Familial Mediterranean fever (FMF) with proteinuria: clinical features, histology, predictors, and prognosis in a cohort of 25 patients
| Autor: | Dganit Dinour, Olga Kukuy, Ilan Ben-Zvi, Alexander Volkov, Aharon Ben-David, Juri Kopolovic, Avi Livneh, Eliezer J. Holtzman, Yael Shinar |
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| Rok vydání: | 2013 |
| Předmět: |
Adult
Male medicine.medical_specialty Pathology Biopsy Immunology Familial Mediterranean fever Kidney Gastroenterology End stage renal disease Nephropathy Rheumatology Predictive Value of Tests Internal medicine medicine Immunology and Allergy Humans Registries Retrospective Studies Proteinuria medicine.diagnostic_test business.industry Amyloidosis Acute Kidney Injury Middle Aged medicine.disease Prognosis Familial Mediterranean Fever medicine.anatomical_structure Female medicine.symptom business Kidney disease |
| Zdroj: | The Journal of rheumatology. 40(12) |
| ISSN: | 0315-162X |
| Popis: | Objective.Reactive (AA) amyloidosis may complicate familial Mediterranean fever (FMF), the prototype of autoinflammatory diseases. Thus, proteinuria in FMF is commonly viewed as resulting from amyloidosis, and kidney biopsy is deemed superfluous. However, nephropathy other than amyloidosis has been described in FMF, but its rate and distinctive characteristics are unknown. Our aim was to determine the rate and underlying pathology of FMF-related nonamyloidotic proteinuria and compare its clinical course, demographic, and genetic features to those of FMF-amyloid nephropathy.Methods.This study is a retrospective analysis of data from patients with FMF undergoing kidney biopsy for proteinuria above 0.5 g/24 h, over 10 years (2001–2011). Clinical, laboratory, genetic, and pathology data were abstracted from patient files. Biopsies were viewed by an experienced pathologist, as necessary.Results.Of the 25 patients referred for kidney biopsy, only 15 (60%) were diagnosed with amyloid kidney disease (AKD), and 10 were diagnosed with another nephropathy. The AKD and nonamyloid kidney disease (NAKD) groups were comparable on most variables, but showed distinct characteristics with regard to the degree of proteinuria (6.45 ± 4.3 g vs 2.14 ± 1.6 g, p = 0.006), rate of severe FMF (14 vs 5 patients, p = 0.022), and rate of development of end stage renal disease (73.3% vs 20%, p = 0.015), respectively.Conclusion.NAKD is common in FMF and, compared to amyloidosis, it is featured with milder course and better prognosis. Contrary to common practice, it is highly recommended to obtain a kidney biopsy from patients with FMF and proteinuria more than 0.5 g/24 h. |
| Databáze: | OpenAIRE |
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