Table 1.
Diseases with known peripheral nervous system involvement and a (potential) link to amyloid proteins.
| Disease | Amyloid Protein | Acquired/ Hereditary |
Local/ Systemic |
Peripheral Nervous System Involvement | Prevalence/Incidence Disease | Prevalence Disease w/w * | Prevalence/Incidence PN (% of Patients) |
|---|---|---|---|---|---|---|---|
| Familial amyloid polyneuropathy | Transthyretin (hATTR) | Hereditary | Systemic | Polyneuropathy Autonomic disturbances Carpal tunnel syndrome [41] |
10,186 persons w/w (range: 5526–38,468)/UN [42] |
0.00013% | UN (it develops in the majority of patients/UN [42,43] |
| Apolipoprotein A-I (AApoAI) |
Hereditary | Systemic | Polyneuropathy [44] | UN/UN | UN | UN/UN | |
| Gelsolin (HGA) | Hereditary | Systemic | Cranial neuropathy Polyneuropathy [28] |
400 to 1000 gene carriers in Finland/UN [45] |
0.01% | UN/UN | |
| Immunoglobulin light-chain amyloidosis | Ig light-chain | Acquired | Systemic | Polyneuropathy Autonomic disturbances Carpal tunnel syndrome [46,47] |
40.5 cases per million in the US/9.7 to 14.0 cases per million per year in US [48] |
0.004% | 15–20%/UN [49] |
| Dialysis-related amyloidosis | β2-microglobulin | Acquired | Systemic | Carpal tunnel syndrome Polyneuropathy [50,51] |
UN/UN (incidence > 95% in. patients > 15 years dialysis in US) [51] |
UN | UN/UN |
| Senile systemic amyloidosis | Transthyretin | Acquired | Systemic | Polyneuropathy Autonomic disturbances carpal tunnel syndrome [52] |
63/256 of the study population in Finland (25% > 80 years old)/UN [53] |
0.45% | UN/UN |
| Type 2 diabetes mellitus | IAPP | Acquired | Local/systemic | Polyneuropathy [54] |
463 million persons (aged 20–79 years) w/w (including T1DM&T2DM)/UN [55] | 5.4% | 31.5–45% [56,57] |
| Rheumatoid arthritis | SAA | Acquired | Systemic | Polyneuropathy [58] | 19,965,115 persons w/w/1,204,599 new cases w/w [59] | 0.26% | 39.19–75.28% [58] |
| Inflammatory bowel disease | SAA | Acquired | Systemic | Polyneuropathy [60,61] | 68 million persons w/w/70,000 new cases per year in USA [62,63] |
0.09% | UN/0.07% after 10 years of IBD [60,64] |
| Osteoarthritis | TTR, Apo-A1 | Acquired | Systemic | Polyneuropathy [65] | 303 million persons w/w (80% of people > 75 years)/14.93 million new cases w/w [66,67] | 3.9% | UN/UN |
| Psoriatic Arthritis | SAA | Acquired | Systemic | Polyneuropathy [68] | 133 per 100,000 persons w/w/83 per 100,000 persons per year w/w [69] | 0.133% | UN/UN |
| Familial Mediterranean fever | SAA | Hereditary | Systemic | Polyneuropathy [22] | 100,000 persons in Turkey /UN (high among people from the eastern Mediterranean e.g., Arabs, Turks, Jews, and Armenians) [70,71] | 0.13% | UN/UN |
| Muckle–Wells syndrome | SAA | Hereditary | Systemic | Polyneuropathy [22] | Rare, MWS is one of the three clinical forms of CAPS and the prevalence of CAPS is 1–10 cases per million in France/UN [72] |
0.001% (based on max 10 per million) | UN/UN |
Abbreviations: CAPS = cryopyrin-associated periodic syndrome; HGA = hereditary gelsolin amyloidosis; hATTR = transthyretin-associated hereditary amyloidosis; IAPP = islet amyloid polypeptide; IGT = impaired glucose tolerance; MWS = Muckle–Wells syndrome; PN = peripheral neuropathy; SAA = serum amyloid A protein; T1DM = type 1 diabetes mellitus; T2DM = type 2 diabetes mellitus, UN = unknown; w/w = worldwide, * The calculated numbers for estimation of the worldwide prevalence are based on the: (1) same prevalence in every country; (2) world population = 7.6 billion, worldwide population aged over 80 years = 143 million, US population = 327 million, Finnish population = 5.5 million, Turkish population = 80 million. Although the prevalence of some diseases may be very different in different parts of the world, these (albeit artificial or fictional) numbers enable a global comparison of the prevalence of these diseases.