Methods of treating transthyretin (TTR) mediated amyloidosis

1. A method of treating polyneuropathy in a patient with hereditary transthyretin-mediated amyloidosis (hATTR), the method comprising administering a dose of patisiran with a formulation as described in Table 1 to the patient, wherein the dose is 0.3 mg siRNA per kg body weight, and the patisiran is administered via IV infusion once every three weeks, and the patient receives a premedication before infusion to reduce the risk of infusion-related reactions, whereby the method reduces a Neuropathy Impairment Score (NIS) or a modified NIS (mNIS+7) or arrests an increase in a NIS or a mNIS+7 in the patient.

2. The method of claim 1 , wherein the patient receives the premedication on the evening before and the day of infusion.

3. The method of claim 1 , wherein the premedication comprises dexamethasone, acetaminophen, diphenhydramine, and ranitidine.

4. The method of claim 1 , wherein the premedication comprises dexamethasone, acetaminophen, cetirizine, and ranitidine.

5. The method of claim 1 , wherein the premedication comprises dexamethasone, paracetamol (acetaminophen), an H2 blocker and an H1 blocker.

6. The method of claim 5 , wherein the H2 blocker is ranitidine or famotidine.

7. The method of claim 5 , wherein the H1 blocker is cetirizine, hydroxyzine or fexofenadine.

8. The method of claim 1 , wherein the patisiran is administered via a 70 minute infusion of 1 mL/min for 15 minutes followed by 3 mL/min for 55 minutes.

9. The method of claim 1 , wherein the patisiran is administered via a 70 minute infusion of 1 mL/min for 15 minutes followed by 3 mL/min for 55 minutes and the premedication comprises dexamethasone, paracetamol (acetaminophen), an H2 blocker and an H1 blocker.

10. The method of claim 1 , wherein the patient has cardiomyopathy.

11. The method of claim 1 , wherein the patient does not have cardiomyopathy.