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Start free with EleplanALys amyloidosis
ORPHA:93561Clinical subtype
Also called Familial amyloid nephropathy due to lysozyme variant · Familial renal amyloidosis due to lysozyme variant · Hereditary amyloid nephropathy due to lysozyme variant · Hereditary renal amyloidosis due to lysozyme variant · Lysozyme amyloidosis
What it is
A rare, hereditary amyloidosis with primary renal involvement characterized by amyloid deposition in the kidney glomeruli and medulla, gastrointestinal tract, liver, spleen and slow disease progression. Symptoms and signs include nausea, vomiting, dyspepsia, gastritis, gastrointestinal hemorrhage, abdominal pain, hepatic rupture, sicca syndrome, purpura and petechiae, lymphadenopathy and renal dysfunction.
Key facts
- Prevalence
- <1 / 1 000 000
- Inheritance
- Autosomal dominant
- Classified as
- Clinical subtype
Recorded for the broader condition
- Age of onset
- All agesHereditary amyloidosis with primary renal involvement
Orphanet records these for the broader condition rather than for this specific form. They are a starting point, not a figure for this subtype — subtypes often differ, and some are defined by being rarer than the condition they sit under.
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.
Gene
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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