Clinical, biopsy, and mass spectrometry characteristics of renal apolipoprotein A-IV amyloidosis (original) (raw)
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Renal amyloidosis: a new time for a complete diagnosis
Brazilian Journal of Medical and Biological Research
Amyloidoses are a group of disorders in which soluble proteins aggregate and deposit extracellularly in tissues as insoluble fibrils, causing organ dysfunction. Clinical management depends on the subtype of the protein deposited and the affected organs. Systemic amyloidosis may stem from anomalous proteins, such as immunoglobulin light chains or serum amyloid proteins in chronic inflammation or may arise from hereditary disorders. Hereditary amyloidosis consists of a group of rare conditions that do not respond to chemotherapy, hence the identification of the amyloid subtype is essential for diagnosis, prognosis, and treatment. The kidney is the organ most frequently involved in systemic amyloidosis. Renal amyloidosis is characterized by acellular pathologic Congo red-positive deposition of amyloid fibrils in glomeruli, vessels, and/or interstitium. This disease manifests with heavy proteinuria, nephrotic syndrome, and progression to end-stage kidney failure. In some situations, it is not possible to identify the amyloid subtype using immunodetection methods, so the diagnosis remains indeterminate. In cases where hereditary amyloidosis is suspected or cannot be excluded, genetic testing should be considered. Of note, laser microdissection/mass spectrometry is currently the gold standard for accurate diagnosis of amyloidosis, especially in inconclusive cases. This article reviews the clinical manifestations and the current diagnostic landscape of renal amyloidosis.
Renal AA amyloidosis: survey of epidemiologic and laboratory data from one nephrology centre
International Urology and Nephrology
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 ...
The Prevalence of Renal Amyloidosis of the Aa-Type in a Series of 1,158 Consecutive Autopsies
Acta Pathologica Microbiologica Scandinavica Series A :Pathology, 1987
The prevalence of renal amyloidosis of the AA-type in a series of 1,158 consecutive autop sies. Acta path. microbiol. immunol. scand. Sect. A, 95: 297-302, 1987. To determine the prevalence of renal amyloidosis of the AA-type in a defined population, formalin-fixed specimens from the kidneys of all the cases autopsied in 1983 at The General Hospital of Malmo, Sweden, were investigated using immunohistochemical techniques. Amyloid deposits of protein AA were found in 10 of 1,158 investigated cases and the calculated prevalence was 0.86 per cent. The mean age at death of the individuals with the AA-type of amyloidosis was 79 years. Six of the cases with amyloidosis had rheumatoid arthritis. The avidin-biotin-peroxidase complex technique was found to be superior to the immunofluorexence method and a high sensitivity and specificity was achieved when sequence-specific antibodies against a synthetized nonapeptide corresponding to a hydrophilic segment of the polypeptide chain of protein AA were used in the assay. Nine cases with other types of amyloid deposits in the kidneys were also detected. None of these cases showed any AA immunoreactivity but all of them demonstrated Congophilic deposits which were immunohistochemically stained by antibodies against the amyloid Pcomponent. The prevalence of renal amyloidosis comprising all types of amyloid protein deposits was 1.64 per cent.
Journal of laboratory physicians
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. 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 . 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 predomin...
Problems in diagnosis of nonaa renal amyloidosis
Turkish Journal of Pathology, 2010
Objective: Amyloidosis represents extracellular deposition of congophilic fibrils of similar morphology but different chemical composition. The types frequently involving the kidney are AA (serum amyloid A) and AL (light chain amyloid). Hereditary amyloidoses, which can also affect the kidney, are being described with increasing frequency. Distinguishing the type of amyloid is important because of the different treatments and outcomes. typing of amyloidosis by immunohistochemistry (iHC) is believed to be unreliable, except for AA amyloid. We reevaluated our cases in this study to draw attention to the nonAA amyloid deposits in renal biopsies with amyloidosis. Material and Method: renal biopsies diagnosed as amyloidosis between 997 and 2008 were stained for amyloid A with iHC. Cases with negative amyloid A staining were included in the study. Results: We found 90 cases of renal amyloidosis (98 biopsies) during the study period. Thirty cases (33 biopsies) with negative antiAA staining were studied. Among those 30 cases with nonAA amyloidosis, secondary AL amyloidosis was due to multiple myeloma in 8 cases and plasma cell dyscrasia in 2 cases. Hereditary renal amyloidosis was suspected in some cases. Conclusion: using amyloid A iHC on a routine basis and the clinical history are essential to diagnose AA amyloidosis. The differential diagnosis of nonAA amyloidosis may be inconclusive on iHC. The diagnostic approach should be multidisciplinary and requires careful clinical and pathological evaluation. The limitation of iHC may necessitate direct typing of the amyloid protein extracted from paraffin-embedded specimens in some cases.
PRIMARY RENAL AMYLOIDOSIS: A CASE REPORT
International Journal of Medical Laboratory Research, 2016
Primary amyloidosis is the most common form of systemic amyloidosis. The morbidity arises from extracellular deposition of immunoglobulin light chain fibrils in some organs such as the kidneys, heart and bowel. Distinctive haematological and biochemical laboratory findings may help in early diagnosis. Here we present a 60-year-old lady with an generalized amyloidosis. On microscopy sections show small fragments of structure less material was seen giving orange-red appearance, when stained with Congo Red. Our comprehensive overview of this rare and often fatal disease aims to increase the awareness of AL type amyloidosis. This may facilitate earlier diagnosis and thus allow initiation of prompt and specific therapies, which are indispensable in order to improve disease prognosis.
Clinicopathological study of renal amyloidosis
2006
Study included 13 cases of renal amyloidosis.Oedema, feet and face was the commonest manifestation (100%), two patients (18.18%) also presented with loose motions, ascites and pain in abdomen and one patient had ankylosing spondylitis and cervical spondylitis. On clinical grounds only one case was diagnosed as primary amyloidosis of light chain type, who presented initially with cervical lymphadenopathy and 4 years later with nephrotic syndrome. About 72.72% cases had some chronic disease in the terms of tuberculosis, ankylosing spondylitis, chronic ulcerative colitis, lepromatous leprosy, rheumatoid arthritis and one patient had carcinoma caecum. Congo red stain was positive in both, light chain deposit disease (LCDD) and amyloidosis but polarizing microscope showed mixed birefringence (red, green, yellow) only in amyloidosis. In AFOG and PAS stain, amyloid appeared negative, only peripheral portion revealed blue and pink staining and central area appeared as cutout spaces. Congo red and methyl violet stains and potassium permanganate treatment was not helpful in distinguishing AL amyloidosis from secondary amyloidosis. Hence immunohistochemistry and myeloma profile is a must. It might be possible that in light chain amyloidosis, treatment with methotrexate and prednisolone may improve survival.
Renal Amyloidosis: A Clinicopathological Study From a Tertiary Care Hospital in Pakistan
Cureus, 2022
Introduction Systemic amyloidosis can affect any organ in the body, but the kidney is the most commonly involved site. It is characterized by the extracellular deposition of insoluble fibrillar proteins. Amyloid deposits can be identified histologically by Congo red stain, which gives apple-green birefringence under polarized light. Typing of renal amyloidosis is done by direct immunofluorescence on frozen tissue. The most common types of amyloidosis seen in renal tissue are amyloid light chain (AL) primary amyloidosis and amyloid A (AA) secondary amyloidosis. Although primary amyloidosis is considered the most common type in western countries, however, in the subcontinent region, secondary amyloidosis is more common. The spectrum of signs and symptoms in renal amyloidosis is variable including isolated proteinuria, nephrotic syndrome, hypertension, hypotension, and renal insufficiency. The present study aims to evaluate the incidence and aetiology of various types of renal amyloidosis, determine their distribution within the kidney, and study various clinicopathological features. Objective The present study aims to evaluate the aetiology and clinicopathological profile of renal amyloidosis, determine its various types, and their distribution within the kidney.