What is the Mainz Severity Score Index?

The MSSI is a validated scoring tool for Fabry disease that evaluates organ damage across renal, cardiac, neurological, and general domains, with scores ranging from 0-73.

What score indicates severe Fabry disease?

MSSI scores are classified as mild (0-20), moderate (21-40), and severe (>40), with higher scores indicating greater organ involvement.

Which organs does Fabry disease primarily affect?

Fabry disease primarily affects the kidneys, heart, peripheral nervous system, and skin due to accumulation of globotriaosylceramide.

How is enzyme replacement therapy monitored?

MSSI scores are tracked over time to assess response to enzyme replacement therapy with agalsidase alfa or beta.

Can females have severe Fabry disease?

Yes, heterozygous females can develop significant organ involvement and symptoms, though typically later and milder than affected males.

Fabry Disease Severity Score Calculator

Calculates the Mainz Severity Score Index (MSSI) for Fabry disease to assess organ involvement and disease burden across cardiac, renal, neurological, and general domains.

Use the full interactive calculator below, or

Related Calculators

Gaucher Score

Medical Calculators

Pompe Progression

Medical Calculators

Wilson Leipzig Score

Medical Calculators

Fabry Disease Severity Score Calculator

Related Calculators

Frequently Asked Questions

What is the Mainz Severity Score Index?

What score indicates severe Fabry disease?

Which organs does Fabry disease primarily affect?

How is enzyme replacement therapy monitored?

Can females have severe Fabry disease?