Details of Disease

General Information of Disease (ID: DIS7ITOG)

• Disease Name: Eosinophilic granulomatosis with polyangiitis
• Synonyms: allergic granulomatous and angiitis; granulomatous allergic Angiitides; CSS; angiitides, allergic granulomatous; vasculitis, Churg-Strauss; granulomatous vasculitis, eosinophilic; allergic angiitis, granulomatous; allergic angiitis; angiitis, allergic granulomatous; syndrome, Churg-Strauss; Churg-Strauss vasculitis; angiitides, granulomatous allergic; allergic granulomatous angiitis; granulomatous angiitides, allergic; allergic granulomatous Angiitides; allergic granulomatosis angiitis; vasculitides, eosinophilic granulomatous; eosinophilic granulomatous Vasculitides; EGPA; granulomatosis, allergic; angiitis, allergic; allergic granulomatosis; Churg-Strauss syndrome; angiitides, allergic; Churg Strauss Syndrome; allergic angiitis and granulomatosis; eosinophilic granulomatous vasculitis; Churg Strauss syndrome; angiitis, granulomatous allergic; granulomatous angiitis, allergic; vasculitis, Churg Strauss; vasculitis, eosinophilic granulomatous; allergic granulomatoses; allergic angiitides; granulomatous allergic angiitis; granulomatous vasculitides, eosinophilic; allergic angiitides, granulomatous; granulomatoses, allergic
• Disease Class: 4A44: Vasculitis
• Definition: Eosinophilic granulomatosis with polyangiitis (EGPA), previously known as Churg-Strauss syndrome, is a systemic vasculitis of small-to medium vessels, characterized by asthma, transient pulmonary infiltrates, and hypereosinophilia.
• Disease Hierarchy:
  DISU3CNU: Anca-associated vasculitis
  DIS6SVEE: Syndromic disease
  DIS7ITOG: Eosinophilic granulomatosis with polyangiitis
• ICD Code:
  ICD-11: ICD-11: 4A44.A2
  ICD-10: ICD-10: M30.1
  Expand ICD-11: '4A44.A2
  Expand ICD-10: 'M30.1
• Disease Identifiers:
  MONDO ID: MONDO_0015943
  MESH ID: D015267
  UMLS CUI: C0008728
  MedGen ID: 3088
  Orphanet ID: 183
  SNOMED CT ID: 82275008

Drug-Interaction Atlas (DIA) of This Disease

This Disease is Treated as An Indication in 2 Approved Drug(s)

Drug Name	Drug ID	Highest Status	Drug Type	REF
Avacopan	DM5LQHB	Approved	NA	[1]
Rituximab	DM1YVZT	Approved	Antibody	[2]

This Disease is Treated as An Indication in 1 Clinical Trial Drug(s)

Drug Name	Drug ID	Highest Status	Drug Type	REF
GSK3511294	DMWKSFY	Phase 3	Monoclonal antibody	[3]

Molecular Interaction Atlas (MIA) of This Disease

This Disease Is Related to 6 DTT Molecule(s)

Gene Name	DTT ID	Evidence Level	Mode of Inheritance	REF
IL5	TTPREZD	Strong	Biomarker	[4]
IL5RA	TTXH9AD	Strong	Biomarker	[5]
PTGDR2	TTQDMX5	Strong	Altered Expression	[6]
TRB	TT84HCW	Strong	Biomarker	[7]
MPO	TTVCZPI	Definitive	Biomarker	[8]
PRTN3	TT5MLC4	Definitive	Biomarker	[9]

This Disease Is Related to 3 DOT Molecule(s)

Gene Name	DOT ID	Evidence Level	Mode of Inheritance	REF
CCL26	OT2B7HR9	Strong	Biomarker	[10]
CMAS	OTFQJG3C	Strong	Biomarker	[11]
HLA-DRB4	OTNXIHQU	Strong	Biomarker	[12]

References

• [1] Clinical pipeline report, company report or official report of the Pharmaceutical Research and Manufacturers of America (PhRMA)
• [2] Update on eosinophilic granulomatosis with polyangiitis. Allergol Int. 2019 Oct;68(4):430-436.
• [3] ClinicalTrials.gov (NCT05263934) A 52-week, Randomized, Double-blind, Double-dummy, Parallel-group, Multi-centre, Non-inferiority Study to Investigate the Efficacy and Safety of Depemokimab Compared With Mepolizumab in Adults With Relapsing or Refractory Eosinophilic Granulomatosis With Polyangiitis (EGPA) Receiving Standard of Care (SoC) Therapy. U.S.National Institutes of Health.
• [4] Mepolizumab for the treatment of eosinophilic granulomatosis with polyangiitis.Expert Opin Biol Ther. 2019 Jul;19(7):617-630. doi: 10.1080/14712598.2019.1623875. Epub 2019 May 31.
• [5] Immunological and hematological effects of IL-5(R)-targeted therapy: An overview.Allergy. 2018 Oct;73(10):1979-1988. doi: 10.1111/all.13451. Epub 2018 Oct 8.
• [6] The molecular and functional characterization of clonally expanded CD8+ TCR BV T cells in eosinophilic granulomatosis with polyangiitis (EGPA).Clin Immunol. 2014 May-Jun;152(1-2):152-63. doi: 10.1016/j.clim.2014.03.001. Epub 2014 Mar 13.
• [7] Clonal CD8+ TCR-Vbeta expanded populations with effector memory phenotype in Churg Strauss syndrome.Clin Immunol. 2008 Jul;128(1):94-102. doi: 10.1016/j.clim.2008.03.505. Epub 2008 May 23.
• [8] Genome-wide association study of eosinophilic granulomatosis with polyangiitis reveals genomic loci stratified by ANCA status.Nat Commun. 2019 Nov 12;10(1):5120. doi: 10.1038/s41467-019-12515-9.
• [9] Five factor score of more than 1 is associated with relapse during the first 2 year-follow up in patients with eosinophilic granulomatosis with polyangiitis.Int J Rheum Dis. 2017 Sep;20(9):1261-1268. doi: 10.1111/1756-185X.13056. Epub 2017 Mar 5.
• [10] Eotaxin-3 in Churg-Strauss syndrome: a clinical and immunogenetic study.Rheumatology (Oxford). 2011 Oct;50(10):1823-7. doi: 10.1093/rheumatology/keq445. Epub 2011 Jan 25.
• [11] The leukotriene receptor antagonist Montelukast can induce adverse skin reactions in asthmatic patients.Pulm Pharmacol Ther. 2020 Feb;60:101875. doi: 10.1016/j.pupt.2019.101875. Epub 2019 Dec 11.
• [12] Genetic aspects of anti-neutrophil cytoplasmic antibody-associated vasculitis.Nephrol Dial Transplant. 2015 Apr;30 Suppl 1:i37-45. doi: 10.1093/ndt/gfu386. Epub 2014 Dec 18.