AA amyloidosis

General Information (adopted from Orphanet):

Synonyms, Signs: Inflammatory amyloidosis
Reactive amyloidosis
Amyloidosis AA
Secondary amyloidosis [IBIS]
Number of Symptoms 15
OrphanetNr: 85445
OMIM Id:
ICD-10: E85.3
UMLs: C0221014
MeSH:
MedDRA: 10039811
Snomed: 274945004
281034005

Prevalence, inheritance and age of onset:

Prevalence: 17
Inheritance:
Age of onset:

Disease classification (adopted from Orphanet):

Parent Diseases: Acquired amyloid peripheral neuropathy
 -Rare neurologic disease
Amyloidosis
 -Rare systemic or rheumatologic disease
Non-familial restrictive cardiomyopathy
 -Rare cardiac disease
Secondary glomerular disease
 -Rare renal disease

Comment:

Secondary amyloidosis is characterized by reactive amyloid fibrils, which are acute-phase reactants produced in response to systemic inflammation such as tuberculosis, leprosy, rheumatoid arthritis, familial Mediterranean fever, inflammatory bowel syndrome, chronic lung diseases, and chronic infections (PMID:16107109). Secondary amyloidosis (AA amyloidosis) includes sub-diseases like e.g. reactive systemic amyloidosis and secondary cutaneous amyloidosis (PMID:26788465). Cardiac involvement in secondary amyloidosis is not common but, when present, carries a poor prognosis. In one study of patients with rheumatic disease who were found to have secondary amyloidosis and cardiac involvement, the survival rate was 31% at 5 years (PMID:16107109).

Symptom Information: Sort by abundance 

1
 (HPO:0001917) Renal amyloidosis Very frequent [IBIS] 26788465 IBIS 8 / 7739
2
 (HPO:0000100) Nephrotic syndrome Very frequent [IBIS] 26788465 IBIS 83 / 7739
3
 (HPO:0012211) Abnormal renal physiology Very frequent [IBIS] 26788465 IBIS 23 / 7739
4
 (HPO:0000093) Proteinuria Very frequent [IBIS] 26788465 IBIS 169 / 7739
5
 (HPO:0012280) Hepatic amyloidosis Very frequent [IBIS] 26788465 IBIS 4 / 7739
6
 (HPO:0001678) Atrioventricular block Rare [IBIS] 10659664 IBIS 59 / 7739
7
 (HPO:0200126) Amyolid cardiomyopathy 16107109 IBIS 2 / 7739
8
 (HPO:0001713) Abnormality of cardiac ventricle 16107109 IBIS 4 / 7739
9
 (HPO:0003116) Abnormal echocardiogram 16107109 IBIS 33 / 7739
10
 (HPO:0004756) Ventricular tachycardia Rare [IBIS] 10659664 IBIS 55 / 7739
11
 (HPO:0011034) Amyloidosis 26788465 IBIS 12 / 7739
12
 (OMIM) Chronic inflammation Frequent [IBIS] 26788465 IBIS 2 / 7739
13
 (OMIM) Polymorphic ventricular tachycardia Rare [IBIS] 10659664 IBIS 2 / 7739
14
 (MedDRA:10039811) Secondary amyloidosis Very frequent [IBIS] 26788465 IBIS 1 / 7739
15
 (MedDRA:10007509) Cardiac amyloidosis Rare [IBIS] 10659664 IBIS 5 / 7739

Associated genes:

ClinVar (via SNiPA)

Gene symbol Variation Clinical significance Reference

Additional Information:

Description: (OMIM) Secondary amyloidosis (AA amyloidosis) is a disorder characterized by the extracellular tissue deposition of fibrils that are composed of fragments of serum amyloid A (SAA) protein. It may occur as a complication of some other chronic infections or inflammatory ...