Insight into Latvian Amyloidosis Registry from 2020 to 2022

Activity: Talk or presentation types › Poster presentation

Description

To analyze diagnosis and treatment trends of amyloidosis patients in Latvia since the beginning of amyloidosis patient registry.. A country-wide ongoing observational cohort study was started in January 2020 with long-term follow-up in patients referred to Pauls Stradiņš Clinical University Hospital. Inclusion criteria were any type of systemic or localized amyloidosis confirmed by tissue biopsy with Congo Red stain or positive 99mTc-PYP cardiac scintigraphy scan for transthyretin (ATTR) amyloidosis and informed consent given by the patient.. From January 2020 to December 2022 the registry had enrolled 40 patients; 13 patients died during the follow-up period. 10 (25%) patients had AA amyloidosis, 19 (48%) had light chain (AL) amyloidosis (5 – local, 14 – systemic), 4 (10%) – ATTR, one patient had lysozyme amyloidosis. In 6 cases, the amyloid type was not identified. Registered AL cases increased from 6 in 2020 and 3 in 2021 to 10 in 2022. Clinically manifested renal involvement was present in 26 (65%) patients, cardiac in 13 (33%), sensorimotor polyneuropathy in 7 (18%), autonomic dysfunction in 8 (20%). In AL patients, 10 received anti-plasma cell treatment. Two received specific ATTR therapy.. Latvian amyloidosis patient cohort demonstrates predominance of AL amyloidosis but is still largely skewed towards AA amyloidosis in comparison with Western countries, likely because of active conduction of kidney biopsies in proteinuric patients. Since the onset of registry, diagnosis of AL has increased possibly due to growing awareness of the disease and referral to specialists. ATTR remains largely unrecognized and underdiagnosed partly due to lack of reimbursed treatment.
Period29 Mar 2023
Event titleRSU International Research Conference 2023: Knowledge for Use in Practice
Event typeConference
OrganiserRīga Stradiņš University
LocationRiga, LatviaShow on map
Degree of RecognitionInternational

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