Emerging treatment approaches for the systemic amyloidoses

Dember, Laura M.

doi:10.1111/j.1523-1755.2005.00535.x

Cited by 32 publications

(22 citation statements)

References 83 publications

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“…Kidney involvement usually presents as nephrotic syndrome with progressive worsening of renal function. In a small proportion of patients (approximately 10%), amyloid deposition occurs in the renal vasculature or tubulointerstitium, causing renal dysfunction without significant proteinuria (13). Amyloid deposition in the heart results in rapidly progressive heart failure because of restrictive cardiomyopathy.…”

Section: Clinical Presentationsmentioning

confidence: 99%

Light-Chain (AL) Amyloidosis

Sanchorawala

2006

Clinical Journal of the American Society of Nephrology

206

163

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Light-chain (AL) amyloidosis is the most common form of systemic amyloidosis and is associated with an underlying plasma cell dyscrasia. The disease often is difficult to recognize because of its broad range of manifestations and what often are vague symptoms. The clinical syndromes at presentation include nephrotic-range proteinuria with or without renal dysfunction, hepatomegaly, congestive heart failure, and autonomic or sensory neuropathy. Recent diagnostic and prognostic advances include the serum free light-chain assay, cardiac magnetic resonance imaging, and serologic cardiac biomarkers. Treatment strategies that have evolved during the past decade are prolonging survival and preserving organ function in patients with this disease. This review outlines approaches to diagnosis, assessment of disease severity, and treatment of AL amyloidosis.

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Section: Clinical Presentationsmentioning

confidence: 99%

Light-Chain (AL) Amyloidosis

Sanchorawala

2006

Clinical Journal of the American Society of Nephrology

206

163

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“…Localized amyloidosis, in comparison, is defined by the production of precursor proteins in the same location as amyloid deposition. 4,5 Systemic amyloidosis can be acquired, as in immunoglobulin light chain (AL) amyloidosis caused by an underlying clonal plasma cell dyscrasia, or hereditary, as most commonly occurs by modification of the transthyretin gene (mutant ATTR). Wild-type transthyretin can also form amyloid disease in older patients, in a process termed senile systemic amyloidosis.…”

Section: Introductionmentioning

confidence: 99%

Amyloidosis of the gastrointestinal tract: a 13-year, single-center, referral experience

et al. 2012

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“…The features for AL-amyloidosis are essentially the same as in LCDD. AL-amyloid is a disease of older adults, and proteinuria is often a presenting finding along with renal insufficiency (7). A paraprotein is found in over 90% of cases and is usually in 70% of cases, not unlike our patient.…”

mentioning

confidence: 84%