Agalsidase Beta side effects: what 9,189 FDA adverse event reports show
Drug class
Hydrolytic Lysosomal Neutral Glycosphingolipid-specific Enzyme
Brand names
Fabrazyme
Who reports
9,189 reports · 52% serious · 699 with death outcome · 40% female
Used for
FABRAZYME ® is indicated for the treatment of adult and pediatric patients 2 years of age and older with confirmed Fabry disease. FABRAZYME is a hydrolytic lysosomal neutral glycosphingolipid-specific enzyme indicated for the treatment of adult and pediatric patients 2 years of age and older with confirmed Fabry disease. ( 1 )
What it is
Agalsidase beta is a recombinant human α-galactosidase A enzyme with the same amino acid sequence as the native enzyme. Purified agalsidase beta is a homodimeric glycoprotein with a molecular weight of approximately 100 kD.
Boxed warning
HYPERSENSITIVITY REACTIONS INCLUDING ANAPHYLAXIS Patients treated with enzyme replacement therapies have experienced life-threatening hypersensitivity reactions, including anaphylaxis. Anaphylaxis has occurred during the early course of enzyme replacement therapy and after extended duration of therapy.
Causality screen of the 40 strongest signals
What the badges mean
- Labeled adverse reaction The exact term appears in the label's warnings or adverse-reactions sections: a known, expected effect. The FAERS signal confirms the label rather than discovering anything.
- Labeled (related wording) The label describes this effect under different wording (e.g. 'neutropenia' for 'neutrophil count decreased'). Treated as known.
- Reporting / coding artifact An administrative, monitoring or exposure term (product issue, off-label use, 'test normal', overdose...). Not a biological reaction; a high PRR here reflects how reports are coded.
- Lab value / disease marker A laboratory result, investigation or disease-activity marker (antibody positive, CRP abnormal, swollen joint count) not described in the label. These usually mirror the monitored disease or testing protocol rather than a drug effect; not screened further.
- Not in label — unassessed Not found in the label text, not an artifact pattern. This is a genuine candidate for a formal causality assessment (Bradford Hill / WHO-UMC); it has NOT had one here.
Strongest disproportionality signals
Flagged terms (PRR ≥ 2, χ² ≥ 4, n ≥ 10), ranked by PRR; infinite PRRs (no comparator reports) excluded. The EBGM column is a second, shrinkage-based estimate (MGPS) with its 5th percentile EB05 and the BCPNN IC025; a pair flagged by PRR alone is marked PRR-only and should be read as fragile. The strata column recomputes the PRR on serious reports only and flags pairs whose reports are unusually skewed by sex or come mostly from consumers rather than clinicians. 962 further terms are in the interactive explorer. Reaction links open the term's cross-drug page.
| Reaction | Reports | PRR 95% CI · ROR · χ²/p | EBGM EB05 · IC025 | Label screen | Serious-only & strata | Trial cross-check |
|---|---|---|---|---|---|---|
| Globotriaosylceramide increased | 97 | 54,584.3 20,084.6–148,344.7ROR 55,166.6 · χ² 207,526 / p <1e-300 | 1,511.4 EB05 1,275.3 · IC025 +7.1 3/3 methods agree | Labeled (related wording) related wording | serious n=5 | no trial data cached |
| Globotriaosylsphingosine increased | 168 | 54,021.6 25,370.1–115,030.0ROR 55,027.6 · χ² 360,856 / p <1e-300 | 1,731.1 EB05 1,522.3 · IC025 +7.9 3/3 methods agree | Lab value / disease marker | serious n=8 · 93% clinician | no trial data cached |
| Fabry's disease | 23 | 4,314.2 2,147.4–8,667.5ROR 4,325.0 · χ² 32,542 / p <1e-300 | 660.8 EB05 463.7 · IC025 +4.8 3/3 methods agree | Not in label — unassessed | serious n=22 · 100% clinician | no trial data cached |
| Angiokeratoma | 27 | 1,105.0 697.5–1,750.6ROR 1,108.2 · χ² 19,241 / p <1e-300 | 485.3 EB05 350.2 · IC025 +5.0 3/3 methods agree | Not in label — unassessed | serious n=23 | no trial data cached |
| Antibody test | 19 | 792.0 469.7–1,335.4ROR 793.6 · χ² 10,525 / p <1e-300 | 368.3 EB05 249.2 · IC025 +4.4 3/3 methods agree | Reporting / coding artifact | – | no trial data cached |
| Neutralising antibodies positive | 58 | 377.3 285.9–497.9ROR 379.7 · χ² 18,323 / p <1e-300 | 292.1 EB05 234.3 · IC025 +6.0 3/3 methods agree | Lab value / disease marker | serious n=3 · ♀ 0% (drug 40%) | no trial data cached |
| Drug specific antibody absent | 19 | 228.7 142.7–366.5ROR 229.2 · χ² 3,706 / p <1e-300 | 172.1 EB05 116.4 · IC025 +4.3 3/3 methods agree | Lab value / disease marker | – | no trial data cached |
| Antibody test negative | 17 | 212.6 129.3–349.4ROR 213.0 · χ² 3,081 / p <1e-300 | 159.8 EB05 105.7 · IC025 +4.1 3/3 methods agree | Reporting / coding artifact | – | no trial data cached |
| Drug specific antibody present | 204 | 74.1 64.5–85.0ROR 75.7 · χ² 14,173 / p <1e-300 | 71.3 EB05 63.5 · IC025 +5.7 3/3 methods agree | Lab value / disease marker | serious n=16 · ♀ 9% (drug 40%) | no trial data cached |
| Anhidrosis | 11 | 63.3 34.8–115.3ROR 63.4 · χ² 597 / p 4e-132 | 56.0 EB05 33.4 · IC025 +3.1 3/3 methods agree | Not in label — unassessed | serious n=4 | no trial data cached |
| Renal transplant | 85 | 55.0 44.4–68.2ROR 55.5 · χ² 4,348 / p <1e-300 | 53.1 EB05 44.3 · IC025 +5.0 3/3 methods agree | Not in label — unassessed | serious n=84 · ♀ 8% (drug 40%) | no trial data cached |
| Cardiac hypertrophy | 24 | 50.5 33.7–75.6ROR 50.6 · χ² 1,091 / p 2e-239 | 47.7 EB05 33.7 · IC025 +4.0 3/3 methods agree | Not in label — unassessed | serious n=23 | no trial data cached |
| Antibody test positive | 35 | 48.5 34.7–67.8ROR 48.7 · χ² 1,549 / p <1e-300 | 46.4 EB05 34.9 · IC025 +4.3 3/3 methods agree | Lab value / disease marker | serious n=10 · ♀ 16% (drug 40%) | no trial data cached |
| Blood immunoglobulin g increased | 21 | 39.9 25.9–61.4ROR 40.0 · χ² 745 / p 2e-164 | 38.0 EB05 26.2 · IC025 +3.6 3/3 methods agree | Lab value / disease marker | serious n=8 | no trial data cached |
| Peritoneal dialysis | 14 | 39.0 23.0–66.1ROR 39.1 · χ² 473 / p 4e-105 | 36.6 EB05 23.2 · IC025 +3.2 3/3 methods agree | Not in label — unassessed | serious n=12 | no trial data cached |
| Urticaria generalised | 10 | 35.3 18.9–66.0ROR 35.4 · χ² 296 / p 2e-66 | 32.8 EB05 19.0 · IC025 +2.7 3/3 methods agree | Labeled (related wording) related wording | serious n=8 | no trial data cached |
| Hypertrophic cardiomyopathy | 19 | 28.4 18.1–44.7ROR 28.5 · χ² 470 / p 2e-104 | 27.4 EB05 18.5 · IC025 +3.3 3/3 methods agree | Not in label — unassessed | serious n=19 | no trial data cached |
| Myocardial necrosis marker increased | 18 | 25.6 16.1–40.7ROR 25.6 · χ² 396 / p 2e-88 | 24.7 EB05 16.5 · IC025 +3.1 3/3 methods agree | Lab value / disease marker | serious n=18 | no trial data cached |
| Glomerulonephritis membranous | 13 | 25.6 14.8–44.1ROR 25.6 · χ² 280 / p 5e-63 | 24.4 EB05 15.0 · IC025 +2.8 3/3 methods agree | Not in label — unassessed | serious n=13 | no trial data cached |
| Renal failure chronic | 85 | 25.1 20.3–31.1ROR 25.4 · χ² 1,925 / p <1e-300 | 24.8 EB05 20.6 · IC025 +4.1 3/3 methods agree | Labeled (related wording) related wording | serious PRR 26.7 · ♀ 9% (drug 40%) | no trial data cached |
| Infusion site extravasation | 65 | 24.1 18.9–30.7ROR 24.3 · χ² 1,401 / p <1e-300 | 23.7 EB05 19.2 · IC025 +3.9 3/3 methods agree | Labeled (related wording) related wording | serious n=18 | no trial data cached |
| Ventricular hypertrophy | 22 | 20.0 13.1–30.4ROR 20.0 · χ² 375 / p 8e-84 | 19.5 EB05 13.4 · IC025 +3.1 3/3 methods agree | Not in label — unassessed | serious n=20 | no trial data cached |
| Poor venous access | 87 | 19.4 15.7–23.9ROR 19.6 · χ² 1,487 / p <1e-300 | 19.2 EB05 16.0 · IC025 +3.8 3/3 methods agree | Not in label — unassessed | serious n=36 | no trial data cached |
| Infusion site rash | 11 | 18.6 10.3–33.6ROR 18.6 · χ² 164 / p 7e-38 | 17.3 EB05 9.4 · IC025 +2.4 3/3 methods agree | Labeled (related wording) related wording | serious n=3 | no trial data cached |
| Exposure to sars-cov-2 | 13 | 17.4 10.1–30.0ROR 17.4 · χ² 183 / p 5e-42 | 16.4 EB05 9.5 · IC025 +2.5 3/3 methods agree | Not in label — unassessed | – | no trial data cached |
| Catheter placement | 14 | 17.3 10.2–29.2ROR 17.3 · χ² 197 / p 4e-45 | 16.4 EB05 9.7 · IC025 +2.6 3/3 methods agree | Not in label — unassessed | serious n=11 | no trial data cached |
| Left ventricular hypertrophy | 30 | 16.1 11.2–23.0ROR 16.1 · χ² 406 / p 1e-90 | 15.7 EB05 11.4 · IC025 +3.1 3/3 methods agree | Not in label — unassessed | serious n=29 | no trial data cached |
| Infusion site reaction | 20 | 15.8 10.2–24.5ROR 15.8 · χ² 261 / p 5e-59 | 15.2 EB05 10.0 · IC025 +2.8 3/3 methods agree | Labeled adverse reaction exact term | 90% clinician | no trial data cached |
| End stage renal disease | 122 | 15.2 12.7–18.2ROR 15.4 · χ² 1,596 / p <1e-300 | 15.1 EB05 12.9 · IC025 +3.5 3/3 methods agree | Not in label — unassessed | serious PRR 16.7 · ♀ 15% (drug 40%) | no trial data cached |
| Vein rupture | 10 | 13.7 7.4–25.5ROR 13.7 · χ² 105 / p 6e-25 | 11.8 EB05 5.9 · IC025 +2.0 3/3 methods agree | Not in label — unassessed | serious n=8 | no trial data cached |
| Cardiac pacemaker insertion | 34 | 13.1 9.3–18.3ROR 13.1 · χ² 365 / p 1e-81 | 12.6 EB05 9.2 · IC025 +2.9 3/3 methods agree | Not in label — unassessed | serious n=29 | no trial data cached |
| Incorrect drug administration rate | 23 | 12.5 8.3–18.8ROR 12.5 · χ² 230 / p 3e-52 | 11.7 EB05 7.8 · IC025 +2.6 3/3 methods agree | Reporting / coding artifact | serious n=15 · ♀ 60% (drug 40%) | no trial data cached |
| Temperature intolerance | 53 | 11.9 9.1–15.6ROR 12.0 · χ² 516 / p 2e-114 | 11.6 EB05 9.1 · IC025 +3.0 3/3 methods agree | Not in label — unassessed | serious n=31 | no trial data cached |
| Haemodialysis | 42 | 11.3 8.4–15.3ROR 11.4 · χ² 384 / p 9e-86 | 10.9 EB05 8.2 · IC025 +2.8 3/3 methods agree | Not in label — unassessed | serious PRR 12.2 · ♀ 20% (drug 40%) | no trial data cached |
| Complications of transplanted kidney | 11 | 11.3 6.3–20.4ROR 11.3 · χ² 93 / p 3e-22 | 9.4 EB05 5.0 · IC025 +1.9 3/3 methods agree | Not in label — unassessed | serious n=10 | no trial data cached |
| Exposure via breast milk | 19 | 11.1 7.1–17.5ROR 11.1 · χ² 164 / p 6e-38 | 10.1 EB05 6.4 · IC025 +2.4 3/3 methods agree | Reporting / coding artifact | – | no trial data cached |
| Catheter site pain | 12 | 10.8 6.1–19.1ROR 10.8 · χ² 97 / p 4e-23 | 9.0 EB05 5.0 · IC025 +2.0 3/3 methods agree | Not in label — unassessed | serious n=7 | no trial data cached |
| Blood immunoglobulin e increased | 11 | 10.3 5.7–18.7ROR 10.4 · χ² 83 / p 3e-20 | 8.4 EB05 4.6 · IC025 +1.9 3/3 methods agree | Lab value / disease marker | serious n=7 | no trial data cached |
| Suspected covid-19 | 15 | 10.3 6.2–17.1ROR 10.3 · χ² 116 / p 2e-27 | 8.9 EB05 5.4 · IC025 +2.1 3/3 methods agree | Not in label — unassessed | serious n=4 | no trial data cached |
| Infusion related reaction (reaction during infusion) | 297 | 10.3 9.2–11.5ROR 10.6 · χ² 2,482 / p <1e-300 | 10.2 EB05 9.3 · IC025 +3.1 3/3 methods agree | Not in label — unassessed | serious PRR 6.9 · ♀ 20% (drug 40%) | no trial data cached |
Trial evidence (ClinicalTrials.gov)
Most frequently reported reactions
By raw co-report count, regardless of disproportionality. Common terms here are often the illness itself or generic outcomes.
| Reaction | Reports | PRR 95% CI · ROR · χ²/p | EBGM EB05 · IC025 | Label screen | Serious-only & strata | Trial cross-check |
|---|---|---|---|---|---|---|
| Pyrexia (fever) | 523 | 3.5 3.2–3.8ROR 3.6 · χ² 922 / p 9e-203 | 3.4 EB05 3.2 · IC025 +1.6 3/3 methods agree | Labeled adverse reaction exact term | serious PRR 2.6 | no trial data cached |
| Pain | 509 | 1.9 1.7–2.1ROR 1.9 · χ² 218 / p 1e-49 | 1.9 EB05 1.8 · IC025 +0.8 | Labeled adverse reaction exact term | serious PRR 1.9 | no trial data cached |
| Malaise (feeling unwell) | 493 | 2.6 2.4–2.8ROR 2.6 · χ² 477 / p 5e-106 | 2.6 EB05 2.4 · IC025 +1.2 3/3 methods agree | Not in label — unassessed | serious PRR 2.4 | no trial data cached |
| Chills | 476 | 9.3 8.5–10.1ROR 9.7 · χ² 3,516 / p <1e-300 | 9.2 EB05 8.5 · IC025 +3.0 3/3 methods agree | Labeled adverse reaction exact term | serious PRR 6.3 · ♀ 11% (drug 40%) | no trial data cached |
| Nausea | 468 | 1.4 1.2–1.5ROR 1.4 · χ² 45 / p 1e-11 | 1.4 EB05 1.3 · IC025 +0.3 | Labeled — boxed warning exact term | serious PRR 1.3 | no trial data cached |
| Fatigue (tiredness) | 428 | 1.3 1.1–1.4ROR 1.3 · χ² 23 / p 8e-7 | 1.3 EB05 1.2 · IC025 +0.2 | Labeled adverse reaction exact term | serious PRR 1.5 | no trial data cached |
| Dyspnoea (shortness of breath) | 423 | 1.7 1.6–1.9ROR 1.7 · χ² 127 / p 1e-29 | 1.7 EB05 1.6 · IC025 +0.6 | Labeled — boxed warning exact term | serious PRR 1.8 | no trial data cached |
| Headache | 377 | 1.4 1.2–1.5ROR 1.4 · χ² 39 / p 2e-10 | 1.4 EB05 1.3 · IC025 +0.3 | Labeled adverse reaction exact term | serious PRR 1.6 | no trial data cached |
| Vomiting | 370 | 1.8 1.6–2.0ROR 1.8 · χ² 134 / p 3e-31 | 1.8 EB05 1.7 · IC025 +0.7 | Labeled — boxed warning exact term | serious PRR 1.6 | no trial data cached |
| Weight decreased (weight loss) | 344 | 2.8 2.5–3.1ROR 2.9 · χ² 406 / p 1e-90 | 2.8 EB05 2.6 · IC025 +1.3 3/3 methods agree | Not in label — unassessed | serious PRR 2.0 | no trial data cached |
| Pain in extremity | 315 | 2.4 2.2–2.7ROR 2.5 · χ² 269 / p 1e-60 | 2.4 EB05 2.2 · IC025 +1.1 3/3 methods agree | Labeled adverse reaction exact term | serious PRR 2.9 | no trial data cached |
| Infusion related reaction (reaction during infusion) | 297 | 10.3 9.2–11.5ROR 10.6 · χ² 2,482 / p <1e-300 | 10.2 EB05 9.3 · IC025 +3.1 3/3 methods agree | Not in label — unassessed | serious PRR 6.9 · ♀ 20% (drug 40%) | no trial data cached |
| Dizziness | 283 | 1.3 1.2–1.5ROR 1.3 · χ² 19 / p 5e-6 | 1.3 EB05 1.2 · IC025 +0.2 | Labeled adverse reaction exact term | serious PRR 1.7 | no trial data cached |
| Weight increased (weight gain) | 275 | 2.8 2.5–3.2ROR 2.9 · χ² 324 / p 1e-72 | 2.8 EB05 2.5 · IC025 +1.3 3/3 methods agree | Not in label — unassessed | serious PRR 1.3 | no trial data cached |
| Condition aggravated | 267 | 2.0 1.8–2.3ROR 2.0 · χ² 138 / p 3e-32 | 2.0 EB05 1.8 · IC025 +0.8 | Reporting / coding artifact | serious PRR 2.4 | no trial data cached |
Unlabeled candidate signals
These flagged terms were not found in the FDA label text and do not match an artifact pattern. They are the pairs that would merit a structured assessment (temporality, dose-response, biological plausibility, consistency across databases, trial or cohort evidence). The screen has not done that; string-matching misses label wording, and a strong PRR can still be stimulated reporting, co-medication, or the disease itself.
- Fabry's disease — n=23, PRR 4,314.2
- Angiokeratoma — n=27, PRR 1,105.0
- Renal transplant — n=85, PRR 55.0
- Cardiac hypertrophy — n=24, PRR 50.5
- Ventricular hypertrophy — n=22, PRR 20.0
- Poor venous access — n=87, PRR 19.4
- Left ventricular hypertrophy — n=30, PRR 16.1
- End stage renal disease — n=122, PRR 15.2
- Cardiac pacemaker insertion — n=34, PRR 13.1
- Temperature intolerance — n=53, PRR 11.9
Want to assess one of these properly? The runbook describes the Bradford Hill / trial-evidence workflow used on the assessment page, and handoff_top1000_signals.csv lists the strongest unassessed pairs across all drugs.
Common questions
What are the most reported side effects of Agalsidase Beta?
In FDA FAERS, the most frequently co-reported reaction terms for Agalsidase Beta are pyrexia, pain, malaise, chills, nausea, fatigue. These are report counts, not rates: FAERS has no denominator, reporting is voluntary and uneven, and a term can be reported because of the illness being treated rather than the drug.
Does Agalsidase Beta have an FDA boxed warning?
Yes. The current FDA label carries a boxed warning: HYPERSENSITIVITY REACTIONS INCLUDING ANAPHYLAXIS Patients treated with enzyme replacement therapies have experienced life-threatening hypersensitivity reactions, including anaphylaxis. Anaphylaxis has occurred during the early course of enzyme replacement therapy and after extended duration of therapy.
Do FAERS signals prove that Agalsidase Beta causes these side effects?
No. A disproportionality signal (PRR, ROR) means a reaction is reported more often with this drug than with others in the database. That can reflect a real effect, but also the disease being treated, stimulated reporting after news or litigation, co-medications, or how reports are coded. Causality needs temporality, dose-response, biological plausibility, consistency across data sources and, ideally, trial or cohort evidence. This page screens each term against the FDA label and ClinicalTrials.gov results; it does not establish causation.
Sources & methods
- FDA label via openFDA (set ID 988513ab-b06f-4dd0-93f7-d2d4531dcce4, effective 20250822; matched on
openfda.generic_name.exact:"AGALSIDASE BETA") - Bayesian shrinkage measures computed offline over all 1,317,300 cached pairs: BCPNN information component (IC, with Norén closed-form IC025) and MGPS empirical-Bayes geometric mean (EBGM, EB05) with a two-component gamma prior fitted by maximum marginal likelihood (α₁=0.57, β₁=0.02, α₂=1.42, β₂=0.70, w=0.17); unstratified
- FAERS pair statistics from the OpenPV build of 2026-09-14 (openFDA
count=aggregation, fieldpatient.drug.openfda.generic_name.exact, 1,002 reaction terms, min 3 co-reports); PRR/ROR with 95% CI, chi-square with Yates correction - Serious-only and stratified counts (serious:1, patient.patientsex, primarysource.qualification) from openFDA count queries; serious-only PRR uses the drug serious report total and the term global serious total (N_serious=11,882,979); strata shown only when n ≥ 20
- Seriousness and sex counts from openFDA
count=serious,patient.patientsex,seriousnessdeath
Page generated 2026-10-08. Label and PubChem text are retrieved automatically and may lag the current label; always check the current prescribing information. OpenPV is a beta research tool of the Open Source Medicine Foundation.