Among therapy modalities of end-stage renal disease, renal transplantation improves survival in patients with amyloidosis

The aim of this study was to investigate the results of renal transplantation in amyloidosis patients compared with those on hemodialysis. We compared a group of 25 patients with systemic amyloidosis and end-stage renal disease (ESRD) treated with renal transplantation with a control group of 30 patients with systemic amyloidosis and ESRD treated with hemodialysis. Overall 1-, 2-, and 5-year survival rates were 86.9%, 82.6%, and 78.2%, respectively, for patients, who had renal transplantations versus 60.7%, 50%, and 46.4%, respectively, for patients on hemodialysis treatments (P < .001). Among the control group 15 patients died at 9.4 +/- 7.5 months after starting hemodialysis. Among transplantation group five patients died during follow-up (mean 12.3 +/- 13.6 months); the major cause of death was infection. Only 18 patients experienced recurrences after renal transplantation; their 5-year survival rate was 84.2% versus 50% for patients who had no recurrence (P < .001). Patients with amyloid recurrence also had better long-term survival rates than patients in hemodialysis group (P < .001). In conclusion amyloidotic patients maintained on chronic dialysis have a high mortality rate. Better survival was noted for patients who had renal transplantations despite recurrences. These results encourage transplantation in amyloid renal end-stage disease.     

Dialysis-related amyloidosis after renal transplantation

There is no effective therapy for dialysis-related amyloidosis (DRA). The restoration of renal function with a functional graft seems the most reasonable therapy for this disturbing disease. Although there are not many prospective series on the efficacy of renal transplantation for DRA, all the studies agree that most of the patients experienced a significantly clinical improvement of the articular symptoms after a successful renal transplantation. Nevertheless, radiologic (bone cysts) and histologic lesions did not disappear after long-term follow-up.     

Fifteen years' experience with renal transplantation in systemic amyloidosis

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.     

Improved outcome of renal transplantation in amyloidosis

Abstract We report our results in 96 patients with amyloidosis who received 105 cadaveric renal allografts. The graft survival of amyloidosis patients has improved with time and with improved immunosuppression. The graft survival of amyloidosis patients is comparable to the results in another systemic disease, i.e., diabetes,     

Renal transplantation in 45 patients with amyloidosis

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.     

Lipoprotein abnormalities in patients with secondary renal amyloidosis

The prevalance of hyperlipidemia in chronic renal failure (CRF) patients is higher than in general population. Secondary amyloidosis is a common cause of CRF in Turkey. In this study, 25 patients with CRF due to secondary renal amyloidosis (amyloid-CRF), 15 patients with CRF without amyloidosis-CRF and 17 healthy controls were studied for serum lipid parameters. The mean serum lipoprotein (a) [LP(a)] level in the patients with amyloid-CRF was significantly higher than in the controls (p < 0.01). The mean serum apolipoprotein B (Apo B), apolipoprotein E (Apo E) and triglyceride levels in the patients with amyloid-CRF were very significantly higher than in the controls (p < 0.001). The mean serum total cholesterol, low- density lipoprotein (LDL) levels in the patients with amyloid-CRF were higher than in the controls (p < 0.05). The mean serum apo AI levels in the patients with amyloid-CRF was very significantly lower than in the controls (p < 0.001).The mean serum high-density lipoprotein (HDL) in the patients with amyloid-CRF was lower than in the controls (p < 0.05). The mean serum Lp (a), Apo AI, Apo B and Apo E levels in the patients with amyloid-CRF were significantly higher than in the patients with CRF (p < 0.01). The mean serum total cholesterol, trigliserides, LDL and HDL levels in the patients with amyloid-CRF were higher than in the patients with CRF (p < 0.05).There was not any correlation with serum lipid parameters and serum albumin and urine protein levels (p < 0.05).Our study suggests that serum lipid parameters are abnormal and might be the risk factor of atherosclerotic vascular disease and contribute to renal disease progression in the patients with secondary renal amyloidosis and lipid abnormalities were different from CRF with various etiology, without amyloidosis. This revised version was published online in June 2006 with corrections to the Cover Date.     

Long-term outcome of renal transplantation in patients with familial Mediterranean fever amyloidosis: a single-center experience

Although recurrence of amyloid A deposition in the allograft can be seen in patients with secondary amyloidosis due to familial Mediterranean fever (FMF), renal transplantation remains to be a choice of treatment for end-stage renal disease. The aim of this study was to determine short- and long-term results of renal transplantation in patients with FMF amyloidosis. We compared the outcomes of 17 patients with FMF amyloidosis among 431 (3.9%) transplants with 209 control patients. We observed 93% and 94% graft and patient survivals at 1 year, and 89% and 90% at 5 years. Also, the mean serum creatinine levels at 1 and 5 years posttransplant were similar. Recurrence of amyloidosis was documented in two allograft recipients presenting with nephrotic range proteinuria (12%), one of whom lost the allograft due to recurrence. Eleven patients had FMF gene analysis. The results of MEFV mutation analyses were: M694V/M694V homozygote in six patients, M694V/EQ148 in one patient, M694V/V726A in one patient, 680M-I/E148Q in one patient. FMF gene analysis was negative in two patients. Recurrence was noticed in one patient with M694V/M694V, while the other did not have an FMF gene analysis. Colchicine was reduced in nine patients due to side effects. In conclusion, the long-term outcomes of transplantation in patients with amyloidosis secondary to FMF is similar to that in the general transplant population and maintenance colchicine, even at low dose, appears to effectively prevent recurrence of amyloidosis in the allograft.     

Heart transplantation in patients with amyloidosis: single-center experience

Introduction

Amyloidosis is a systemic disease. Heart transplantation in this subset of patients is contraindicated by the majority of authors. In our center, patients with heart failure due to amyloidosis have been evaluated for cardiac transplantation since 1991. The aim of this study was to analyze the outcome of these patients waiting for transplant and the effectiveness of this therapy.

Materials and methods

Since 1991, eight patients affected by amyloidosis have been evaluated and enrolled on the waiting list for transplant: five affected by AL lambda type; two by APO A1; and one by TTR. Four were transplanted, three died waiting for a donor (two from cardiac failure, one from sudden death), and one has been recently transplanted after 17 months on waiting list.

Results

Since 1985, 713 patients underwent heart transplantation in our center, five of whom were affected by amyloidosis (0.7%). Two are still alive (60 and 41 months) without evidence of cardiac amyloidotic infiltration. One patient recently underwent a combined heart-liver transplantation. Two patients died after the intervention: one sudden death after 23 months with amyloidotic infiltration of transplanted heart, and one multiple organ failure (MOF) due to progression of the systemic disease.

Conclusions

Despite the small size of the group preventing us from drawing definitive conclusion, heart transplantation may prevent therapy to arrest organ damage in patients with isolated cardiac involvement. Cardiac events are the main cause of death. Patients must be followed-up for evolution of systemic disease. The midterm survival is encouraging.     

Study of live donor kidney transplantation outcome in recipients with renal amyloidosis

We studied the results of renal transplanta tion in 16 patientswith renal amyloidosis and in 46 controls with primary glomerulonephritis.Amyloidosis was primary in five and secondary to familial Mediterraneanfever (FMF) in 11. All patients received live related donorkidneys and the majority had onehaplotype HLA match. One- and5-year graft and patient survival rates were comparable in bothgroups. Moreover, the frequency of acute rejection episodesand the mean serum creatinine values were not signi ficantlydifferent between members of the two groups. Significant gastrointestinalsymptoms in the form of nausea, vomiting, abdominal pains, anddiarrhoea occurred in seven of the patients with amyloidosis(43.7%) and in only one of the controls (2%) (P=0.001). Allseven recipients with amyloidosis who developed the gastrointestinalmanifestations were receiving cyclosporin and six had FMF. Maintenancecolchicine treatment prevented recurrence of FMF symptoms. Inone patient discontinuation of colchicine was followed by recurrenceof FMF symptoms. Recurrence of renal amyloidosis was not observedin five patients subjected to Trucut graft biopsies 1, 2, 3,18 and 72 months post-transplantation. It is concluded that live-related donor kidney transplantationis a safe procedure in patients with amyloidosis and followsa course similar to glomerulo nephritis patients.     

Apolipoprotein and anticardiolipin antibodies in patients with renal amyloidosis

BACKGROUND: Thromboembolic events are seen more frequently in patients with chronic renal failure (CRF) and amyloidosis. The anticardiolipin antibody (ACA) that is important for thromboembolic events has never been studied. METHODS: This study included 43 amyloidosis patients of different aetiologies; 28 with CRF as well as 20 patients who had CRF without amyloidosis. Thirty normal subjects were included as a control group. We determined the serum levels of ACA, apolipoprotein AI (ApoAl), ApoE and lipoprotein (a) (Lp(a)) in these groups. RESULTS: Anticardiolipin antibody was found to be positive in 30.2% of patients with amyloidosis, this is in contrast to 3.3% in the control group (chi = 8.25, P < 0.005). We also showed that there was a statistically significant difference (chi = 5.03, P < 0.05) between the CRF patients with amyloidosis (31%) and the CRF patients without amyloidosis (5%). The average levels of serum ApoAI were shown to be significantly lower (P < 0.05) in CRF patients with amyloidosis in comparison with the amyloidosis patients who had normal renal functions (93.60 +/- 27.84 vs 119.8 +/- 36.26 mg/dL, P < 0.05). There was also significant a difference in ApoAI levels between CRF patients with and without amyloidosis (P < 0.001). The serum Lp (a) levels were significantly higher in CRF patients with amyloidosis when compared with the controls (41.2 +/- 22.39 vs 19.13 +/- 8.78 mg/dL, P < 0.001). The serum Lp (a) levels were also positively correlated with ACA (r = 0.211; P < 0.05). CONCLUSION: In conclusion, ACA positivity is more common in all patients with amyloidosis as compared with CRF patients and normal controls. This study is the first to show the presence of high levels of ACA in patients with CRF, which is caused by secondary amyloidosis. Further studies are recommended to investigate the mechanism of this finding.     

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.     

Renal transplantation: Complications in patients with juvenile diabetes

Four cases of patients with juvenile diabetes who received kidney transplants are reported. Problems with selection and management are discussed, and the high mortality and morbidity rates in this group of patients is emphasized.     

Delayed autologous stem cell transplantation following cardiac transplantation experience in patients with cardiac amyloidosis

This study sought to retrospectively investigate the outcomes of patients with light‐chain amyloidosis (AL) with advanced cardiac involvement who were treated with a strategy of heart transplantation (HT) followed by delayed autologous stem cell transplantation (ASCT) at 1‐year posttransplant. Patients with AL amyloidosis with substantial cardiac involvement have traditionally had very poor survival (eg, several months). A few select centers have reported their outcomes for HT followed by a strategy of early ASCT (ie, 6 months) for CA. The outcomes of patients undergoing a delayed strategy have not been reported. All patients with AL amyloidosis at a single institution undergoing evaluation for HT from 2004‐2018 were included. Retrospective analyses were performed. Sixteen patients underwent HT (including two combined heart‐kidney transplant) for AL amyloidosis. ASCT was performed in a total of nine patients to date at a median 13.5 months (12.8‐32.9 months) post‐HT. Survival was 87.5% at 1 year and 76.6% at 5 years, comparable to institutional outcomes for nonamyloid HT recipients. In addition to these 16 patients, two patients underwent combined heart‐lung transplantation. A strategy of delayed ASCT 1‐year post‐HT for patients with AL amyloidosis is feasible, safe, and associated with comparable outcomes to those undergoing an earlier ASCT strategy.     

Long-term effects of losartan on proteinuria and renal function in patients with renal amyloidosis

OBJECTIVE: To investigate the effect of the angiotensin II receptor antagonist losartan on proteinuria in secondary amyloidosis cases. MATERIAL AND METHODS: Sixteen patients with renal biopsy-proven AA amyloidosis with proteinuria were included in the study. All the patients had received colchicine treatment for at least 18 months. The patients were divided into two groups with similar age and gender distributions. Eight patients were given losartan at a dose of 50 mg/day for 12 months and the other 8 patients served as controls. Mean arterial blood pressure, proteinuria, serum albumin level and renal function were determined at the initiation of the study and after 1 and 12 months. RESULTS: There were no significant differences in proteinuria, serum albumin level, renal function or mean arterial blood pressure at the initiation of the study. In the losartan group daily proteinuria decreased significantly from 5.2 +/- 0.7 g at the initiation of the study to 3.9 +/- 1.2 g at 1 month and 3.6 +/- 0.8 g at 12 months, while in the control group it changed from 4.6 +/- 1.0 g to 4.7 +/- 1.0 g and 6.1 +/- 1.2 g, respectively. The increment at 12 months was significant. After 12 months of treatment with losartan, proteinuria was significantly lower in comparison to the degree of proteinuria in the control group. Serum albumin level increased significantly in the losartan group but was unchanged in the control group. In the control group, creatinine clearance showed a significant decrease. There was no significant difference in mean arterial blood pressure measurements, serum creatinine levels, total protein, albumin and creatinine clearance levels between the two groups. CONCLUSIONS: Losartan seemed to prevent an increase in proteinuria without altering the creatinine clearance level in patients with amyloidosis type AA during a 12-month period. This indicates that losartan may be used to decrease proteinuria in this patient group. However, our results are only preliminary and need to be confirmed by larger studies.     

Myeloablative chemotherapy and stem cell transplantation in myeloma or primary amyloidosis with renal involvement

BACKGROUND: High-dose chemotherapy and stem cell transplantation are being applied increasingly to the treatment of selected patients with multiple myeloma or primary systemic amyloidosis. Stem cell transplantation presents unique challenges to the nephrologist because of the high prevalence of renal involvement in myeloma and the issues that are associated with high-dose chemotherapy in patients with the nephrotic syndrome due to renal amyloid. METHODS: We review the published literature on stem cell transplantation in patients with reduced renal function. RESULTS AND CONCLUSIONS: The specifics of transplantation pertaining to patients with renal amyloid nephrotic syndrome are discussed in detail.