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Risk for amyloidosis in rheumatic diseases is associated with a long-lasting inflammation. To assess possible changes in the incidence of terminal uraemia due to amyloidosis associated with rheumatic diseases on a nationwide basis, we scrutinised the files of the Finnish Registry for Kidney Diseases for patients suffering from amyloidosis associated with rheumatoid arthritis (RA), ankylosing spondylitis (AS) or juvenile idiopathic arthritis (JIA) over the period 1995-2008. The registry has an estimated 97-99% coverage of all patients accepted for renal replacement therapy (RRT) in the country. Data on the consumption of antirheumatic drugs were collected from two sources: the Social Insurance Institution's Drug Reimbursement Register, and the Sales Register of the National Agency for Medicines from the above period. Altogether 264 cases were identified. Two hundred twenty-nine of them had RA, 15 AS and 20 JIA. When the total annual number of new admissions to RRT varied between 20 and 37 at the end of 1990s, it was under half of that from 2002 onwards. Over this period, the number of users of low-dose methotrexate (MTX) has increased 3.6-fold, the drug being the most frequently used disease modifying anti-rheumatic drug in Finland. The present nationwide series is the first to show that the incidence of end-stage renal disease due to amyloidosis associated with rheumatic diseases is decreasing. An obvious reason for this is intensive anti-rheumatic drug therapy.

Objective: To assess the incidence, prevalence, and outcome of amyloidosis associated with inflammatory rheumatic diseases., Methods: An observational study was performed in the outpatient department of Kainuu Central Hospital from 1993 to 2007. The following criteria were used for the performance of abdominal subcutaneous fat aspiration (ASFA) and/or rectal biopsies: erythrocyte sedimentation rate (ESR) > 40 mm/h at two consecutive visits; and proteinuria (> 0.5 g/day) or serum creatinine > 150 μmol/L. Renal biopsy was performed when there was a high suspicion of amyloidosis in cases with negative findings in the above-mentioned biopsies. In addition, amyloid staining was used routinely for mucosal specimens taken in gastroscopy and colonoscopy. The patients were followed until death or to the end of 2007., Results: New diagnoses of amyloidosis in the consecutive 5-year periods from 1993 onwards numbered 11, 3, and 5, respectively. During the study period, there was a mean annual incidence of amyloidosis of 1.8 [95% confidence interval (CI) 1.1-2.8)/100,000]. At the end of 2007 there were eight subjects with amyloidosis, giving a point prevalence of 12.0/100,000 (95% CI 5.2-23.6). Five patients out of the 19 underwent haemodialysis because of terminal uraemia and three of them also had renal transplantation. Overall, 12 (63%) patients died after a median survival time of 6 (95% CI 4-8) years, one-third from amyloidosis. The 5-year survival rate of the series was 67% (95% CI 41-86)., Conclusion: Amyloidosis is rarely encountered today. ASFA or rectal biopsy facilitates its early diagnosis.

Objective: To assess the incidence and outcome of renal replacement therapy (RRT) among patients with amyloidosis associated with inflammatory rheumatic diseases., Methods: Patients with amyloidosis entering RRT from 1987 to 2002 were identified from the Finnish Registry for Kidney Diseases. Five hundred two patients were identified, 80% of whom had amyloidosis associated with an underlying rheumatic disease. They were followed from the time of entering RRT until death or until the end of 2003 using the Finnish national mortality files., Results: During the study period, there was no decline in the number of patients with amyloidosis entering RRT. Mean age of patients with rheumatoid arthritis (RA) and juvenile idiopathic arthritis (JIA) increased significantly from 1987 to 2002 (p < 0.001). Male sex and a diagnosis of JIA indicated an increased risk of mortality. The median survival time after entering RRT was 2.11 years for RA (95% CI 1.93 to 2.69), 2.37 years for ankylosing spondylitis (95% CI 1.11 to 4.31), and 3.05 years for JIA (95% CI 2.19 to 4.23). The 5-year survival rates among patients with the corresponding diagnoses were 18% (95% CI 14% to 23%), 30% (95% CI 14% to 48%), and 27% (95% CI 14% to 41%), respectively., Conclusion: No decline was seen in the number of patients with amyloidosis associated with inflammatory rheumatic diseases accepted for RRT, but over the years, the age of patients with RA or JIA entering RRT was seen to increase. The outcome of patients with amyloidosis and endstage renal disease associated with rheumatic diseases remains poor.

Objective: To determine the outcome of amyloidosis associated with juvenile idiopathic arthritis (JIA) in a hospital-based series., Methods: Patient registers and amyloidosis biopsy files of the Department of Pediatrics of Rheumatism Foundation Hospital, the main tertiary center for inflammatory joint disorders in children in Finland, were scrutinized from 1976 to the end of 2003 to look for amyloidosis in patients under age 19 years. Medical records were reviewed and patients were interviewed by telephone. The causes of any deaths were obtained from death certificates., Results: Twenty-four patients under age 19 years with biopsy-proven amyloidosis were found. As a sign of renal disease at the time of diagnosis of amyloidosis, 16 patients (67%) had proteinuria, but none had renal insufficiency. The 5-year survival rate of the series was 87.5% (95% CI 75% to 100%), and 10-year survival was 75% (54% to 92%). Ten patients (42%) out of the 24 died during a mean followup of 15.4 (range 1.5-27.6) years. The main cause of death was related to JIA in all patients but one. Patients treated with prednisolone alone from the diagnosis of amyloidosis onward had a mortality rate significantly higher than those taking disease modifying antirheumatic drugs and/or cytostatics (p = 0.001). At the end of followup, 14 patients (58%) were alive, 12 with normal renal function (3 of them had undergone renal transplantation), one had renal insufficiency, and one proteinuria. Proteinuria disappeared in 4 patients who were proteinuric (2 with nephrotic syndrome) at baseline, and their renal function remained normal. All the live patients had completed at least the 9 years of compulsory education, and 4 had academic degrees. Two female patients had delivered healthy children., Conclusion: The outcome of JIA-associated amyloidosis is poor. However, renal disease regressed in some patients under vigorous treatment. Successful treatment makes an active life possible for these patients.

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.