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At least 973 records · Page 54Linked to original sources
PubMed1999

Chlorambucil in severe juvenile chronic arthritis: longterm followup with special reference to amyloidosis.

OBJECTIVE: To assess the effectiveness and side effects of chlorambucil therapy and patient outcome in juvenile chronic arthritis (JCA) treated with chlorambucil. METHODS: Followup of 79 consecutive patients with JCA refractory to previous therapy, in whom chlorambucil treatment was initiated from 1982 to 1995. Mean treatment duration was 7 months in 72 patients and more than 20 months in 6 patients with amyloidosis and 1 with severe iridocyclitis. RESULTS: Within 6 months, remission was attained in 41 patients (52%): 29 of these relapsed after a mean period of 2.5 years (range 0.3-8.3). Twenty-one (27%) patients did not respond. Seven out of 11 patients with secondary amyloidosis had proteinuria, which cleared completely in 4 and almost completely in 1. In 16 patients (20%) chlorambucil was stopped because of side effects, and in 1 because of infection. After a mean followup of 8.5 years, 14 patients (18%) were in complete remission without drugs, 34 (43%) had minor symptoms only, but 26 (33%) had limitations in daily activities. Five (6%) had died (2 of leukemia; 2 of infection, 0.5 and 3.3 years after withdrawal of chlorambucil; 1 of amyloidosis). CONCLUSION: Chlorambucil was found to be a very potent drug for JCA with acceptable short term, but serious longterm side effects. It may still be useful in JCA complicated by amyloidosis.

Adolescent↗
PubMed1999

[Serum amyloid A (SAA) 1, SAA 2 and apolipoprotein E isotype frequencies in rheumatoid arthritis patients with AA amyloidosis].

OBJECTIVES: To examine the relationship between polymorphism of serum amyloid A (SAA) 1, SAA 2 and Apolipoprotein E (Apo E) and susceptibility to AA amyloidosis (AA) in rheumatoid arthritis (RA). METHODS: We compared the frequencies of SAA 1 alleles (alpha, beta, gamma), SAA 2 alleles (alpha, beta) and apo E alleles (epsilon 2, epsilon 3, epsilon 4) in AA-positive RA with those in AA-negative RA. Each isotype was analyzed by the following method: SAA 1 and SAA 2 by PCR-RFLP and Apo E by Western blotting method. Blood samples were obtained from 50 AA-positive RA patients with SAA 1 isotype, 50 AA-negative RA patients with SAA 1 isotype, 27 AA-positive RA patients with SAA 2 isotype, and 26 AA-negative RA patients with SAA 2 isotype, respectively. Likewise, Apo E isotype was determined by withdrawing blood samples from 61 AA-positive RA cases and 51 AA-negative RA cases. RESULTS: In AA-positive RA, each frequency of three different alleles of SAA 1, i.e., alpha, beta and gamma was 15%, 32% and 53%, while it was 32%, 28% and 40% in AA-negative RA. The allelic distribution between AA-positive RA group and AA-negative RA group was significantly different (P = 0.00163) with a lower frequency of alpha allele and a higher gamma allele frequency observed in AA-positive RA group. The frequency of each SAA 2 alleles (alpha & beta) was almost identical: 88.9% and 11.1% in AA-positive RA versus 90.4% and 9.6% in AA-negative RA with p value of 0.8007. Each frequency of three different Apo E alleles (epsilon 2, epsilon 3 & epsilon 4) was 4.9%, 85.2% and 9.8% in AA-positive RA, while in AA-negative RA it was 7.8%, 86.3% and 5.9%, respectively. The AA-positive RA group showed a slightly higher prevalence of epsilon 4 allele than the AA-negative RA group, yet the difference did not reach statistical significance (P = 0.3969). CONCLUSIONS: These results suggest the possibilities that SAA 1 alpha may be working protectively against and SAA 1 gamma provocatively for the development of AA amyloidosis in RA. However, there was no significant association between SAA 2 isotype patterns and the development of AA amyloidosis in RA. Furthermore, there was no discernible association between AA amyloidosis in RA and Apo E 4 isotype.

Amyloidosis↗
PubMed1999

[A complete atrioventricular block and polymorphic ventricular tachycardia as the initial manifestation of secondary amyloidosis].

Cardiac injury is a frequent characteristic in primary, senile and myeloma related amyloidosis, but it is unusual in secondary amyloidosis. We report a patient with complete atrioventricular block and polymorphic ventricular tachycardia as the initial manifestation of secondary amyloidosis. Necropsy demonstrated amyloidosis deposits in the specific conduction system.

Adult↗
PubMed2000

Primary localized amyloidosis of the ureter and bladder managed by ileal interposition.

Amyloidosis of the ureter is a rare condition. It is even rarer when it involves both the ureter and bladder. The case presented is the second known case of combined amyloidosis of the bladder and ureter and the first combined case to be treated successfully by ileal ureter replacement. Historically, amyloidosis of the ureter has been treated by nephroureterectomy. Based on the benign nature of the disease, amyloidosis of the ureter is optimally treated with a kidney-sparing procedure such as ileal ureter replacement.

Aged↗
PubMed2000

Secondary amyloidosis: a severe complication of ankylosing spondylitis. Two case-reports.

STUDY OBJECTIVE: To report two cases of amyloidosis secondary to ankylosing spondylitis. PATIENTS AND RESULTS: Of the 47 ankylosing spondylitis patients who have received follow-up at our department over the last few years, two have developed AA amyloidosis. Both have extremely severe, long-standing joint disease, with virtually complete spinal ankylosis and destructive peripheral arthritis of the hips and wrists; one also has tarsal joint destruction. Renal dysfunction was the first manifestation of amyloidosis in both cases. One patient required chronic hemodialysis and developed peritonitis due to colonic perforation, probably at a site of amyloid deposition. CONCLUSIONS: Secondary amyloidosis is a rare complication of ankylosing spondylitis that can cause severe renal and gastrointestinal complications. No treatment capable of clearing established amyloid deposits is available to date.

Adult↗
PubMed1975

Experimental murine amyloidosis: a model system for studying amyloid formation.

Myeloma-associated and casein-induced murine amyloidosis were used as models to study the role of lymphocytes and macrophages in amyloid formation. Amyloidosis occurred rarely and in small amounts in Balb/C mice with immunoglobulin (Ig)-producing myeloma tumours but large amounts could be induced by injections of casein. Fluorescent staining of both forms of amyloid deposits by means of anti-casein- and anti-myeloma-amyloid antibodies indicated that they either crossreacted or coexisted. Nor abnormality of Ig biosynthesis was detected in amyloidosis, suggesting that abnormal degradation was responsible for production of the Ig form of amyloid. Although spleen lymphocytes of casein-injected mice with amyloidosis demonstrated diminished cellular immunologic responses, this did not indicate generalized immunologic incompetence. The non-Ig form of amyloid in casein-injected mice was shown to be produced by macrophages, and a technique was developed for increasing the yield of amyloid-containing cells.

Amyloid↗
PubMed2000

Diagnosis and treatment of AL amyloidosis.

AL (amyloid light-chain) amyloidosis is a plasma cell disorder in which depositions of amyloid light-chain protein cause progressive organ failure. Virtually all patients with AL amyloidosis have a monoclonal protein in the serum or urine or a monoclonal population of plasma cells in the bone marrow. The most common target organ is the kidney and renal amyloidosis manifests as proteinuria or nephrotic syndrome in 3/4 of the patients. The median survival is one to two years. It is important to recognize that the amyloidosis is a dynamic process, and chemotherapy induced reduction of the activity of the plasma cell clone reduces the supply of the amyloid precursor protein and can result in a major regression of the deposits. Amyloid-related nephrotic syndrome and renal failure are potentially reversible. Conventional-dose melphalan as standard treatment can prolong the median duration of survival about 10 months, but the clinical response rates with improvement of impaired organ function are low with a slow response. Upfront high-dose chemotherapy with autologous peripheral blood stem cell transplantation is much more effective and can result in a major improvement of the patient's clinical condition, but the treatment-related toxicity can be relevant due to impaired organ function. The initial use of a conventional-dose chemotherapy consisting of vincristine, doxorubicin and dexamethasone (VAD) to achieve a remission and subsequent high-dose chemotherapy is the concept of a German trial. The improvement of the condition of the patient by this approach may increase the tolerability of high-dose chemotherapy and reduce transplantation-related problems.

Amyloid↗
PubMed2000

Long-term survival in primary amyloidosis of the laryngotracheobronchial tract by treating complications only.

Primary amyloidosis limited to the laryngotracheobronchial tract is relatively uncommon; therefore, knowledge of survival in these patients is poor and consensus regarding appropriate therapy unreached. The case is reported of a patient affected by primary amyloidosis limited to the laryngotracheobronchial tract who had a long survival and a good quality of life after only medical and neodymium-yttrium aluminium garnet (Nd-YAG) laser therapy. The patient had amyloidosis first detected in the larynx, which, 4 yrs later, progressed to involve the tracheobronchial tract, without pulmonary involvement. The patient was treated three times with Nd-YAG laser to obtain the recanalization of obstructed bronchi, with evident improvement of symptoms and pulmonary function. In conclusion, laryngotracheobronchial amyloidosis survival may be lengthened and quality of life made more acceptable by treating potential complications only.

Amyloidosis↗
PubMed1975

Amyloidosis in rheumatoid arthritis.

The clinical and pathological features of amyloidosis are reviewed with special references to amyloidosis associated with rheumatoid arthritis. Data concerning the nature of the amyloid substance are presented. The amyloid fibril which is the unique and principal component of all amyloid substances, can be constituted by different protein subunits. The amyloid fibril protein AA is a major subunit of the amyloid fibrils particularly seen in secondary amyloidosis including that associated with rheumatoid arthritis. The immunological and chemical properties of protein AA and a structurally related serum protein (SAA) are discussed. Increased knowledge of the nature of amyloid and its precursor proteins may provide better methods for the diagnosis and treatment of amyloidosis.

Amino Acid Sequence↗
PubMed2000

[Current treatment of AL amyloidosis].

AL amyloidosis is a plasma cell disorder characterized by the tissue accumulation of immunoglobulin light chains. The prognosis of AL amyloidosis is poor, with a median survival inferior to two years. Amyloid deposits, responsible for the clinical manifestations of this disease, can regress at least partially after the suppression of the production of amyloid precursors. Therefore, current treatment strategies in AL amyloidosis aim at reducing the plasma cell clones, using chemotherapy regimens applied in multiple myeloma. A combination of melphalan and prednisone is the standard therapy of AL amyloidosis, but its results remain disappointing. Intensive chemotherapy regimens, with high-dose melphalan followed by peripheral blood stem cell transplantation, lead to increased response rates and improved survival, but are complicated by severe short term morbidity and mortality. These regimens offer the best clinical perspectives for selected patients, until the introduction of innovative agents capable of interfering with amyloid fibril formation.

Amyloid↗
PubMedDate not supplied

[Localized laryngeal amyloidosis. Endoscopic surgery with CO2 laser].

Laryngeal amyloidosis is a very rare disease. In the upper airways, the most common localization is the laryngeal organ, particularly the supraglotic region. Usually laryngeal amyloidosis is a localized primary form, although it has occasionally been reported in conjunction with system amyloidosis. We are reporting a new case of localized primary laryngeal amyloidosis in the right arytenoid mucosa in a 66 years old woman who underwent CO2 laser endoscopic surgery.

Aged↗
PubMed2001

[Beta 2-microglobulin and dialysis-related amyloidosis].

Dialysis-related amyloidosis is a common complication encountered in patients receiving chronic hemodialysis. beta 2-microglobulin(beta 2-m) constitutes the causative protein of amyloidosis, and its deposition in tissues has been proven to be a primary cause of the onset. However, many other risk factors are also associated including (1) prolonged duration of dialysis, (2) advanced age, (3) dialysate of low purity, and (4) the use of a dialysis membrane with poor biocompatibility. Recently we confirmed that apolipoprotein E is a risk factor for the onset of carpal tunnel syndrome associated with dialysis-related amyloidosis. Dialysis using high-flux membranes and the clinical application of a column that selectively adsorbs beta 2-m are currently being assessed interms of the ability to remove as much beta 2-m from the blood as possible. Drug therapy may also be useful at present in symptomatic treatment; a low dose of adrenocorticosteroids has recently been demonstrated as extremely effective for joint pain associated with beta 2-m amyloid deposits. The long-term use of drugs, however, carries a risk of side effects. As a therapeutic approach to dialysis-related amyloidosis, high-flux dialysis membranes permitting the elimination of beta 2-m with satisfactory biocompatibility have been developed and positive clinical effects have been observed both in the retrospective and prospective studies in the use of high-flux membranes together with a decrease in the serum beta 2-m level. However, there is no evidence yet of the long term reduction of amyloid deposits when the elimination of beta 2-m is maintained using a high-flux membrane.

Amyloid beta-Protein Precursor↗
PubMed1979

Diffuse parenchymal amyloidosis of lungs and breast. Its association with diffuse plasmacytosis and kappa-chain gammopathy.

A patient had systemic amyloidosis that extensively involved the lungs and breasts. Diffuse parenchymal pulmonary amyloidosis is rare but well described. Involvement of the breast in systemic amyloidosis is, however, most unusual. This patient's amyloidosis was associated with diffuse plasmacytosis and a kappa-chain gammopathy. The plasmacytosis was most prominent in the renal interstitium. Immunoperoxidase staining of the renal infiltrate and breast demonstrated IgG/kappa-staining plasma cells exclusively, suggesting that these cells were a monoclonal proliferation that contributed to the patient's M-protein and possibly to the patient's amyloid deposits.

Amyloidosis↗
PubMed2001

[Long-term experience with renal transplantation in systemic amyloidosis: a case report].

Renal transplantation was performed on a 39-year old woman with secondary amyloidosis due to rheumatoid arthritis. She remains alive and renal function has been maintained satistfactorily with the exception of proteinuria ten years after transplantation. Recent renal biopsy showed no amyloid recurrence, but the presence of chronic rejection reaction and mild cyclosporin arteriolopathy. Symptoms related to systemic amyloidosis and rheumatoid arthritis improved after transplantation. Renal transplantation is the recommended therapy for the type AA systemic amyloidosis. This is the second report of long-term experience with renal transplantation in systemic amyloidosis in Japan.

Aged↗
PubMed1975

Amyloidosis of the kidneys. Review of patients and literature.

The clinical findings in 49 patients (27 males) who had histologically confirmed amyloidosis of the kidney, are reviewed. In 28 patients, the diagnosis was arrived at by percutaneous renal biopsy, and in 21 patients it was made at autopsy. The youngest patient was diagnosed at the age of 16 years. In 11 patients (22,5%) no associated disease was found, in a further 11 patients the amyloidosis was of the type connected with familial Mediterranean fever, and in 27 patients (55%) the amyloidosis was of the secondary type. Forty-six patients (94%) presented with proteinuria at some stage of their disease. Twenty-one patients developed the nephrotic syndrome. Thirty-two patients died, and in 18 instances uraemia was the cause of death. In 18 patients there was evidence of renal pathology other than amyloidosis, and in some patients this may have contributed to the impairment of renal function and the appearance of proteinuria.

Adolescent↗
PubMed1975

[Effect of histamine and diprazin on the course of experimental amyloidosis in mice].

In experiment on 33 mice, line C57Bl, the effect of changed vascular-tissue permeability following administration of histamine and anti-histamine preparation diprazin (pipolphen) on the development of amyloidosis was studied. It was shown that histamine stimulated the development of amyloidosis in mice, causing besides generalized vascular disorders and a pronounced mast-cell reaction with degranulation of these cells, whereas diprazin, on the contrary, inhibited amyloidogenesis in mice. Differences between experimental and control groups were identified using the morphometric method and were confirmed statistically. The difference in the degree of amyloidosis revealed against the background of administration of histamine and diprazin testified to a certain extent to the role of impaired vascular-tissue permeability in the pathogenesis of amyloidosis.

Amyloidosis↗
PubMed2001

Primary localized amyloidosis of the urinary bladder. A case report.

We present case 96 (the first one in Bulgaria) of primary localized amyloidosis of the urinary bladder in a 72-year-old woman with episodes of painless hematuria. The amyloid tumor is situated on the anterior wall of the bladder--a yellowish prominence 15 mm in diameter with superficial erosion that is surrounded by a reddish ring and bullous edema of the mucosa. Rough and regularly pink perivascular deposits, thickened vessel walls and focal lymphoplasmocyte infiltrates are present in the submucosa. After preliminary treatment with KHMnO4, AL-amyloid is detected in polarized light by Congorot staining and in ultraviolet light using thioflavin. Immunoglobulins and C3 deposits are not found. On electron microscopy deposits of amyloid fibrils are seen in abundance close to collagen bundles. Diseases that may be associated with systemic amyloidosis have been clinically excluded. Nodular amyloidosis resulting from a local immune dyscrasia is accepted in the case. The patient is in good health 11 months after the first biopsy. A literature review on this rare form of localized amyloidosis has been made. Generally, the disease has a favorable prognosis and non-active behavior is recommended. Intravesical instillations of dimethyl sulfoxide, sectional resection or plastic surgery that preserve the urinary tract are used in the rare case of progressive course.

Aged↗
PubMed2002

The importance of complete screening for amyloid fibril type and systemic disease in patients with amyloidosis in the respiratory tract.

BACKGROUND: Patients with symptomatic thoracic involvement by amyloidosis are virtually all of AL-type, and have historically been divided into systemic and localised disease, a subdivision that helps predict outcome and aids management. Assessment and classification of amyloid has evolved in recent years to include a variety of tests, including radiolabelling of the serum amyloid P component (SAP scan) to assess anatomical distribution and immunohistochemical studies to assess fibril subtypes. Furthermore, CT scanning is now a frequent investigation for the diagnosis of thoracic disease. We wished to determine the value of these investigations on the management of such patients with amyloidosis. METHODS: Clinicopathological data, including immunohistochemical analysis, CT scans and SAP scan results, were retrospectively reviewed from patients presenting with amyloidosis in the respiratory tract. These were then analysed to determine their impact on classification and prognosis. RESULTS: Seventeen patients over ten years were identified, one case being related to metastatic medullary carcinoma of the thyroid. Of the remaining 16 cases, one was shown to have hereditary amyloid of transthyretin-type (TTR) on immunohistochemistry, altering management. The remaining 15 cases were AL-type, with 6 cases being classified as localised and 9 cases as systemic, after evaluation for serum and/or urine monoclonal products, cardiac involvement via echocardiography, plasma cell abnormalities on bone marrow examination, CT scan and SAP scan. 3/3 patients with localised AL-type disease had a negative SAP scan, whilst 3/5 patients with systemic AL-type disease had a positive scan. SAP scan of the patient with TTR-type disease provided information on extent of disease and supported the diagnosis by the pattern of distribution. Using CT scans to discriminate between localised and systemic disease showed a significant association with mortality at 2 years (p = 0.03). CONCLUSION: Although the majority of symptomatic patients with pulmonary amyloidosis have AL-type disease, immunohistochemical confirmation is necessary in order not to miss rarer subtypes with completely different treatment regimes. Furthermore, a comprehensive evaluation, including SAP scan and CT scan of the thorax, in conjunction with echocardiography, bone marrow, serum and urine studies, needs to be undertaken in order to achieve maximum accuracy with regard to localised and systemic disease.

Aged↗