الفهرس 
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Abstract
Amyloidosis is an inherited or acquired storage disease which may be localized or systemic. In amyloidosis, amorphous substance is deposited in the extracellular space of various tissues. The amyloid is produced because of abnormal protein metabolism and resistance to proteolysis. As a result of the deposits, destruction of normal tissue structure and disruption of its function are happened. The course of the disease may be progressive and fatal
Renal affection in systemic amyloidosis is frequent and often is the major source of morbidity for individuals with these disorders. Without treatment, amyloidosis-associated kidney disease usually progresses to end-stage renal disease (ESRD).
Clinically, renal involvement mainly occurs in AL or AA amyloidosis. Renal involvement can also occur in some hereditary forms of amyloidosis, most commonly in AFib, AApoAI, AApoAII, ALys, and AGel, but it is rare in ATTR. Recently, LECT2 has already been established as a frequent form of systemic amyloidosis in the United States, with predominant involvement of the kidney and liver.
Data of archived native renal biopsies, received in a single private pathology lab between period of 1st of January 2012 to 1st of October 2016 were revised. The total number of the cases that were diagnosed as renal amyloidosis was 310 cases however, 207 cases were adequate to be enrolled in this study. The remaining 103 cases were excluded from the study due to lack/paucity of remaining tissue in the paraffin blocks which prohibits doing extra sections for further work up.
The original slides as well as the paraffin blocks of the study cases were retrieved. The slides were stained with Haematoxylin and Eosin (H&E), Masson trichrome and Periodic acid Schiff (PAS) and Congo red stain. When the original slides were not available, new sections were done to be stained with the previously mentioned stains. Moreover, IHC was done on all cases with the following antibodies: anti SAA, anti Kappa, anti Lambda and anti LECT2 antibodies. Histological assessment for all the cases was done to evaluate the type and the extent of amyloid deposits in the embedded renal tissue.
The clinical data of the study patients were revised including age, sex, the association with chronic diseases including hypertension, diabetes mellitus and hepatitis C virus, serum creatinine and proteinuria level. Relevant clinical conditions to certain types of amyloidosis were revised. For AA amyloidosis; history of chronic inflammatory or infectious diseases was counted. In terms of AL amyloidosis; history of plasma cell dyscrasis was considered.
The over all prevalence of renal amyloidosis in this study was 6% which is considered a high percentage in comparison to the published studies. Regarding the immunophenotyping; AA amyloidosis was the most common type representing 58% of the study cases, followed by AL cases at 22.7% and finally LECT2 cases at 17.8%. One case was diagnosed as AFIB and two were unclassified, negative to all antibodies used in this study.
In terms of demographic data; the mean age of the study patients was 48.3%, however, about three quarters of AL and LECT2 cases were aged above 51years. When gender of the patients was reviewed; there was statistically significant male predominance.
The correlation with the clinical findings showed that the mean scr in this series was 4.78mg% and 26% of the patients presented with acute renal failure and were on dialysis at the time of biopsy. Regarding the level of proteinuria, 43% of the patients had nephrotic proteinuria and 57% had subnephrotic range.
Hypertension was considerably noticed in AL and LECT2 patients with statistical significance. In addition; LECT2 was associated with DM more than the other types of amyloidosis with statistical significance.
Among AA group; 49 patients had an associated chronic inflammatory conditions. FMF was the most common (18%). Forty percent of AL amyloid cases had an associated plasma cell dyscrasia. MM was the most common type (26%).
Regarding histopathological finding; 38% of the study patients had isolated glomerular deposits, 19% had isolated tubulointerstitial deposits and 47% showed combined amyloid deposits in both glomeruli and tubules. Arterioles were basically affected in most of the patients (84%).
Isolated glomerular deposits were identified in 56% of the AA group while they were not identified among LECT2 group and was recorded in 26% of AL group. Isolated tubulointerstitial amyloid deposits were highly significant among LECT2 group (64%), in comparison to the AL (23%) and AA (4%).
We can conclude from this study that renal amyloidosis is not a rare disease among the Egyptian population. Also, there are other types of renal amyloidosis rather than AA amyloidosis such as AL and LECT2 amyloidosis. Last but not the least; LECT2 amyloidosis is relatively common among the Egyptian population in comparison to other studies done on different nations and it shows a particular tubulointerstitial pattern of deposits.
Amyloidosis is an inherited or acquired storage disease which may be localized or systemic. In amyloidosis, amorphous substance is deposited in the extracellular space of various tissues. The amyloid is produced because of abnormal protein metabolism and resistance to proteolysis. As a result of the deposits, destruction of normal tissue structure and disruption of its function are happened. The course of the disease may be progressive and fatal
Renal affection in systemic amyloidosis is frequent and often is the major source of morbidity for individuals with these disorders. Without treatment, amyloidosis-associated kidney disease usually progresses to end-stage renal disease (ESRD).
Clinically, renal involvement mainly occurs in AL or AA amyloidosis. Renal involvement can also occur in some hereditary forms of amyloidosis, most commonly in AFib, AApoAI, AApoAII, ALys, and AGel, but it is rare in ATTR. Recently, LECT2 has already been established as a frequent form of systemic amyloidosis in the United States, with predominant involvement of the kidney and liver.
Data of archived native renal biopsies, received in a single private pathology lab between period of 1st of January 2012 to 1st of October 2016 were revised. The total number of the cases that were diagnosed as renal amyloidosis was 310 cases however, 207 cases were adequate to be enrolled in this study. The remaining 103 cases were excluded from the study due to lack/paucity of remaining tissue in the paraffin blocks which prohibits doing extra sections for further work up.
The original slides as well as the paraffin blocks of the study cases were retrieved. The slides were stained with Haematoxylin and Eosin (H&E), Masson trichrome and Periodic acid Schiff (PAS) and Congo red stain. When the original slides were not available, new sections were done to be stained with the previously mentioned stains. Moreover, IHC was done on all cases with the following antibodies: anti SAA, anti Kappa, anti Lambda and anti LECT2 antibodies. Histological assessment for all the cases was done to evaluate the type and the extent of amyloid deposits in the embedded renal tissue.
The clinical data of the study patients were revised including age, sex, the association with chronic diseases including hypertension, diabetes mellitus and hepatitis C virus, serum creatinine and proteinuria level. Relevant clinical conditions to certain types of amyloidosis were revised. For AA amyloidosis; history of chronic inflammatory or infectious diseases was counted. In terms of AL amyloidosis; history of plasma cell dyscrasis was considered.
The over all prevalence of renal amyloidosis in this study was 6% which is considered a high percentage in comparison to the published studies. Regarding the immunophenotyping; AA amyloidosis was the most common type representing 58% of the study cases, followed by AL cases at 22.7% and finally LECT2 cases at 17.8%. One case was diagnosed as AFIB and two were unclassified, negative to all antibodies used in this study.
In terms of demographic data; the mean age of the study patients was 48.3%, however, about three quarters of AL and LECT2 cases were aged above 51years. When gender of the patients was reviewed; there was statistically significant male predominance.
The correlation with the clinical findings showed that the mean scr in this series was 4.78mg% and 26% of the patients presented with acute renal failure and were on dialysis at the time of biopsy. Regarding the level of proteinuria, 43% of the patients had nephrotic proteinuria and 57% had subnephrotic range.
Hypertension was considerably noticed in AL and LECT2 patients with statistical significance. In addition; LECT2 was associated with DM more than the other types of amyloidosis with statistical significance.
Among AA group; 49 patients had an associated chronic inflammatory conditions. FMF was the most common (18%). Forty percent of AL amyloid cases had an associated plasma cell dyscrasia. MM was the most common type (26%).
Regarding histopathological finding; 38% of the study patients had isolated glomerular deposits, 19% had isolated tubulointerstitial deposits and 47% showed combined amyloid deposits in both glomeruli and tubules. Arterioles were basically affected in most of the patients (84%).
Isolated glomerular deposits were identified in 56% of the AA group while they were not identified among LECT2 group and was recorded in 26% of AL group. Isolated tubulointerstitial amyloid deposits were highly significant among LECT2 group (64%), in comparison to the AL (23%) and AA (4%).
We can conclude from this study that renal amyloidosis is not a rare disease among the Egyptian population. Also, there are other types of renal amyloidosis rather than AA amyloidosis such as AL and LECT2 amyloidosis. Last but not the least; LECT2 amyloidosis is relatively common among the Egyptian population in comparison to other studies done on different nations and it shows a particular tubulointerstitial pattern of deposits.