Most cited article - PubMed ID 12817067
Current therapeutic possibilities in primary and secondary amyloidosis and our experience with 31 patients
Renal amyloid involvement results, especially, from AL (primary) or AA (secondary) amyloidosis. The extent of amyloid tissue deposits in the kidneys and the clinical course of amyloidosis not only depend on the type of basic process but also reflect the time of diagnosis and the ability to affect the underlying disease. We analyzed laboratory and clinical data from patients with bioptically proven renal amyloidosis. Renal amyloidosis was found in 99 patients (4.65%) from an overall number of 2,128 renal biopsies (RB) performed in our department during a period of 11 years (from 1995 to 2006). AA amyloidosis was diagnosed in 46 patients. Nephrotic syndrome was diagnosed in 27 patients (59%) with AA amyloidosis; all these patients had different degrees of proteinuria. Impaired renal function was discovered in 24 patients (52%); in three of these patients (6.5%) we had to start renal replacement therapy. Patients were treated with corticosteroids, disease-modifying antirheumatic drugs (DMARDs), and biological therapy in various regimens. Nine patients (19.5%) died during the one-year follow-up period; complications such as sepsis and cardiac failure were the leading causes of death. Median survival in the AA group was 54 months. Although for approximately half of patients different treatment regimens can lead to a partial remission or disease stabilization, the prognosis of patients with amyloidosis could be regarded as unsatisfactory.
- MeSH
- Amyloid metabolism MeSH
- Amyloidosis diagnosis epidemiology MeSH
- Survival Analysis MeSH
- Antirheumatic Agents therapeutic use MeSH
- Kidney Failure, Chronic diagnosis epidemiology MeSH
- Adult MeSH
- Risk Assessment MeSH
- Immunohistochemistry MeSH
- Incidence MeSH
- Biopsy, Needle MeSH
- Cohort Studies MeSH
- Drug Therapy, Combination MeSH
- Middle Aged MeSH
- Humans MeSH
- Nephrology statistics & numerical data MeSH
- Nephrotic Syndrome diagnosis epidemiology MeSH
- Kidney Diseases diagnosis drug therapy epidemiology MeSH
- Statistics, Nonparametric MeSH
- Prednisolone therapeutic use MeSH
- Prognosis MeSH
- Retrospective Studies MeSH
- Sex Distribution MeSH
- Aged MeSH
- Severity of Illness Index MeSH
- Age Distribution MeSH
- Kidney Function Tests MeSH
- Health Surveys MeSH
- Check Tag
- Adult MeSH
- Middle Aged MeSH
- Humans MeSH
- Male MeSH
- Aged MeSH
- Female MeSH
- Publication type
- Journal Article MeSH
- Research Support, Non-U.S. Gov't MeSH
- Geographicals
- Czech Republic epidemiology MeSH
- Names of Substances
- Amyloid MeSH
- Antirheumatic Agents MeSH
- Prednisolone MeSH