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Start free with EleplanWild type ATTR amyloidosis
ORPHA:330001Disease
Also called ATTRwt amyloidosis · ATTRwt-related amyloidosis · Senile systemic amyloidosis · Wild type ATTR-related amyloidosis
What it is
A common form of systemic amyloidosis characterized by deposition of wild type transthyretin predominantly in the heart and the soft tissues (mainly the carpal tunnel region, lumbar canal and tendons).
Key facts
- Prevalence
- 1-5 / 10 000
- Age of onset
- Adult, Elderly
- Inheritance
- Not applicable
- Classified as
- Disease
Prevalence figures describe populations, not individuals, and are often estimates from a single study. Where the figure is an incidence or a birth prevalence, it says so.
Signs and symptoms
Very common80–99%
9Common30–79%
17- Abnormal autonomic nervous system physiology
- Arrhythmia
- Atrial fibrillation
- Autonomic bladder dysfunction
- Bowel incontinence
- Chronic diarrhea
- Constrictive median neuropathy
- Decreased QRS voltage
- Elevated circulating alkaline phosphatase concentration
- Gastrointestinal dysmotility
- Hepatomegaly
- Impaired vibratory sensation
- Increased circulating NT-proBNP concentration
- Increased circulating troponin I concentration
- Increased circulating troponin T concentration
- Intermittent diarrhea
- Sensory neuropathy
Sometimes5–29%
15- Abnormal atrioventricular conduction
- Aortic valve stenosis
- Ascites
- Bradycardia
- Hypoesthesia
- Myocardial infarction
- Nephropathy
- Nephrotic syndrome
and 7 more in this range
These ranges are how often a sign appears across everyone diagnosed, not a prediction for one person — most people have some of these and not others. Orphanet also records findings that are specifically absent in this disease; those are in the data but not listed here.
ICD-10 codes
ICD-10 has no distinct code for most rare diseases, so many are filed under the nearest available one. A shared code identifies the group, not this disease — the ORPHAcode above is what identifies it.
Related health topics
Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.
Cross-references
Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.
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