What is the NIS used to measure in ATTR?

The Neuropathy Impairment Score measures sensory, motor, and autonomic neuropathy on a 0-244 scale; lower is better. NIS progression ≥2 points/year indicates inadequate disease control.

How does patisiran differ from inotersen for hATTR?

Patisiran (IV RNAi every 3 weeks) and inotersen (SC antisense oligonucleotide weekly) both silence TTR mRNA; patisiran also showed cardiac benefits; inotersen has thrombocytopenia monitoring requirement.

What are the cardiac staging criteria for ATTR?

Granzyme staging uses NT-proBNP and troponin: Stage 1 (both low): median survival 70 months; Stage 2 (one elevated): 46 months; Stage 3a (both elevated, NT-proBNP 3000): 3.5 months.

What Val30Met variant predicts earlier disease onset?

Val30Met (p.Val50Met) causes most hereditary ATTR polyneuropathy; early-onset (before age 50) is endemic in Portugal and Japan; late-onset is sporadic worldwide with milder course.

When should liver transplant be considered for hATTR?

Liver transplant remains an option for early-stage polyneuropathy before significant cardiac involvement; it prevents further amyloid deposition but does not clear existing cardiac amyloid.

Transthyretin Amyloidosis (ATTR) Progression Calculator

Calculates ATTR amyloidosis progression using the Neuropathy Impairment Score (NIS), FAS composite, polyneuropathy disability score, and cardiac biomarkers (NT-proBNP, troponin) to guide patisiran/inotersen dosing.

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Frequently Asked Questions

What is the NIS used to measure in ATTR?

How does patisiran differ from inotersen for hATTR?

What are the cardiac staging criteria for ATTR?

What Val30Met variant predicts earlier disease onset?

When should liver transplant be considered for hATTR?