Systemic amyloidosis

  title={Systemic amyloidosis},
  author={Ashutosh D. Wechalekar and Julian D. Gillmore and Philip N. Hawkins},
  journal={The Lancet},
Cardiac Amyloidosis: A Review of Current Imaging Techniques
Key radiological features of CA are discussed, focusing on the two most common types; immunoglobulin light chain (AL) and transthyretin (ATTR) CA, highlighting recent advances in imaging techniques particularly in respect of their clinical application and utility in diagnosis of CA as well as for tracking disease change over time.
Amyloid cardiomyopathy.
  • Lucie Karafiatova, T. Pika
  • Medicine, Biology
    Biomedical papers of the Medical Faculty of the University Palacky, Olomouc, Czechoslovakia
  • 2017
An overview of amyloidsosis is provided, providing information about the characteristics of cardiac amyloidosis, and a structured approach to diagnosis, treatment and prognosis of this condition is presented.
Advances in diagnostics and therapy of systemic amyloidoses
Significant progress has been recently achieved concerning the first type of strategies in two most common systemic amyloidoses: AL and ATTR.
Diagnosis of amyloid beyond Congo red.
  • M. Picken
  • Medicine, Biology
    Current opinion in nephrology and hypertension
  • 2020
Despite significant advances in the treatment of the systemic amyloidoses, outcomes remain poor, primarily due to delays in diagnosis, and Precise diagnosis of the amyloids protein type is critical for treatment selection.
Cardiac amyloidosis
Recent developments in diagnosis and management of cardiac amyloidosis are reviewed, with a focus on transthyretin type cases.
Amyloidosis with Cardiac Involvement: Identification, Characterization, and Management
Although cardiac amyloidosis contributes significantly to the morbidity and mortality associated with systemic disease, new tools are available to assist with diagnosis, prognosis, and management.
Recent advances in the diagnosis and management of amyloid cardiomyopathy
Treatment of CA includes the management of restrictive cardiomyopathy with preserved or reduced ejection fraction in addition to treating the amyloid deposition, and it is likely that a multistep treatment approach will be optimal for both AL CA and ATTR CA.
A Case of Lambda AL Type Skeletal Amyloidosis with Vascular Myopathy Achieved Clinical Response after Autologous Stem Cell Transplant
Early diagnosis of amyloidosis is a key to better outcomes, this results in disease progression-not to mention a more difficult time treating it-before it is finally discovered in a patient.
Mass spectrometry analysis for amyloidosis typing - is the future bright for its clinical implementation?
With a new era of amyloid therapeutics upon us, such as antisense therapies, transthyretin stabilizers, and anti-amyloid fibril antibodies, accurate diagnosis has become more important than ever.


Transthyretin-derived senile systemic amyloidosis: clinicopathologic and structural considerations
It is suggested that ATTR fibrils may develop both from full-length TTR and from C-terminal TTR fragments and that the N- terminal part is not necessary for the fibril integrity.
Pathogenesis, diagnosis and treatment of systemic amyloidosis.
  • M. Pepys
  • Biology, Medicine
    Philosophical transactions of the Royal Society of London. Series B, Biological sciences
  • 2001
Radiolabelled SAP is an extremely useful, safe, specific, non-invasive, quantitative tracer for scintigraphic imaging of systemic amyloid deposits, and its use has demonstrated that elimination of the supply ofAmyloid fibril precursor proteins leads to regression of amyloids deposits with clinical benefit.
Natural history and outcome in systemic AA amyloidosis.
The effects of renal dysfunction dominate the course of AA amyloidosis, which is associated with a relatively favorable outcome in patients with SAA concentrations that remain in the low-normal range (<4 mg per liter).
Systemic amyloidosis and the gastrointestinal tract
No specific treatments for the gastrointestinal symptoms of systemic amyloidosis are available; however, case reports and small published series encourage nutritional support for patients with motility disorders and pharmacological agents for treatment of diarrhea.
Diagnosis and Management of the Cardiac Amyloidoses
The purpose of this review is to familiarize the reader with the clinical features of amyloidosis and to address the approach to the patient with this disease, focusing on the various types of ameloidosis, their prognosis and treatment.
Misdiagnosis of hereditary amyloidosis as AL (primary) amyloidosis.
A genetic cause should be sought in all patients with amyloidsosis that is not the reactive systemic amyloid A type and in whom confirmation of the light-chain (AL) type cannot be obtained.
Evaluation of systemic amyloidosis by scintigraphy with 123I-labeled serum amyloid P component.
Scintigraphy after the injection of 123I-SAP can be used for diagnosing, locating, and monitoring the extent of systemic amyloidosis.
Systemic Cardiac Amyloidoses: Disease Profiles and Clinical Courses of the 3 Main Types
AL, ATTRm, and ATTRwt should be considered 3 different cardiac diseases, probably characterized by different pathophysiological substrates and courses.
Renal Transplantation in Systemic Amyloidosis—Importance of Amyloid Fibril Type and Precursor Protein Abundance
  • J. Pinney, H. Lachmann, J. Gillmore
  • Medicine, Biology
    American journal of transplantation : official journal of the American Society of Transplantation and the American Society of Transplant Surgeons
  • 2013
Outcomes following RTx were generally excellent in these diseases, reflecting their slow natural history; median graft survival was 13.1 years and post‐RTx chemotherapy was administered successfully to four AL patients.