advanced cases. Awareness of the diagnosis and its correct management are important in investigation of patients with unexplained systemic symptoms, especially associated with systemic amyloidosis.

Adult↗
PubMed1996

Secondary amyloidosis in patients with rheumatoid arthritis: diagnostic and prognostic value of gastroduodenal biopsy.

Upper gastrointestinal endoscopy was performed in patients with rheumatoid arthritis (RA) during the period 1989-1991, and biopsy specimens were obtained from the stomach and from the duodenum for examining amyloid deposits. Among 407 patients, gastrointestinal amyloidosis was confirmed in 54 (13.3%). Twenty-two patients were regarded as having slight amyloid deposits, while 32 patients were categorized as having marked amyloid deposits. The incidence of clinical manifestations suggestive of systemic amyloidosis was more frequent in the marked deposits group than in the slight deposits group (47% vs 14%, P<0.05). Among the patients who died of manifestations associated with amyloidosis, the survival period following endoscopy was shorter in the marked deposits group than in the slight deposits group. These findings suggest that gastroduodenal biopsies may be useful for diagnosing secondary amyloidosis and that the degree of amyloid deposits seems to be correlated with the clinical manifestations of RA.

Amyloid↗
PubMed2004

Treatment of renal amyloidosis with etanercept in tumour necrosis factor receptor-associated periodic syndrome.

OBJECTIVE: To describe the effect of Etanercept treatment in systemic AA amyloidosis in tumour necrosis factor receptor-associated periodic syndrome (TRAPS). METHODS: Etanercept therapy was given to a 27 year old woman, with systemic amyloidosis and nephrotic syndrome, and to her 51 year old father, also affected by TRAPS, who had previously undergone renal transplant for amyloidosis. Serum SAA levels, plasma cytokines, glomerular filtration rate and serum amyloid P scanning were monitored. RESULTS: Etanercept treatment resulted in initial clinical resolution of nephrotic syndrome in the 27 year old female. Both subjects demonstrated improvements in GFR and initial reduction or stabilisation of amyloid deposits on SAP scanning. CONCLUSION: Etanercept may reverse or slow the progression of systemic AA amyloidosis in subjects with C33Y TNFRSF1A mutation. Treatment may however need to be continuous and life-long to prevent progression to end stage disease.

Adult↗
PubMed1998

Dermatopathologic findings in 20 cases of systemic amyloidosis.

The clinical findings of systemic amyloidosis are often variable and nonspecific. The early recognition of this disorder relies upon clinical suspicion, and definite diagnosis requires the confirmation of amyloid deposits in tissue. We reviewed 31 skin biopsy specimens from 20 patients, including 17 with amyloid light chain (AL) protein amyloidosis and 3 with serum amyloid A (AA) protein amyloidosis. Histologically, amyloid can be present in the papillary dermis, reticular dermis, subcutis, around the appendages, and in or around blood vessels. In our series, all 20 patients had at least one positive skin biopsy. Amyloid was found in 30 of 31 specimens (97%), a higher rate than in most series. Skin biopsy is a simple, safe procedure with high yield and may be used to obtain a firm diagnosis of systemic amyloidosis.

Aged↗
PubMed1994

Case report: acute renal vein thrombosis in patients with spinal cord injury and secondary amyloidosis.

Chronic spinal cord injury, when complicated by chronic suppurative infections, has replaced chronic tuberculosis as a leading cause of secondary amyloidosis. Renal involvement with secondary amyloidosis is characterized by the presence of nephrotic range proteinuria and an increased incidence of renal vein thrombosis. Two cases of acute renal vein thrombosis associated with secondary amyloidosis in patients with spinal cord injury are presented. In both cases, a past history of extensive decubitus ulcerations and urinary tract infections preceded the development of nephrotic range proteinuria. In case 1, nonoliguric acute renal failure occurred after the development of acute bilateral renal vein thrombosis. The patient declined dialytic therapy and expired with uremia. In case 2, worsening renal function and increased proteinuria resulted after the development of acute unilateral renal vein thrombosis. These cases include the clinical and anatomic findings of acute renal vein thrombosis that occur as a complication of secondary amyloidosis. Acute renal vein thrombosis should be considered whenever an acute change in renal function or increase in proteinuria is noted in this setting.

Aged↗
PubMed2002

Beta-2 microglobulin amyloidosis presenting as bilateral ovarian masses: a case report and review of the literature.

We report a case of systemic beta-2 microglobulin amyloidosis (B2M) in which the initial clinical presentation was that of bilateral ovarian masses. A 56-year-old woman who had been on renal dialysis for 12 years because of familial glomerulonephritis underwent a total hysterectomy and bilateral salpingo-oophorectomy for suspected ovarian malignancy. Pathologic findings included extensive amyloid infiltration of both ovaries, fallopian tubes, and focal perivascular deposition in the myometrium. The diagnosis of amyloidosis was confirmed with Congo red stain, B2M immunohistochemistry, and electron microscopy. Systemic amyloidosis in renal dialysis patients commonly presents as bone and/or joint disease, although visceral involvement has been reported. This is the first report in the English language literature to describe amyloidosis presenting as bilateral ovarian masses.

Amyloidosis↗
PubMed1993

Advances in amyloidosis.

Major advances of the past year in the amyloidoses include a better understanding of the polymorphism of the acute-phase reactant serum amyloid A protein and the appearance of a new mouse model for primary amyloidosis. The list of single point mutations in transthyretin in different families with slightly varied clinical manifestations of the disease continues to grow. Gelsolin, with its asparagine 187 mutation, was found to cause amyloidosis beyond the borders of Finland, where it has been extensively evaluated. The incredible range of osteoarticular lesions due to beta 2-microglobulin in hemodialysis amyloidosis continues to expand and includes severe manifestations of spondyloarthropathy. The greatest number of papers in the amyloid literature have involved amyloid beta protein, amyloid beta protein precursor associated with Alzheimer's disease, and prion protein associated with the spongiform encephalopathies. The widespread systemic involvement of amyloidosis has led to the appearance of a host of manifestations, some common and some rare. Treatment advances focus on the use of liver transplantation in familial amyloid polyneuropathy to remove the source of mutant protein synthesis.

Amyloidosis↗
PubMed1986

Renal amyloidosis. Evaluation by gallium imaging.

A study has been performed to evaluate the efficacy of gallium imaging in the detection of renal amyloidosis. Ten of the 11 patients who had biopsy-proven renal amyloidosis demonstrated marked uptake in both kidneys. One patient revealed moderate gallium uptake in his kidneys. None of the patients had underlying renal or extrarenal pathology other than amyloidosis, which could account for renal gallium uptake (renal infection, neoplasm, hepatic failure or frequent blood transfusions). Four patients also had extrarenal foci of abnormal gallium uptake, suggesting other sites of amyloid deposits. Our data strongly suggest that gallium imaging has a high sensitivity for detection of renal amyloidosis. Its specificity is enhanced significantly by careful review of the clinical history to exclude other known causes of renal gallium uptake. Potentially, gallium imaging may be used to monitor the progress of patients under experimental therapy.

Adult↗
PubMed2003

Amyloidosis and gastric bleeding in a patient with Gaucher disease.

GOALS: To describe the clinical course of a patient with Gaucher disease who subsequently developed amyloidosis. BACKGROUND: We present a case of a splenectomized patient with Gaucher disease who developed portal hypertension secondary to an enlarged, cirrhotic-like liver, and recurrent life-threatening upper gastrointestinal bleeding. STUDY: Despite repeated diagnostic biopsies, amyloidosis was only ascertained after death. RESULTS: Albeit very rare, there are four other similar cases in the literature, but unlike these previous reports of concurrence of Gaucher disease and amyloidosis, in this patient the gastrointestinal symptoms were life-threatening but there was no evidence of gammopathy or renal disease. Also, this is the first patient who was treated with enzyme replacement therapy for 5 years prior to manifestation of amyloidosis. CONCLUSIONS: Coexistence of apparently unrelated diseases with Gaucher disease demands a greater awareness of abnormalities at the biochemical and/or molecular level to adequately manage patients with Gaucher disease, regardless of concurrent enzyme replacement therapy.

Adult↗
PubMed1986

Jaw claudication in primary systemic amyloidosis.

Amyloid deposits in the temporal artery were observed 40 years ago, but the presence of vascular ischemic symptoms in patients with amyloidosis has been infrequently recognized. We examined 22 patients who had typical jaw claudication with biopsy-proven primary amyloidosis. In none was vasculitis a contributing cause of the claudication. However, two patients were misdiagnosed initially as having temporal arteritis and polymyalgia rheumatica and were treated with corticosteroids, which resulted in significant toxicity. Subsequent temporal artery biopsy revealed extensive amyloid deposits in both patients. Jaw claudication was associated with other ischemic vascular symptoms, such as arm or calf claudication. The median survival for the subset of patients with amyloidosis and jaw claudication was 42 months, and that for the entire group of patients with amyloidosis was 12 months. Appropriate staining of temporal artery biopsy specimens is necessary for the correct diagnosis in such cases.

Aged↗
PubMed1995

Pathogenesis of dialysis-related amyloidosis.

Beta 2-microglobulin has been demonstrated to be a major constituent of amyloid fibrils in dialysis-related amyloidosis. However, the molecular pathogenesis of this complication remains unknown. Several lines of evidence suggest that beta 2-microglobulin is not an innocent bystander, but plays an active role in the development of dialysis-related amyloidosis. The evidence remains inconclusive, however, as to whether it is intact or modified beta 2-microglobulin which is amyloidogenic and contributes to bone and joint destruction. Recent biochemical and immunohistological studies have revealed a new modification of beta 2-microglobulin in amyloid fibrils, the advanced glycation end products formed nonenzymatically between aldoses and proteins. Further study has suggested that the interaction of advanced glycation end product-modified beta 2-microglobulin with monocytes/macrophages gives a plausible, albeit incomplete, explanation for the mechanism of bone and joint destruction in dialysis-related amyloidosis. This review focuses on new aspects of the pathogenesis of dialysis-related amyloidosis.

Amyloid beta-Peptides↗
PubMed2002

Serum amyloid P component scintigraphy for diagnosis and monitoring amyloidosis.

Serum amyloid P component is a normal plasma protein and a universal non-fibrillar constituent of amyloid deposits. Radiolabelled serum amyloid P component scintigraphy is a non-invasive and quantitative method for imaging amyloid deposits, which produces diagnostic images in most patients with systemic amyloidosis, and can be used repeatedly to monitor the course of the disease. The scintigraphy technique and biopsy histology are complementary, providing a detailed microscopic analysis and a quantitative whole body survey respectively. Clinically useful observations provided by the imaging method include different organ distributions of amyloid in different types of the disease, demonstration of amyloid in anatomic sites not available for biopsy, and evidence for rapid progression and sometimes regression of amyloid deposits with different rates in different organs. Labelled serum amyloid P component studies thus make a unique contribution to the diagnosis and management of individual patients with systemic amyloidosis, and to systematic studies of existing and novel therapies. The technique is available routinely for all known or suspected cases of amyloidosis in the NHS National Amyloidosis Centre at the Royal Free Hospital, but it has not been developed commercially.

Amyloidosis↗
PubMedDate not supplied

Surgical management of recurrent localized eyelid amyloidosis.

Amyloidosis is a disease of unknown origin characterized by the widespread or localized deposition of amorphous hyaline material, amyloid, in various body tissues, including the ocular and orbital structures. Primary advanced localized amyloidosis involving the eyelid and conjunctiva is a rare cause of diffuse eyelid thickening. Management of these cases is often unsatisfactory because of the diffuse deposition of amyloid tissue and the increased risk of intraoperative hemorrhage, due to the fragile vessels encountered in amyloidosis. The possibility of recurrence also presents challenging treatment dilemmas. Therapeutic options include en bloc removal of the involved eyelid tissue, radiation therapy or, in advanced cases, no treatment. We describe an effective surgical approach that offers a safe, effective, and, most notably, repeatable procedure for the treatment of advanced ocular adnexal amyloidosis.

Aged↗