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DOI: 10.4103/JLP.JLP_148_17
Revisiting renal amyloidosis with clinicopathological characteristics, grading, and scoring: A single-institutional experience
Financial support and sponsorship NilAbstract
INTRODUCTION: Kidney involvement is a major cause of mortality in systemic amyloidosis. Glomerulus is the most common site of deposition in renal amyloidosis, and nephrotic syndrome is the most common presentation. Distinction between AA and AL is done using immunofluorescence (IF) and immunohistochemistry (IHC). Renal biopsy helps in diagnosis and also predicting the clinical course by applying scoring and grading to the biopsy findings.
MATERIALS AND METHODS: The study includes all cases of biopsy-proven renal amyloidosis from January 2008 to May 2017. Light microscopic analysis; Congo red with polarization; IF; IHC for Amyloid A, kappa, and lambda; and bone marrow evaluation were done. Classification of glomerular amyloid deposition and scoring and grading are done as per the guidelines of Sen S et al.
RESULTS: There are 40 cases of biopsy-proven renal amyloidosis with 12 primary and 23 secondary cases. Mean age at presentation was 42.5 years. Edema was the most common presenting feature. Secondary amyloidosis cases were predominant. Tuberculosis was the most common secondary cause. Multiple myeloma was detected in four primary cases. Grading of renal biopsy features showed a good correlation with the class of glomerular involvement.
CONCLUSION: Clinical history, IF, and IHC are essential in amyloid typing. Grading helps provide a subtle guide regarding the severity of disease in the background of a wide range of morphological features and biochemical values. Typing of amyloid is also essential for choosing the appropriate treatment.
Publication History
Received: 24 September 2017
Accepted: 16 January 2018
Article published online:
19 February 2020
© 2018.
Thieme Medical and Scientific Publishers Private Ltd.
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References
- 1 Glenner GG. Amyloid deposits and amyloidosis. The beta-fibrilloses ( first of two parts). N Engl J Med 1980;302:1283-92.
- 2 Westermark P, Benson MD, Buxbaum JN, Cohen AS, Frangione B, Ikeda S, et al. Aprimer of amyloid nomenclature. Amyloid 2007;14:179-83.
- 3 Pepys MB. Amyloidosis. Annu Rev Med 2006;57:223-41.
- 4 Sipe JD, Benson MD, Buxbaum JN, Ikeda S, Merlini G, Saraiva MJ, et al. Amyloid fibril protein nomenclature: 2012 recommendations from the Nomenclature Committee of the International Society of Amyloidosis. Amyloid 2012;19:167-70.
- 5 Sipe JD, Benson MD, Buxbaum JN, Ikeda S, Merlini G, Saraiva MJ, et al. Nomenclature 2014: Amyloid fibril proteins and clinical classification of the amyloidosis. Amyloid 2014;21:221-4.
- 6 Gejyo F, Yamada T, Odani S, Nakagawa Y, Arakawa M, Kunitomo T, et al. Anew form of amyloid protein associated with chronic hemodialysis was identified as beta 2-microglobulin. Biochem Biophys Res Commun 1985;129:701-6.
- 7 Real de Asúa D, Costa R, Contreras MM, Gutiérrez Á, Filigghedu MT, Armas M, et al. Clinical characteristics of the patients with systemic amyloidosis in 2000-2010. Rev Clin Esp (Barc) 2013;213:186-93.
- 8 Dember LM. Amyloidosis-associated kidney disease. J Am Soc Nephrol 2006;17:3458-71.
- 9 Keeling J, Teng J, Herrera GA. AL-amyloidosis and light-chain deposition disease light chains induce divergent phenotypic transformations of human mesangial cells. Lab Invest 2004;84:1322-38.
- 10 Gertz MA. Amyloidosis: Diagnosis and prognosis. Fut Rheumatol 2008;3:369-80.
- 11 Sen S, Sarsik B. A proposed histopathologic classification, scoring, and grading system for renal amyloidosis: Standardization of renal amyloid biopsy report. Arch Pathol Lab Med 2010;134:532-44.
- 12 Said SM, Sethi S, Valeri AM, Leung N, Cornell LD, Fidler ME, et al. Renal amyloidosis: Origin and clinicopathologic correlations of 474 recent cases. Clin J Am Soc Nephrol 2013;8:1515-23.
- 13 Sasatomi Y, Kiyoshi Y, Uesugi N, Hisano S, Takebayashi S. Prognosis of renal amyloidosis: A clinicopathological study using cluster analysis. Nephron 2001;87:42-9.
- 14 Verine J, Mourad N, Desseaux K, Vanhille P, Noël LH, Beaufils H, et al. Clinical and histological characteristics of renal AA amyloidosis: A retrospective study of 68 cases with a special interest to amyloid-associated inflammatory response. Hum Pathol 2007;38:1798-809.
- 15 Carone FA, Epstein FH. Nephrogenic diabetes insipidus caused by amyloid disease. Evidence in man of the role of the collecting ducts in concentrating urine. Am J Med 1960;29:539-44.
- 16 Asmundsson P, Snaedal J. Persistent water diuresis in renal amyloidosis. A case report. Scand J Urol Nephrol 1981;15:77-9.
- 17 Dumoulina M, Bader R. A short historical survey of developments in amyloid research. Protein Pept Lett 2006;13:213-7.
- 18 Kebbel A, Röcken C. Immunohistochemical classification of amyloid in surgical pathology revisited. Am J Surg Pathol 2006;30:673-83.
- 19 Strege RJ, Saeger W, Linke RP. Diagnosis and immunohistochemical classification of systemic amyloidoses. Report of 43 cases in an unselected autopsy series. Virchows Arch 1998;433:19-27.
- 20 Schönland SO, Hegenbart U, Bochtler T, Mangatter A, Hansberg M, Ho AD, et al. Immunohistochemistry in the classification of systemic forms of amyloidosis: A systematic investigation of 117 patients. Blood 2012;119:488-93.
- 21 Watanabe T, Saniter T. Morphological and clinical features of renal amyloidosis. Virchows Arch A Pathol Anat Histol 1975;366:125-35.
- 22 Shiiki H, Shimokama T, Yoshikawa Y, Toyoshima H, Kitamoto T, Watanabe T, et al. Renal amyloidosis. Correlations between morphology, chemical types of amyloid protein and clinical features. Virchows Arch A Pathol Anat Histopathol 1988;412:197-204.
- 23 Shiiki H, Shimokama T, Yoshikawa Y, Onoyama K, Morimatsu M, Watanabe T, et al. Perimembranous-type renal amyloidosis: A peculiar form of AL amyloidosis. Nephron 1989;53:27-32.
- 24 Nagata M, Shimokama T, Harada A, Koyama A, Watanabe T. Glomerular crescents in renal amyloidosis: An epiphenomenon or distinct pathology? Pathol Int 2001;51:179-86.
- 25 Pinney JH, Smith CJ, Taube JB, Lachmann HJ, Venner CP, Gibbs SD, et al. Systemic amyloidosis in England: An epidemiological study. Br J Haematol 2013;161:525-32.
- 26 Shah VB, Phatak AM, Shah BS, Kandalkar BM, Haldankar AR, Ranganathan S, et al. Renal amyloidosis – A clinicopathologic study. Indian J Pathol Microbiol 1996;39:179-85.
- 27 Gertz MA, Kyle RA. Secondary systemic amyloidosis: Response and survival in 64 patients. Medicine (Baltimore) 1991;70:246-56.
- 28 Dixit R, Gupta R, Dave L, Prasad N, Sharma S. Clinical profile of patients having pulmonary tuberculosis and renal amyloidosis. Lung India 2009;26:41-5.
- 29 Tuglular S, Yalcinkaya F, Paydas S, Oner A, Utas C, Bozfakioglu S, et al. Aretrospective analysis for aetiology and clinical findings of 287 secondary amyloidosis cases in Turkey. Nephrol Dial Transplant 2002;17:2003-5.
- 30 Bergesio F, Ciciani AM, Santostefano M, Brugnano R, Manganaro M, Palladini G, et al. Renal involvement in systemic amyloidosis – An Italian retrospective study on epidemiological and clinical data at diagnosis. Nephrol Dial Transplant 2007;22:1608-18.
- 31 Usha, Singh RG, Parkash J, Kapoor R, Rai S, Sinha DK. Clinicopathological study of renal amyloidosis. Clinicopathological study of renal amyloidosis. JK Sci 2006;8:18-23.
- 32 Chugh KS, Datta BN, Singhal PC, Jain SK, Sakhuja V, Dash SC, et al. Pattern of renal amyloidosis in Indian patients. Postgrad Med J 1981;57:31-5.
- 33 Hopfer H, Wiech T, Mihatsch MJ. Renal amyloidosis revisited: Amyloid distribution, dynamics and biochemical type. Nephrol Dial Transplant 2011;26:2877-84.