Abstract
Introduction
Latvia is a Northern European country with a 1.9 million population. An observational cohort study was started in January 2020 with long-term follow-up of patients referred to Pauls Stradiņš Clinical University Hospital, the main referral center for amyloidosis in Latvia.
Methods
Inclusion criteria were any systemic or localized amyloidosis confirmed by tissue biopsy with Congo red stain or a positive 99mTc-PYP scan for transthyretin (ATTR) amyloidosis.
Results
54 patients were included, 26 (46%) female. 20 patients (37%) died during follow-up. The most prevalent type was light chain (AL) amyloidosis (n=33 [61%]) with 26 cases of systemic disease and 7 localized. The greatest proportion (n=9 [38%]) of AL cases were diagnosed at stage IIIb (Mayo2004/European). AA was diagnosed in 9 patients, ATTR in 7, two of them with TTR mutations (Arg54Thr, Val30Met). One case of lysozyme amyloidosis was registered.
Kidney involvement was present in 37 (69%) cases, cardiac in 27 (50%), polyneuropathy in 21 (39%), orthostatic hypotension in 12 (22%). Mortality was not associated with type but was associated with mean age: 60±14 years in survivors versus 68±8 in non-survivors.
We observed an increase in AL and ATTR cases and a decrease in AA with AL dominating as the leading type in 2022-2023. Our data demonstrates a relatively small proportion of ATTR.
Conclusions
Since the initiation of the national registry, amyloidosis has become more widely recognized as a clinical condition in Latvia with increasing incidence of AL type. ATTR remains underdiagnosed.
Latvia is a Northern European country with a 1.9 million population. An observational cohort study was started in January 2020 with long-term follow-up of patients referred to Pauls Stradiņš Clinical University Hospital, the main referral center for amyloidosis in Latvia.
Methods
Inclusion criteria were any systemic or localized amyloidosis confirmed by tissue biopsy with Congo red stain or a positive 99mTc-PYP scan for transthyretin (ATTR) amyloidosis.
Results
54 patients were included, 26 (46%) female. 20 patients (37%) died during follow-up. The most prevalent type was light chain (AL) amyloidosis (n=33 [61%]) with 26 cases of systemic disease and 7 localized. The greatest proportion (n=9 [38%]) of AL cases were diagnosed at stage IIIb (Mayo2004/European). AA was diagnosed in 9 patients, ATTR in 7, two of them with TTR mutations (Arg54Thr, Val30Met). One case of lysozyme amyloidosis was registered.
Kidney involvement was present in 37 (69%) cases, cardiac in 27 (50%), polyneuropathy in 21 (39%), orthostatic hypotension in 12 (22%). Mortality was not associated with type but was associated with mean age: 60±14 years in survivors versus 68±8 in non-survivors.
We observed an increase in AL and ATTR cases and a decrease in AA with AL dominating as the leading type in 2022-2023. Our data demonstrates a relatively small proportion of ATTR.
Conclusions
Since the initiation of the national registry, amyloidosis has become more widely recognized as a clinical condition in Latvia with increasing incidence of AL type. ATTR remains underdiagnosed.
| Original language | English |
|---|---|
| Article number | 363 |
| Pages (from-to) | S151 |
| Number of pages | 1 |
| Journal | Amyloid: The Journal of Protein Folding Disorders |
| Volume | 31 |
| Issue number | Suppl.1 |
| Publication status | Published - May 2024 |
| Event | XIX International Symposium on Amyloidosis - Mayo Civic Center, Rochester, United States Duration: 26 May 2024 → 30 May 2024 Conference number: 19 https://www.oneamyloidosisvoice.com/news-meeting/isa-symposium-amyloidosis/ |
Field of Science*
- 3.2 Clinical medicine
Publication Type*
- 3.4. Other publications in conference proceedings (including local)