Kyle Cass, Lila Luna, William Kivlin, Buckley Fechter, Hari Paudel
{"title":"淀粉样轻链淀粉样变性并发急性心力衰竭和肾衰竭。","authors":"Kyle Cass, Lila Luna, William Kivlin, Buckley Fechter, Hari Paudel","doi":"","DOIUrl":null,"url":null,"abstract":"<p><strong>Introduction: </strong>Amyloid light chain (AL) amyloidosis is a multisystem disease with significant variability in patient presentation. This case describes the presentation and workup of a patient with unique multiorgan involvement on initial presentation.</p><p><strong>Case presentation: </strong>A 69-year-old African American male presented with weakness, leg swelling, and shortness of breath. Initial workup demonstrated acute heart failure and acute-on-chronic renal failure with nephrotic range proteinuria (5.78 protein to creatinine ratio). Further workup showed elevated serum protein electrophoresis, urine protein electrophoresis, and light chains. Subsequent renal biopsy showed lambda-restricted AL-type renal amyloidosis.</p><p><strong>Discussion: </strong>A variety of systemic presentations have been described in the literature; however, concurrent heart and renal failure as primary presentation is uncommon.</p><p><strong>Conclusions: </strong>This case emphasizes the importance of considering systemic inflammatory diseases, such as amyloidosis, in the differential diagnoses of patients with unexplained multiorgan disease. Early diagnosis and treatment initiation are essential for improving patient outcomes. Improved recognition of common clinical manifestations and laboratory abnormalities will likely improve outcomes through earlier diagnosis.</p>","PeriodicalId":94268,"journal":{"name":"WMJ : official publication of the State Medical Society of Wisconsin","volume":null,"pages":null},"PeriodicalIF":0.0000,"publicationDate":"2024-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Concurrent Acute Heart Failure and Renal Failure in Amyloid Light Chain Amyloidosis.\",\"authors\":\"Kyle Cass, Lila Luna, William Kivlin, Buckley Fechter, Hari Paudel\",\"doi\":\"\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><strong>Introduction: </strong>Amyloid light chain (AL) amyloidosis is a multisystem disease with significant variability in patient presentation. This case describes the presentation and workup of a patient with unique multiorgan involvement on initial presentation.</p><p><strong>Case presentation: </strong>A 69-year-old African American male presented with weakness, leg swelling, and shortness of breath. Initial workup demonstrated acute heart failure and acute-on-chronic renal failure with nephrotic range proteinuria (5.78 protein to creatinine ratio). Further workup showed elevated serum protein electrophoresis, urine protein electrophoresis, and light chains. Subsequent renal biopsy showed lambda-restricted AL-type renal amyloidosis.</p><p><strong>Discussion: </strong>A variety of systemic presentations have been described in the literature; however, concurrent heart and renal failure as primary presentation is uncommon.</p><p><strong>Conclusions: </strong>This case emphasizes the importance of considering systemic inflammatory diseases, such as amyloidosis, in the differential diagnoses of patients with unexplained multiorgan disease. Early diagnosis and treatment initiation are essential for improving patient outcomes. Improved recognition of common clinical manifestations and laboratory abnormalities will likely improve outcomes through earlier diagnosis.</p>\",\"PeriodicalId\":94268,\"journal\":{\"name\":\"WMJ : official publication of the State Medical Society of Wisconsin\",\"volume\":null,\"pages\":null},\"PeriodicalIF\":0.0000,\"publicationDate\":\"2024-07-01\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"WMJ : official publication of the State Medical Society of Wisconsin\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"\",\"JCRName\":\"\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"WMJ : official publication of the State Medical Society of Wisconsin","FirstCategoryId":"1085","ListUrlMain":"","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"","JCRName":"","Score":null,"Total":0}
Concurrent Acute Heart Failure and Renal Failure in Amyloid Light Chain Amyloidosis.
Introduction: Amyloid light chain (AL) amyloidosis is a multisystem disease with significant variability in patient presentation. This case describes the presentation and workup of a patient with unique multiorgan involvement on initial presentation.
Case presentation: A 69-year-old African American male presented with weakness, leg swelling, and shortness of breath. Initial workup demonstrated acute heart failure and acute-on-chronic renal failure with nephrotic range proteinuria (5.78 protein to creatinine ratio). Further workup showed elevated serum protein electrophoresis, urine protein electrophoresis, and light chains. Subsequent renal biopsy showed lambda-restricted AL-type renal amyloidosis.
Discussion: A variety of systemic presentations have been described in the literature; however, concurrent heart and renal failure as primary presentation is uncommon.
Conclusions: This case emphasizes the importance of considering systemic inflammatory diseases, such as amyloidosis, in the differential diagnoses of patients with unexplained multiorgan disease. Early diagnosis and treatment initiation are essential for improving patient outcomes. Improved recognition of common clinical manifestations and laboratory abnormalities will likely improve outcomes through earlier diagnosis.