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PubMed1981

Morphologic and clinical correlates in renal amyloidosis.

Morphologic studies and clinical correlations were undertaken in 59 patients with renal amyloidosis. Spicularly arranged amyloid deposits in the glomerular capillary wall were found in all clinical groups but were more frequent and more extensive in primary amyloidosis and multiple myeloma. The severity of proteinuria correlated with the presence of spicules and podocyte destruction rather than with the amount of amyloid in the glomerulus. The spicules were associated with morphologic and clinical evidence of rapid amyloid deposition and a fulminant clinical course. The absence of spicules and the presence of extensive new basement membrane material may produce basement membrane thickening, lamination, and double capillary wall contours, which are associated with mild proteinuria and, rarely, resolution of amyloidosis. Nodular or mixed nodular-diffuse patterns of glomerular amyloid deposits were more frequent in patients with secondary amyloidosis and a longer clinical course. Renal failure generally corresponded to severe glomerular amyloidosis and tubular atrophy. However, a relatively precipitous, usually irreversible decrease in renal function frequently occurred in patients with renal amyloidosis and did not always have a morphologic explanation. The duration of life from the time of biopsy no death in patients with primary amyloidosis (nine months) was markedly shorter than in those with secondary amyloidosis (more than 50 months).

Amyloid↗
PubMed1986

Abdominal fat tissue aspirate in human amyloidosis: light, electron, and immunofluorescence microscopic studies.

Abdominal fat tissue aspirates from 12 patients with biopsy-proved amyloidosis were investigated by different morphologic techniques. By light microscopy, after staining of the fat tissue aspirates with Congo red and examination with a polarizing microscope, positive results were obtained in nine patients with amyloidosis, two of the three with primary (AL) amyloidosis and seven of the nine with secondary (AA) amyloidosis. By indirect immunofluorescence, using AA antiserum, positive results were obtained in five of the nine cases of AA amyloidosis (aspirates from these five patients were positive on Congo red staining). By electron microscopy, amyloid fibrils were observed in five cases of amyloidosis (two of the AL and three of the AA type, all positive on Congo red staining). Although amyloid was demonstrated less frequently by immunofluorescence and electron microscopy, perhaps because of the small numbers of fat particles examined, it seems that, with Congo red staining, abdominal fat tissue aspiration is a simple and sensitive method for the diagnosis of amyloidosis. Immunofluorescence studies allow discrimination between the different types of amyloidosis. The method could be used in patients in whom other types of tissue biopsy are not recommended because of risks of bleeding or other problems.

Adipose Tissue↗
PubMed1993

Colchicine in the treatment of AA and AL amyloidosis.

Colchicine is an effective medication in the prevention and treatment of amyloidosis of familial Mediterranean fever. Its therapeutic effect depends on the stage of renal disease and the drug dose. To evaluate colchicine effect in AA amyloidosis of other diseases and in primary AL amyloidosis, the literature was reviewed. Findings were that (1) the effect of colchicine in reactive amyloidosis has not been methodically studied, but anecdotal reports suggest it may be beneficial; and (2) the results of studies and case reports on the effect of colchicine in primary amyloidosis are conflicting. Because a therapeutic effect of colchicine in primary and reactive amyloidosis has been shown in sporadic cases, a prospective, controlled, multicenter study assessing the effect of colchicine in all types of amyloidosis appears to be justified. Until such a study is available, the addition of colchicine in an appropriate dose to any therapeutic regimen of patients with AA or AL amyloidosis should be considered.

Adult↗
PubMed1997

Primary solitary amyloidosis of the spine: a case report and review of the literature.

BACKGROUND: Amyloidosis is a term that encompasses a group of disorders that have as their common feature the intercellular deposition of the amyloid protein by several different pathogenetic mechanisms. Primary solitary amyloidosis, or amyloidoma, is a rare subset of amyloidosis in which the amyloid deposition is focal and not secondary to a systemic process or plasma cell dyscrasia. We present the second reported case of a cervical spine amyloidoma and discuss its presentation and management. METHODS: This 58-year-old man presented with a 1-year history of intermittent chest pain that would radiate into both legs precipitated by valsalva maneuvers. A magnetic resonance imaging (MRI) of the cervical spine revealed a homogenously enhancing lesion focally involving the C-7 vertebral level with significant spinal cord compression. He underwent combined anterior and posterior decompressive procedures with instrumentation for spinal stabilization. Histopathology revealed amyloid deposits and a systemic work-up was negative for amyloidosis. RESULTS: The patients is free of any tumor recurrence at 24 months and has a stable spine construct. CONCLUSIONS: Primary solitary amyloidosis is a rare subtype of amyloidosis which, unlike other forms of amyloidosis, has an excellent prognosis with local resection. Diagnosis requires special stains and therefore a degree of suspicion for the disease. Management of vertebral amyloidoma involves aggressive local resection of the tumor when feasible and stabilization of the spine as mandated by the degree of tumor involvement. Complete evaluation for the diagnosis of systemic amyloidosis is essential for the management and prognostication of each case.

Amyloidosis↗
PubMed2002

Value of testis biopsy in the diagnosis of systemic amyloidosis.

OBJECTIVES: To evaluate testis biopsy as a diagnostic tool in systemic amyloidosis, to assess how amyloidosis affects testicular tissue, and to examine the relationship between testicular amyloidosis and infertility. METHODS: Testicular biopsies from 72 patients with confirmed systemic amyloidosis were examined for amyloid deposition after Congo red and crystal violet staining. A rectal biopsy was also done in each case, and the results were compared with the testicular biopsy findings. RESULTS: Testicular amyloid deposition was detected in 62 (86.1%) of 72 patients. Fifty-one (85%) of 60 patients with secondary amyloidosis, 11 (91.7%) of 12 patients with primary amyloidosis, and 28 (87.5%) of 32 patients with familial Mediterranean fever showed amyloid deposition in the testis. Rectal biopsies were positive in 40 cases (55.6%). Only 4 of the 62 patients with testicular amyloid showed normal spermatogenesis. The remaining 58 exhibited abnormal spermatogenesis, and 77.7% of patients had seconder infertility. Of 62 patients with positive testis biopsies, 30 had serum creatinine levels less than 1.5 mg/dL, and 29 patients with testicular amyloid manifested nephrotic syndrome. CONCLUSIONS: The testis biopsy is a valuable and more sensitive method than rectal biopsy for diagnosing systemic amyloidosis. The results also showed that testicular amyloidosis causes infertility at a higher rate than expected.

Adult↗
PubMed1988

Amyloid and amyloidosis.

Cutaneous lesions are present in up to 40% of patients with primary and myeloma-associated systemic amyloidosis and occur as a result of tissue deposition of immunoglobulin light chain material derived from a circulating paraprotein. The occurrence of waxy, purpuric mucocutaneous lesions provides a crucial early pointer to underlying occult plasma cell dyscrasia; the combination of the symptoms of the carpal tunnel syndrome, macroglossia, and specific mucocutaneous lesions is highly characteristic. Although secondary systemic (reactive) amyloidosis rarely gives rise to clinically evident cutaneous lesions, it may be etiologically related to a number of chronic dermatoses. Lesions of nodular primary localized cutaneous amyloidosis are indistinguishable from those of primary and myeloma-associated systemic amyloidosis, and they result from local plasma cell infiltration. Macular and papular (lichen amyloidosus) variants of primary localized cutaneous amyloidosis may have a familial or racial basis and are characterized by a tendency for keratinocytes to undergo filamentous degeneration and apoptosis. The prognosis of patients with plasma cell dyscrasia-related systemic amyloidosis remains poor, since there is little response to therapy with cytotoxic agents, colchicine, or dimethylsulfoxide. Colchicine is the drug of choice in the prevention and treatment of the renal amyloidosis associated with familial Mediterranean fever, and dimethylsulfoxide may be useful in the management of patients with secondary systemic amyloidosis. Macular amyloid and lichen amyloidosus generally follow a chronic course with intractable pruritus; there have been isolated reports of the beneficial effect of dermabrasion, topical dimethylsulfoxide, and therapy with the aromatic retinoid, etretinate.

Amyloid↗
PubMed2000

[Clinical or biological symptoms leading to the search for amyloidosis].

INTRODUCTION: The extracellular, multifocal, disseminated or diffuse localization of amyloidosis accounts for the diversity of clinical presentations and late diagnosis. CURRENT KNOWLEDGE AND KEY POINTS: However, early diagnosis is important, as new drugs have been recently introduced. Both clinical picture and biology help guide diagnosis, including either kidney, heart, skin or neurologic involvement associated with monoclonal gammapathy (primary idiopathic amyloidosis AL); underlying inflammatory or infectious disease, familial Mediterranean fever with proteinuria (secondary amyloidosis AA); cardiac, neurologic or ocular involvement (heredofamilial amyloidosis); carpal tunnel syndrome, joint pain (amyloidosis of hemodialysis). Furthermore, amyloid fibrils are identified on salivary gland biopsy. FUTURE PROSPECTS AND PROJECTS: Due to the introduction of new specific drugs aimed at curing various amyloidoses, early diagnosis is important. Chemotherapy and hematopoietic stem cell transplantation are promising regarding AL amyloidosis, while liver transplantation has proven remarkably successful in heredofamilial amyloidosis. Progress in molecular biology should allow identification of various forms of familial amyloidosis.

Amyloidosis↗
PubMed1994

[Localized or systemic amyloidosis? Value and limitations of Iodine-123 labelled amyloid P component scintigraphy, role of biopsy of minor salivary glands].

We evaluated the value of serum amyloid P component scintigraphy (123I-SAP) and labial salivary gland biopsy in four patients with apparently localized amyloidosis. One patient had pharyngeal amyloidosis, the second laryngeal amyloidosis, the third retro-ocular amyloidosis and the fourth had carpal tunnel syndrome. Three patients (no 2, no 3, no 4) have abnormal whole body retention of 123I-SAP and positive labial salivary gland biopsy for amyloidosis. Only the first patient had effectively localized amyloidosis and local treatment was sufficient. Melphalan was given to the patients no 2 and no 3, colchicine was given to the patient no 4. Labial salivary gland biopsy and 123I-SAP scintigraphy may give positive argument for the diagnosis of systemic amyloidosis in patients with apparently localized amyloidosis.

Adult↗
PubMed2000

Physical properties of the mitral valve tissue assessed by tissue sound speed in cardiac amyloidosis: relationship to the severity of mitral regurgitation.

Cardiac amyloidosis has been documented to show mitral regurgitation (MR) and a thickened mitral valve (MV) due to amyloid deposits. However, the changes in the physical properties of the thickened MV tissue in cardiac amyloidosis, which may be a causative factor of the MR, have not been described. Physical properties of the tissue, which are expressed by the elastic bulk modulus, can be evaluated by tissue sound speed. If biological tissue is assumed to be fluid-like, the tissue sound speed may be given by c= square root of K/rho, where c is the tissue sound speed, K is the elastic bulk modulus, and rho is the density. A reduction in tissue sound speed indicates a reduction in the elastic bulk modulus of the tissue, assuming that there is little change in rho. This suggests that the tissue is less elastic. The purpose of this study was to assess the physical properties of MV tissue by evaluating the sound speed of the MV tissue in cardiac amyloidosis. MV specimens were obtained at autopsy from 20 control adults without cardiovascular diseases and from 20 patients with cardiac amyloidosis. An acoustic microscope operating at 450 MHz was used to measure the tissue sound speed in the tip and basal portions of the MV tissue. The density of MV tissue was measured by microgravimetry. The severity of the MR had been evaluated by Doppler echocardiography before death, and it was compared with the tissue sound speed measured after death. In cardiac amyloidosis showing mild MR, the tissue sound speed of the MV in the tip portion (1605 +/- 19 m/s) and in the basal portion (1791 +/- 64 m/s) were lower than the corresponding values in control subjects (1637 +/- 42 m/s and 1851 +/- 62 m/s). However, these differences were not statistically significant. In cardiac amyloidosis showing moderate MR, the tissue sound speed of MV in the tip portion (1563 +/- 17 m/s) and in the basal portion (1654 +/- 59 m/s) were significantly lower than the corresponding values in the control subjects (p < 0.001) and the patients with mild MR (p < 0.05). No significant differences were observed in the density of MV tissue among the three groups. Therefore, the low value of the MV tissue sound speed in patients with cardiac amyloidosis indicated a reduced elastic bulk modulus, suggesting the less elasticity of the MV tissue. Furthermore, the patients with moderate MR demonstrated the greater reduction in the tissue sound speed than the patients with mild MR. The data suggest that the changes in physical properties of the MV tissue may be one of the causes of MR in cardiac amyloidosis.

Acoustics↗
PubMed2001

Transmission of mouse senile amyloidosis.

SUMMARY: In mouse senile amyloidosis, apolipoprotein A-II polymerizes into amyloid fibrils (AApoAII) and deposits systemically. Peripheral injection of AApoAII fibrils into young mice induces systemic amyloidosis (Higuchi et al, 1998). We isolated AApoAII amyloid fibrils from the livers of old R1.P1-Apoa2(c) mice and injected them with feeding needles into the stomachs of young R1.P1-Apoa2(c) mice for 5 consecutive days. After 2 months, all mice had AApoAII deposits in the lamina propria of the small intestine. Amyloid deposition extended to the tongue, stomach, heart, and liver at 3 and 4 months after feeding. AApoAII suspended in drinking water also induced amyloidosis. Amyloid deposition was induced in young mice reared in the same cage for 3 months with old mice who had severe amyloidosis. Detection of AApoAII in feces of old mice and induction of amyloidosis by the injection of an amyloid fraction of feces suggested the propagation of amyloidosis by eating feces. Here, we substantiate the transmissibility of AApoAII amyloidosis and present a possible pathogenesis of amyloidosis, ie, oral transmission of amyloid fibril conformation, where we assert that exogenous amyloid fibrils act as templates and change the conformation of endogenous amyloid protein to polymerize into amyloid fibrils.

Amyloid↗
PubMed2000

Curative hepatorenal transplantation in systemic amyloidosis caused by the Glu526Val fibrinogen alpha-chain variant in an English family.

A 53-year-old English woman who had been thought to have systemic monoclonal immunoglobulin light chain (AL) amyloidosis was investigated further because of her unusually long 17-year history and a suggestion of renal disease in the family. She was found to have the Glu526Val fibrinogen alpha-chain variant that causes autosomal dominant hereditary systemic amyloidosis. This has not previously been described in a British family. The mutant gene was associated with the same haplotype as in all other reported cases, suggesting a common founder. The patient had already received a renal transplant, but the graft failed within 6 years due to amyloid deposition. Progressive hepatic amyloidosis eventually caused liver failure, although the function of other organs was well preserved. She therefore received hepatic and renal transplants to replace the failed organs and the hepatic source of the amyloidogenic variant fibrinogen. Three years later she is completely well and has no amyloid deposits identifiable by serum amyloid P component scintigraphy. This is the first detailed report of hepatic transplantation for liver failure caused by amyloidosis of any type. The substantial follow-up suggests that fibrinogen alpha-chain amyloidosis is one of the inherited metabolic diseases that can be cured by liver transplantation. The mutation underlying Glu526Val fibrinogen alpha-chain amyloidosis is incompletely penetrant and has a variable phenotype that can clinically mimic AL amyloidosis. Hereditary fibrinogen amyloidosis may be more prevalent than previously suspected and, since AL amyloid is sometimes a diagnosis of exclusion, genotyping for other amyloidogenic proteins is mandatory in all cases in which the amyloid fibrils cannot be positively identified as AL.

Amyloidosis↗
PubMed1999

Amyloidosis in a nationwide series of 1666 subjects with rheumatoid arthritis who died during 1989 in Finland.

OBJECTIVES: Virtually all studies dealing with the occurrence of amyloidosis in subjects with rheumatoid arthritis (RA) have been based on selected series collected from university clinics. The purpose of the study was to obtain information on the true prevalence of amyloidosis and the role of amyloidosis as a cause of death. METHODS: The study included all 1666 subjects (480 men and 1186 women) who had died in 1989 and had been entitled under the national sickness insurance scheme to receive specially reimbursed medication for RA. RESULTS: Amyloidosis was regarded as an immediate cause or an intervening antecedent cause of death in 64 cases (3.8%) and as a contributory cause of death in 33 cases (2%), corresponding to a prevalence of 5.8%. Amyloidosis had been diagnosed during life in 89 instances and was detected at autopsy in eight instances. Twenty-three (4.8%) of the subjects were men and 74 (6.2%) were women (P = 0.25). Compared with the remaining subjects in the study series, the lifespan of the subjects with amyloidosis was shortened by 7.7 yr. CONCLUSIONS: The prevalence of amyloidosis was lower than apparent from most earlier studies. Monitoring information derived from the Finnish sickness insurance system is a useful way of following trends in the occurrence of amyloidosis complicating RA.

Adult↗
PubMed2003

Amyloidosis modifier genes in the less amyloidogenic a/j mouse strain.

Apolipoprotein A-II is deposited as an amyloid fibril in aged mice (senile AApoAII amyloidosis). Although mouse strains with the apolipoprotein A-II c allele (Apoa2(c)) generally develop early-onset and severe senile amyloidosis, the A/J strain shows significantly less amyloid deposition. To identify genes that modify spontaneous amyloidosis development in the A/J mouse, we performed a genome-wide screening using hybrid mice derived from A/J and SAMP1 mice, which have Apoa2(c) and age-associated severe amyloid deposition. Our genetic analysis revealed that the lower levels of amyloidosis in the A/J strain were polygenically controlled. We found two chromosome locations associated with amyloidosis. One of these regions was in the chromosome 19 telomeric region, where the A/J alleles modify amyloidosis in an additive manner. The second region was in the chromosome 4 telomeric region, where the A/J alleles modify amyloidosis in a dominant manner. Perlecan and group II secretory phospholipase A2, located on the significantly linked region of chromosome 4, were compared in this study. These findings are for understanding the genetic mechanism of amyloidosis-related diseases and their prevention.

Amyloid↗
PubMed2003

A novel tool for detecting amyloid deposits in systemic amyloidosis in vitro and in vivo.

We synthesized (trans,trans)-1-bromo-2,5-bis-(3-hydroxycarbonyl-4-hydroxy)styrylbenzene (BSB) and used this compound to detect amyloid fibrils in autopsy and biopsy samples from patients with localized amyloidosis, such as familial prion disease, and systemic amyloidosis, such as familial amyloidotic polyneuropathy, amyloid A (AA) amyloidosis, light chain (AL) amyloidosis, and dialysis-related amyloidosis. BSB showed reactions in all Congo red-positive and immunoreactive regions of the samples examined in the study, and some amyloid fibrils in the tissues could be detected more precisely with BSB than with the other methods. In the mouse model of AA amyloidosis, injected BSB reacted with amyloid in all regions in the serial sections in which Congo red staining was positive. A highly sensitive 27-MHz quartz crystal microbalance analysis revealed that BSB showed a significant affinity for amyloid fibrils purified from familial amyloidotic polyneuropathy and dialysis-related amyloidosis samples and suppressed formation of transthyretin amyloid in vitro. These results suggest that BSB may become a valuable tool for detection of amyloid deposits in amyloidosis and of the mechanism of amyloid formation.

Adult↗
PubMed2004

Renal transplantation in amyloidosis: effects of HLA matching and donor type on recurrence of primary disease.

The aim of this study is to evaluate the effect of HLA-matching and donor type on recurrence of amyloidosis after renal transplantation. The study includes 30 patients with systemic amyloidosis who received kidney transplants between 1985 and 2001. Donor source and HLA tissue typing of the donor and recipient were evaluated in each case. Of the 30 patients, 20 developed a recurrence of amyloidosis in their allografts, as confirmed by biopsy. The time from transplantation to diagnosis of amyloidosis in the graft ranged from 18 months to 10 years. Of the 20 patients with recurrence, 18 had received their grafts from living related donors (LRDs), and 2 had received their grafts from cadaveric donors (P < 0.01). There was a strong correlation between amyloidosis recurrence and degree of HLA-DR matching (P < 0.05). Furthermore, in the recipients of LRD grafts, the risk of amyloidosis recurrence was much higher if the donor-recipient pair were HLA-identical than if they were not perfectly matched (P < 0.01). The incidence of amyloidosis recurrence in our patients was significantly higher than the rates reported for other series. Most of the cases in previous reports involved cadaveric grafts. The higher rate of amyloidosis recurrence in our patients may be explained by the high proportion of LRD grafts and by genetic susceptibility.

Adult↗
PubMed1998

The liver in systemic amyloidosis: insights from 123I serum amyloid P component scintigraphy in 484 patients.

BACKGROUND AND AIMS: The liver is frequently involved in amyloidosis but the significance of hepatic amyloid has not been systematically studied. We have previously developed scintigraphy with 123I serum amyloid P component (123I-SAP) to identify and monitor amyloid deposits quantitatively in vivo and we report here our findings in hepatic amyloidosis. METHODS: Between 1988 and 1995, 805 patients with clinically suspected or biopsy proven systemic amyloidosis were evaluated. One hundred and thirty eight patients had AA amyloidosis, 180 had AL amyloidosis, 99 had hereditary amyloid syndromes, and 67 had dialysis related (beta 2 microglobulin) amyloid. One hundred and ninety two patients with amyloidosis were followed for six months to eight years. RESULTS: Hepatic amyloid was found in 98/180 (54%) AL and 25/138 (18%) AA patients but in only 1/53 patients with familial transthyretin amyloid polyneuropathy and in none with dialysis related amyloidosis. There was complete concordance between hepatic SAP scintigraphy and the presence or absence of parenchymal amyloid deposits on liver histology. Amyloidosis was never confined to the liver. Mortality was rarely due to hepatic failure, although hepatic involvement with AA amyloid carried a poor prognosis. Successful therapy to reduce the supply of amyloid fibril protein precursors was followed by substantial regression of all types of amyloid. CONCLUSIONS: SAP scintigraphy is a specific and sensitive method for detecting and monitoring hepatic amyloid. Liver involvement is always associated with major amyloid in other organ systems and carries a poor prognosis in AA type. Appropriate therapy may substantially improve prognosis in many patients.

Adolescent↗
PubMed1981

Pattern of renal amyloidosis in Indian patients.

Two hundred and thirty-three patients with renal amyloidosis were studied in an attempt to identify the incidence and pattern of the disease in northern India. The incidence of amyloidosis was 1.01% of 6431 post-mortems and 8.4% of 1980 renal biopsies from patients who presented with clinical evidence of glomerular disease. Two hundred and three patients (87.1%) had secondary amyloidosis, 22 (9.4%) had primary amyloid and 8 patients (3.5%) had amyloidosis associated with multiple myeloma. Tuberculosis of various organs was the commonest predisposing disease accounting for 59.1% of secondary amyloidosis, followed by chronic suppurative lung disease in 24.1%. Rheumatoid arthritis, chronic osteomyelitis and lepromatous leprosy were seen in a small percentage of patients (2 to 8%). Proteinuria of varying degree was present in all the 233 patients and 12.9% of them had a daily protein excretion of more than 10 g. Post-mortem examination of 65 patients with renal amyloidosis showed that 75.3% also had amyloid deposit in the spleen, 63% in the liver, and 50.8% in the adrenals. Clinical evidence of disappearance of proteinuria was observed in 3 patients with secondary amyloidosis; in 2 of them, the regression of amyloidosis was confirmed by serial renal biopsy performed 3 and 5 years after the initial diagnosis.

Adolescent↗
PubMed2001

Pulmonary hypertension in patients with amyloidosis.

Pulmonary hypertension (PH) with right-sided cardiac failure is a rare complication of amyloidosis, and its natural history is not well-defined. The aim of our study was to evaluate patients who were seen at our institution who had PH and amyloidosis and to describe the natural history of this complication. The study was a retrospective chart review of patients seen at the Mayo Clinic with both PH and amyloidosis listed as major diagnoses between January 1, 1980, and December 31, 1999. Patients with known causes of PH were excluded. Five patients met our criteria (four women and one man). Four patients had light-chain amyloidosis and one had amyloid A deposition secondary to familial Mediterranean fever. All patients had symptoms related to PH without echocardiographic evidence of left ventricular dysfunction. The median survival time after the diagnosis of amyloidosis was 2.8 years, and PH was found a median of 73 days before death. Five patients died of cardiac complications, including one with sudden cardiac death. PH is an unusual complication of amyloidosis. Patients develop PH late in the disease process and do not have a worse prognosis compared to other patients with cardiac amyloidosis. PH is a marker of advanced amyloidosis.

Aged↗