Hungarian National Systemic Amyloidosis Registry

Sex (male or female as recorded in the medical record) of participants at the time of systemic amyloidosis diagnosis

Age of participants in years at the time of systemic amyloidosis diagnosis

Participant place of residence recorded at the time of systemic amyloidosis diagnosis, reported at postal code (ZIP code) level. No street-level address information will be collected

Healthcare institution where the participant received the initial diagnosis of systemic amyloidosis

Classification of systemic amyloidosis subtype (e.g., AL, ATTR, AA, or other specified subtype) as determined at the time of diagnosis according to clinical, histological, and/or laboratory criteria recorded in the medical record

Date when the first symptoms attributable to systemic amyloidosis were first documented or reported by the participant, as recorded in the medical record. Symptoms refer to clinical manifestations later attributed to systemic amyloidosis

Date on which systemic amyloidosis was first formally diagnosed based on clinical, histological, and/or laboratory criteria as documented in the medical record. The date corresponds to the first confirmed diagnosis recorded in the healthcare system

Time interval in days between first reported symptom onset attributable to systemic amyloidosis and date of first confirmed diagnosis of systemic amyloidosis. The variable is calculated as the difference between the date of symptom onset and the date of diagnosis based on medical record data

Categorized main presenting symptom prompting diagnostic evaluation for systemic amyloidosis, based on predefined clinical categories (cardiac, renal, neurological, gastrointestinal, constitutional, or other). Classification is based on medical record review at the time of diagnosis

Presence of organ involvement due to systemic amyloidosis at the time of diagnosis, categorized as cardiac, renal, neurological, gastrointestinal, or other involvement based on predefined clinical criteria documented in the medical record

Whether a tissue biopsy was performed during the diagnostic evaluation of systemic amyloidosis. Biopsy refers to any tissue sampling procedure (e.g., fat pad, bone marrow, or organ biopsy) performed as part of the diagnostic workup, as documented in the medical record

Whether a bone scintigraphy study (e.g., 99mTc-PYP, 99mTc-DPD, or 99mTc-HMDP) was performed during the diagnostic evaluation of systemic amyloidosis, as documented in the medical record

Whether a cardiac magnetic resonance imaging (MRI) study was performed during the diagnostic evaluation of systemic amyloidosis, as documented in the medical record

Whether carpal tunnel syndrome was present or previously diagnosed at the time of systemic amyloidosis diagnosis, as documented in the medical record

Polyneuropathy Disability (PND) score at the time of systemic amyloidosis diagnosis, assessed using the standard PND classification (Grade 0-IV), as documented in the medical record. The PND score reflects the severity of peripheral neuropathy

Plasma N-terminal pro-B-type natriuretic peptide (NT-proBNP) concentration measured at the time of systemic amyloidosis diagnosis, as recorded in the medical record. Values are reported in standard clinical units (e.g., pg/mL)

Serum high-sensitivity cardiac troponin T (hs-cTnT) concentration measured at the time of systemic amyloidosis diagnosis, as recorded in the medical record. Values are reported in standard clinical units (e.g., ng/L)

Estimated glomerular filtration rate (eGFR) measured at the time of systemic amyloidosis diagnosis, calculated using a standardized equation (e.g., CKD-EPI), as recorded in the medical record. Values are reported in mL/min/1.73 m²

Serum concentration of kappa free light chains measured at the time of systemic amyloidosis diagnosis, as recorded in the medical record. Values are reported in mg/L using a standardized immunoassay

Serum concentration of lambda free light chains measured at the time of systemic amyloidosis diagnosis, as recorded in the medical record. Values are reported in mg/L using a standardized immunoassay

Result of serum immunofixation electrophoresis performed at the time of systemic amyloidosis diagnosis. Results are categorized as positive or negative, and when positive, the detected monoclonal protein type (e.g., kappa, lambda, or other) is recorded as documented in the medical record

Interventricular septal thickness measured by transthoracic echocardiography during diagnostic evaluation of systemic amyloidosis. Measurement is reported in millimeters (mm) as recorded in the medical record

Presence of clinically diagnosed aortic valve stenosis at the time of systemic amyloidosis diagnosis, based on echocardiographic findings and/or medical record documentation

Heart failure functional status assessed using the New York Heart Association (NYHA) classification (Class I-IV) at the time of systemic amyloidosis diagnosis, as documented in the medical record

Presence of low voltage QRS complexes on ECG at diagnosis

Presence of conduction abnormalities (e.g., AV block, bundle branch block) on ECG at diagnosis

Presence of atrial fibrillation on ECG at diagnosis

Presence of pharmacological treatment for heart failure at the time of systemic amyloidosis diagnosis. Heart failure therapy includes guideline-directed medical treatments such as diuretics, beta-blockers, ACE inhibitors, or ARBs, as documented in the medical record

Presence of transthyretin (ATTR) amyloidosis-specific disease-modifying therapy at the time of registry enrollment (data entry). ATTR-specific therapy includes transthyretin-targeted treatments such as tafamidis or other approved disease-modifying agents, as documented in the medical record

Whether genetic testing (gene sequencing) was performed during the diagnostic evaluation of systemic amyloidosis, as documented in the medical record

Result of genetic testing performed during diagnostic workup for systemic amyloidosis. Results are categorized as negative or positive for pathogenic variants. If positive, the specific gene mutation identified (e.g., TTR variants or other relevant pathogenic mutations) is recorded as documented in the medical record

Annual mortality rate

Annual hospitalization rate