Renal Involvement in Hereditary Transthyretin Amyloidosis: An Italian Single-Centre Experience

Objective: Hereditary transthyretin amyloidosis (ATTRv) represents a diagnostic challenge considering the great variability of clinical presentation and multiorgan involvement. In the present study, we report the prevalence of kidney involvement and kidney function over time in a cohort of ATTRv pat...

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Bibliographic Details
Published in:Brain sciences 2021-07, Vol.11 (8), p.980
Main Authors: Ferraro, Pietro Manuel, D’Ambrosio, Viola, Di Paolantonio, Andrea, Guglielmino, Valeria, Calabresi, Paolo, Sabatelli, Mario, Luigetti, Marco
Format: Article
Language:English
Subjects:
amyloid
Amyloidosis
Apolipoproteins
Cardiovascular disease
Creatinine
Epidermal growth factor receptors
Genetic diversity
Glomerular filtration rate
Immunoglobulins
Kidney diseases
Kidneys
Mutation
nephropathy
neuropathy
Patients
Proteins
proteinuria
Sudoscan
Transthyretin
TTR
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Summary:Objective: Hereditary transthyretin amyloidosis (ATTRv) represents a diagnostic challenge considering the great variability of clinical presentation and multiorgan involvement. In the present study, we report the prevalence of kidney involvement and kidney function over time in a cohort of ATTRv patients with different transthyretin gene mutations. Patients and Methods: For this study, we systematically collected data from all patients with a diagnosis of ATTRv followed at the Neurology Unit of Fondazione Policlinico Universitario A. Gemelli IRCCS. Kidney involvement was defined as presence of estimated glomerular filtration rate (eGFR) < 60 mL/min/1.73 m2 obtained with CKD-EPI equation, abnormal urinary protein excretion (UPE) (>150 mg/24 h) and/or albuminuria > 30 mg/24 h (or mg/g creatinine). The analysis included data from 46 patients with 122 measurements of serum creatinine. Results: Among the 46 patients included in the analysis, kidney involvement was present in 37%, with 15% showing reduced eGFR and 22% abnormal UPE (63% of patients with available UPE data). No single predictor was associated with either eGFR values or its slope over time. Conclusions: Kidney involvement is quite common in patients with ATTRv regardless of the underlying genetic variant. In particular, abnormal UPE appears to be a common feature of the disease.
ISSN:2076-3425
2076-3425
DOI:10.3390/brainsci11080980