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Renal and systemic amyloidosis in systemic lupus erythematosus 总被引:2,自引:0,他引:2
A young male presented with oral ulceration for two years; swelling face and feet of seven days duration; diffuse goiter without signs of thyroid disease; normocytic normochromic anemia, thrombocytopenia, deranged renal functions, albuminuria of 2.5 g/24h with active urinary sediment. ANA and anti-ds DNA were positive, sonography of abdomen suggested medical renal disease. Testing for HIV, HBV, VDRL, CRP, rheumatoid factor, p-ANCA and c-ANCA were negative. Thyroid hormone assays were normal. Kidney biopsy done to stage lupus nephritis did not show any evidence of lupus involvement but staining for SAA amyloid was positive. Subsequent biopsies from the liver and rectum also stained positive for amyloid. Diagnosis of "Systemic lupus erythematosus with renal and systemic secondary amyloidosis with euthyroid diffuse goiter" was made. The case is being reported and discussed because of the interesting and rare association between amyloidosis and systemic lupus erythematosus. 相似文献
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Renal transplantation in 45 patients with amyloidosis 总被引:2,自引:0,他引:2
The results of renal transplantation in patients with amyloidosis were studied in 45 patients receiving primary cadaver grafts at a single center between March 1973 and October 1981. A control group of 45 patients with glomerulonephritis receiving primary cadaver grafts during the same period was also studied. These were matched according to the number of A and B locus incompatibilities and the date of transplantation. The 3-year survival of the patients with amyloidosis was statistically significantly inferior (51%) to that of the controls (79%). Age over 40 years was the major factor determining low survival in these patients. Mortality was concentrated in the early posttransplantation period. The 3-year graft survival rate was the same in amyloidotics (38%) and controls (45%); with death of patients not included in graft loss, the corresponding figures were 53% and 49%. Appearance of amyloid in the transplant was established by biopsy in four patients at 11-37 months of follow-up. Renal transplants functioning for more than one year were calculated to incur a minimum risk of 20% of acquiring amyloid. 相似文献
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A. Hartmann H. Holdaas P. Fauchald K. P. Nordal K. J. Berg T. Talseth T. Leivestad I. B. Brekke A. Flatmark 《Transplant international》1992,5(1):15-18
At our center 62 renal transplantations (31 living donor and 31 cadaveric donor grafts) have been performed in 58 patients with amyloid renal disease since 1974. The amyloidosis was secondary to rheumatic disease in 74% of the patients. Predialytic transplantation was performed in 28% of the patients. Mean follow-up time was 5.1 years (0.3–14.5 years). One-year actuarial patient survival was 79%, decreasing to 65% after 5 years. First graft survival was 74% at 1 year and 62% at 5 years. Patient death with a functioning graft caused 16 out of 25 graft losses. Infections caused 11 out of 18 deaths (61%), more than half of them within 3 months. Renal transplant amyloid was diagnosed in about 10% of the cases (6/62); however, only about 3% of the grafts (2/62) were lost. These long-term results encourage transplantation in amyloid renal end-stage disease. 相似文献
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Renal amyloidosis is a detrimental disease caused by the deposition of amyloid fibrils. A child with renal amyloidosis may
present with proteinuria or nephrotic syndrome. Chronic renal failure may follow. Amyloid fibrils may deposit in other organs
as well. The diagnosis is through the typical appearance on histopathology. Although chronic infections and chronic inflammatory
diseases used to be the causes of secondary amyloidosis in children, the most frequent cause is now autoinflammatory diseases.
Among this group of diseases, the most frequent one throughout the world is familial Mediterranean fever (FMF). FMF is typically
characterized by attacks of clinical inflammation in the form of fever and serositis and high acute-phase reactants. Persisting
inflammation in inadequately treated disease is associated with the development of secondary amyloidosis. The main treatment
is colchicine. A number of other monogenic autoinflammatory diseases have also been identified. Among them cryopyrin-associated
periodic syndrome (CAPS) is outstanding with its clinical features and the predilection to develop secondary amyloidosis in
untreated cases. The treatment of secondary amyloidosis mainly depends on the treatment of the disease. However, a number
of new treatments for amyloid per se are in the pipeline. 相似文献
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Tsuda S Nishikido M Koga S Kanetake H Harada T Taguchi T Matsuya F Shindo K 《Hinyokika kiyo. Acta urologica Japonica》2001,47(6):415-419
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. 相似文献
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Ozen S 《Kidney international》2004,65(3):1118-1127
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Maurer MS Raina A Hesdorffer C Bijou R Colombo P Deng M Drusin R Haythe J Horn E Lee SH Marboe C Naka Y Schulman L Scully B Shapiro P Prager K Radhakrishnan J Restaino S Mancini D 《Transplantation》2007,83(5):539-545
BACKGROUND: Systemic amyloidosis complicated by heart failure is associated with high cardiovascular morbidity and mortality. Heart transplantation for patients with systemic amyloidosis is controversial due to recurrence of disease in the transplanted organ or progression of disease in other organs. METHODS: All patients with systemic amyloidosis and heart failure referred for heart transplant evaluation from 1997 to 2004 were included in this retrospective cohort analysis. An interdisciplinary protocol for cardiac transplantation using extended-donor criteria organs, followed in 6 months by either high-dose chemotherapy and stem cell transplantation for patients with primary (AL) or by orthotopic liver transplantation for familial (ATTR) amyloidosis, was developed. Survival of the transplanted amyloid cohort was compared to survival of those amyloid patients not transplanted and to patients transplanted for other indications. RESULTS: A total of 25 patients with systemic amyloidosis and heart failure were included in the study; 12 patients received heart transplants. Amyloid heart transplant recipients were more likely female (58% vs. 8%, P=0.02) and had lower serum creatinine (1.3+/-0.5 vs. 2.0+/-0.7 mg/dL, P=0.01) than nontransplanted amyloid patients. Survival at 1-year after heart transplant evaluation was higher among transplanted patients (75% vs. 23%) compared to patients not transplanted (P=0.001). Short-term survival posttransplant did not differ between transplanted amyloid patients and contemporaneous standard and extended-donor criteria heart transplant patients (P=0.65). CONCLUSIONS: Cardiac transplantation for amyloid patients with extended-donor criteria organs followed by either stem cell or liver transplantation is associated with improved survival compared to patients not transplanted. Short- to intermediate-term survival is similar to patients receiving heart transplantation for other indications. This clinical management strategy provides cardiac amyloid patients a novel therapeutic option. 相似文献
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Renal involvement in systemic amyloidosis: an Italian collaborative study on survival and renal outcome. 总被引:1,自引:0,他引:1
F Bergesio A M Ciciani M Manganaro G Palladini M Santostefano R Brugnano A M Di Palma M Gallo A Rosati P L Tosi M Salvadori 《Nephrology, dialysis, transplantation》2008,23(3):941-951
BACKGROUND: Few data are available from large population-based studies on survival and renal outcome of patients with renal involvement and different types of systemic amyloidosis. METHODS: Two hundred and ninety of over 373 patients affected from systemic amyloidosis with renal involvement diagnosed in Italy between January 1995 and December 2000 were followed from diagnosis to death or until the last available clinical control. Eighty-three patients were excluded from analysis either because the amyloid type remained undetermined or they were lost at follow-up. Clinical and laboratory information was collected according to the different types of amyloidosis using a specific form which included renal function with 24 h proteinuria at diagnosis and at the end of follow-up, the type and the date of onset of dialysis and the kind of treatment they underwent. RESULTS: The median time of follow-up was 24 months in primary (AL) amyloidosis (range: 1-88 months), 16 months in AL with associated multiple myeloma (MM + AL: range 1-76 months), 30 months in reactive (AA) amyloidosis (range: 1-99 months) and 52 months in patients with familial forms (AF: range 14-82 months). Patients with AL showed a significantly shorter survival than AA. Despite no significant differences of renal outcome or survival on dialysis being observed between the two groups, a lower renal survival with a higher number of patients who progressed to end-stage renal disease (ESRD) was observed in patients with AA. Overall survival was markedly improved in patients with AL who underwent a specific therapy (conventional chemotherapy or autologous stem cell transplantation (ASCT)) even in the absence of a positive kidney response. Multivariate analysis showed cardiac involvement and specific therapy to significantly influence survival in AL whereas age, serum creatinine (sCr) and heart involvement significantly affected survival in AA. In both groups, sCr and heart involvement were the most relevant predictors for renal outcome, together with urinary protein excretion, in patients with AA. CONCLUSIONS: Our results show a worse survival in AL due to the higher prevalence of heart involvement in this group and emphasize that a specific therapy significantly prolongs survival and slows the progression of renal disease in patients with AL. We suggest that a late nephrological referral is likely the cause of the higher sCr found at presentation in patients with AA and probably accounts for the lower renal survival observed in the short term in these patients. At the time being, renal transplantation and ASCT are still rare therapeutic options for renal patients affected from systemic amyloidosis. 相似文献
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Michael W. Rich 《Head & neck》1995,17(4):343-345
Background. Systemic amyloidosis leads to functional compromise of various organs through infiltration of these tissues by amyloid protein. The majority of affected patients develop infiltration of the thyroid gland; yet thyroid dysfunction rarely occurs. Methods and Results. Described herein is a case of hypothyroidism occurring in a patient with amyloid goiter. An alternative explanation for her hypothyroidism could not be determined by histologic or serologic evaluation. Hormonal assays were consistent with progressive hypothyroidism in spite of oral and, finally, parenteral replacement therapy. A review of the literature describes other cases of thyroid dysfunction reported in patients with systemic amyloidosis. Conclusions. This case illustrates how amyloid infiltration may be a cause of hypothyroidism in patients with systemic amyloidosis. Therefore, all patients who develop a goiter, including those with systemic amyloidosis, must be screened for thyroid dysfunction. When a physician diagnoses the patient with hypothyroidism, that physician must be aware of potential pitfalls in the administration of thyroxine replacement as outlined below. 相似文献
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R R Razonable R Patel M P Wilhelm M A Gertz M R Litzow D J Inwards J A Dearani B S Edwards C G McGregor 《American journal of transplantation》2001,1(1):93-95
Infectious complications are a major cause of morbidity and mortality in transplant recipients. We describe a case of fatal disseminated aspergillosis immediately following autologous peripheral stem cell reconstitution in a patient who had undergone orthotopic heart transplantation for systemic amyloidosis. The case described suggests that the infectious risks in patients undergoing these sequential procedures may be distinct from those occurring in patients undergoing either procedure independently. Potential prophylactic and therapeutic interventions are discussed. Since this experimental and evolving approach for the management of systemic amyloidosis is potentially applicable to a limited number of patients, multicenter collaboration may be needed to further define the infectious risks in this unique subset of transplant recipients. 相似文献
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Otukesh H Hoseini R Behzadi AH Fereshtehnejad SM Mehrazma M Parvin M Mojtahedzadeh M Razizadeh N Pourfakharan M 《Saudi journal of kidney diseases and transplantation》2011,22(5):1019-1021
Amyloidosis represents a heterogeneous group of disorders of protein metabolism and is characterized by deposition of fibrillar proteins in the intra- and extracellular spaces. Here, a case of generalized amyloidosis associated with neutropenia is presented. She had a medical history of multiple bacterial infections. At the age of 14 years, she developed nephrotic syndrome. An increase of antigenic stimulation during the intermittent bouts of acute infections would have been the main factor responsible for the development of secondary amyloidosis in this case. To the best of our knowledge, coexistence between neutropenic disorders and renal amyloidosis in children has not been reported till date. The purpose of this report is to present a case of secondary amyloidosis associated with neutropenia in pediatric age group, probably for the first time. 相似文献
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The term amyloidosis refers to the deposition of an amorphous substance defined by the presence of a fibrillar structure by electron microscopy and a characteristic beta-pleated sheet structure by x-ray diffraction. The existence of this group of diseases has been recognized for centuries, but major advances in the elucidation of the nature of the deposits have been achieved in the past few years. It is now recognized that the beta-sheet configuration responsible for the general appearance of amyloid deposits is common to a large array of proteins with a different origin. This tertiary structure is responsible for the characteristic tinctorial and optical properties evident by Congo red staining. The material that accumulates in the extracellular compartment progressively destroys the involved organ. The accumulation of amyloid deposits may complicate the course of a wide variety of diseases. Classifications of amyloid disorders reflect some of the current concepts concerning the protein composition of amyloid fibrils in different diseases. 相似文献
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As reported in the previous chapter (Part I) the term amyloidosis refers to the deposition of an amorphous substance defined by the presence of fibrillary structure by electron microscopy and a characteristic betapleated sheet structure by X-ray diffraction. Renal biopsy is fundamental in diagnostic terms because morphological demonstration is necessary and the tissue most frequently involved by amyloidosis is the kidney. In the previous chapter we presented the different histochemical and immunohistochemical techniques that are necessary to differentiate the two main types of amyloidosis defined as AL and AA. Here we show the variability of deposition of amyloid in glomerular, vascular and interstitial compartments. 相似文献