Original contributionClinical and histological characteristics of renal AA amyloidosis: a retrospective study of 68 cases with a special interest to amyloid-associated inflammatory response
Introduction
Amyloidoses constitute a heterogeneous group of diseases characterized by extracellular deposition of amyloid proteins in different tissues and organs. Amyloid deposits are identified on the basis of their morphologic, structural, and tinctorial characteristics [1]. In human pathology, 25 different proteins are known to have the ability to aggregate in insoluble fibrillar deposits, including amyloid A protein responsible for AA amyloidosis [1]. The AA amyloid proteins result from a proteolytic cleavage of acute phase serum amyloid A protein (A-SAAs) usually in the C-terminal half of the 104 amino acid residues long precursor. Acquired and hereditary diseases can lead to systemic AA amyloidosis provided they are associated with chronic inflammation. Renal involvement, extremely frequent in systemic AA amyloidosis, is a major cause of morbidity and mortality. The aim of this study was to analyze the histological characteristics of a series of renal AA amyloidosis and their possible clinical implications. We were interested in the distribution pattern of amyloid deposits in renal parenchyma and more particularly in the 2 principal histological forms of renal AA amyloidosis, that is, glomerular and vascular forms [2], [3], [4], [5]. We also studied the inflammatory reaction related to renal AA amyloidosis. Although inflammation against amyloid deposits plays an important role in Aβ and Aβ2m amyloidoses [6], [7], few data are currently available on this subject in renal AA amyloidosis.
Section snippets
Patients and materials
One hundred seventy-four cases of renal amyloidosis were observed between January 1990 and December 2005 in 3 departments of renal Pathology (Hôpital Necker, Hôpital Pitié-Salpêtrière, and Hôpital Saint-Louis). They included 68 cases (39.1%) of AA amyloidosis and 105 cases (60.3%) of AL amyloidosis (AA/AL amyloidosis ratio, 1:1.5). The remaining case concerned a patient with a mutation in the fibrinogen A alpha chain gene. We gathered in our series the 68 cases of renal AA amyloidosis and
Clinical findings
Among the 68 observations of renal AA amyloidosis, 2 were previously reported [11], [12]. These 68 patients included 33 females and 35 males. The mean age at diagnosis was 57.6 ± 15.1 years, 58.9 ± 16.5 years for women and 56.4 ± 13.8 years for men. A total of 40 (58.8%) patients originated from Europe, 19 (27.9%) from North Africa, 6 (8.8%) from sub-Saharan Africa, 2 (3%) from Turkey, and 1 (1.5%) from Armenia. All the amyloidogenic diseases are listed in Table 1, except for 3 patients without
AA/AL amyloidosis ratio and etiology of AA amyloidosis
The relative proportion of AA amyloidosis in systemic amyloidosis largely varies according to geographic area. AA/AL amyloidosis ratio was 2 per 1 in west Europe versus 1 per 17 in the United States of America [13]. In our study, this ratio was 1 per 1.5. However, it was 1 per 0.8 during the previous decade with an unchanged recruitment (personal data). This result tends to confirm the current preponderance of renal AL amyloidosis in France.
Etiology of AA amyloidosis in western countries has
Acknowledgments
The authors would like to thank Prs J. P. Grünfeld, G. Deray, C. Legendre, J. C. Piette, S. Herson, P. Bourgeois, L. -J. Couderc, and Drs P. Lebon, L. Dupouët, C. Barbanel, J. Montseny, J. -M. Trawale, G. Moret, and F. Beaufils for their kind contributions to this study.
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