PMID- 37752586
OWN - NLM
STAT- MEDLINE
DCOM- 20231023
LR  - 20231121
IS  - 1750-1172 (Electronic)
IS  - 1750-1172 (Linking)
VI  - 18
IP  - 1
DP  - 2023 Sep 26
TI  - Low-dose anti-IL 5 treatment in idiopathic hypereosinophilic syndrome: towards a 
      precision medicine approach for remission maintenance.
PG  - 302
LID - 10.1186/s13023-023-02918-9 [doi]
LID - 302
AB  - Mepolizumab at the dose of 300 mg/4 weeks has been recently approved as an add-on 
      therapy for patients with uncontrolled hypereosinophilic syndrome (HES) without 
      any identifiable non-hematologic secondary cause. According to the available 
      real-life evidence mepolizumab 300 mg and 100 mg, licensed for severe 
      eosinophilic asthma, are comparable in terms of drug efficacy. However, the 
      clinical rationale for selecting one dose or the other has not been explored. We 
      investigated the efficacy and safety of mepolizumab 100 mg in idiopathic HES 
      (I-HES) patients as a steroid sparing strategy for disease remission maintenance 
      by assessing clinical conditions, blood eosinophil count (BEC) and adverse events 
      at baseline and at 3-6-12 months follow-up. Overall, 11 patients were enrolled 
      (females 4-36%) with a median age of 62 years (IQR 55.0-72.0). At 3-month visit 
      both prednisone daily dose and BEC significantly decreased from baseline, whilst 
      a substantial improvement of Brief fatigue inventory score (BFI) was not recorded 
      before the 6 months assessment. More than 70% of patients completely stopped 
      prednisone at 12-months follow-up, without any flare in terms of BEC and BFI. No 
      adverse event was registered. Although larger studies are needed, our report 
      firstly describes that in a well-defined population, diagnosed with I-HES and in 
      disease remission, low dose mepolizumab is a safe and effective steroid-sparing 
      option for remission maintenance. It suggests that a personalized treatment dose 
      might be explored according to the disease classification and activity at the 
      time of biologic treatment start.
CI  - (c) 2023. Institut National de la Sante et de la Recherche Medicale (INSERM).
FAU - Caminati, Marco
AU  - Caminati M
AUID- ORCID: 0000-0001-7383-1487
AD  - Department of Medicine, University of Verona & AOUI Verona, Policlinico GB Rossi, 
      Piazzale L.A. Scuro, 10, 37134, Verona, Italy. marco.caminati@univr.it.
FAU - Maule, Matteo
AU  - Maule M
AD  - Asthma Center and Allergy Unit, AOUI Verona, Policlinico GB Rossi, Verona, Italy.
FAU - Benoni, Roberto
AU  - Benoni R
AD  - Department of Diagnostics and Public Health, University of Verona, Verona, Italy.
FAU - Micheletto, Claudio
AU  - Micheletto C
AD  - Respiratory Unit, AOUI Verona, Policlinico GB Rossi, Verona, Italy.
FAU - Tecchio, Cristina
AU  - Tecchio C
AD  - Haematology Unit, AOUI Verona, Policlinico GB Rossi, Verona, Italy.
FAU - Vaia, Rachele
AU  - Vaia R
AD  - Asthma Center and Allergy Unit, AOUI Verona, Policlinico GB Rossi, Verona, Italy.
FAU - De Franceschi, Lucia
AU  - De Franceschi L
AD  - Department of Medicine, University of Verona & AOUI Verona, Policlinico GB Rossi, 
      Piazzale L.A. Scuro, 10, 37134, Verona, Italy.
FAU - Guarnieri, Gabriella
AU  - Guarnieri G
AD  - Department of Cardiac Thoracic Vascular Sciences and Public Health, University of 
      Padova, Padua, Italy.
FAU - Vianello, Andrea
AU  - Vianello A
AD  - Department of Cardiac Thoracic Vascular Sciences and Public Health, University of 
      Padova, Padua, Italy.
FAU - Senna, Gianenrico
AU  - Senna G
AD  - Department of Medicine, University of Verona & AOUI Verona, Policlinico GB Rossi, 
      Piazzale L.A. Scuro, 10, 37134, Verona, Italy.
AD  - Asthma Center and Allergy Unit, AOUI Verona, Policlinico GB Rossi, Verona, Italy.
LA  - eng
PT  - Letter
DEP - 20230926
PL  - England
TA  - Orphanet J Rare Dis
JT  - Orphanet journal of rare diseases
JID - 101266602
RN  - VB0R961HZT (Prednisone)
SB  - IM
MH  - Female
MH  - Humans
MH  - Middle Aged
MH  - Aged
MH  - Prednisone/therapeutic use
MH  - *Precision Medicine
MH  - *Hypereosinophilic Syndrome/drug therapy
PMC - PMC10521477
OTO - NOTNLM
OT  - Hypereosinophilic syndrome
OT  - Mepolizumab
OT  - Remission maintenance
COIS- The authors declare that they have no competing interests.
EDAT- 2023/09/27 00:43
MHDA- 2023/10/23 01:18
PMCR- 2023/09/26
CRDT- 2023/09/26 23:54
PHST- 2023/04/24 00:00 [received]
PHST- 2023/09/15 00:00 [accepted]
PHST- 2023/10/23 01:18 [medline]
PHST- 2023/09/27 00:43 [pubmed]
PHST- 2023/09/26 23:54 [entrez]
PHST- 2023/09/26 00:00 [pmc-release]
AID - 10.1186/s13023-023-02918-9 [pii]
AID - 2918 [pii]
AID - 10.1186/s13023-023-02918-9 [doi]
PST - epublish
SO  - Orphanet J Rare Dis. 2023 Sep 26;18(1):302. doi: 10.1186/s13023-023-02918-9.