Details of Disease

General Information of Disease (ID: DISZGA69)

• Disease Name: Idiopathic pulmonary fibrosis
• Synonyms: pulmonary fibrosis, idiopathic; fibrosing alveolitis; familial idiopathic pulmonary fibrosis; Hamman-rich disease; ILD2; interstitial pneumonitis, usual; fibrocystic pulmonary dysplasia; usual interstitial pneumonia; UIP; fibrosing alveolitis, cryptogenic; idiopathic pulmonary fibrosis, familial; cryptogenic fibrosing alveolitis; IPF; Idiopathic Pulmonary Fibrosis; CFA
• Disease Class: CB03: Idiopathic interstitial pneumonitis
• Definition: A nonneoplastic pulmonary disease that is characterized by the formation of scar tissue within the lungs in the absence of any known cause.
• Disease Hierarchy:
  DISLXQHV: Telomere syndrome
  DIS6UKR7: Inherited interstitial lung disease
  DISH7LPY: Idiopathic interstitial pneumonia
  DISQKVLA: Pulmonary fibrosis
  DISZGA69: Idiopathic pulmonary fibrosis
• ICD Code:
  ICD-11: ICD-11: CB03.4
  ICD-10: ICD-10: J84.1
  ICD-9: ICD-9: 516.3
  Expand ICD-11: 'CB03.4
  Expand ICD-10: 'J84.1
  Expand ICD-9: 516.3
• Disease Identifiers:
  MONDO ID: MONDO_0800029
  MESH ID: D054990
  UMLS CUI: C5561926
  OMIM ID: 178500
  MedGen ID: 1794136
  Orphanet ID: 2032
  SNOMED CT ID: 700250006

Drug-Interaction Atlas (DIA) of This Disease

This Disease is Treated as An Indication in 3 Approved Drug(s)

Drug Name	Drug ID	Highest Status	Drug Type	REF
Actimmune	DM3APM9	Approved	NA	[1]
Intedanib	DMSTA36	Approved	Small molecular drug	[2]
Pirfenidone	DM6VZFQ	Approved	Small molecular drug	[3]

This Disease is Treated as An Indication in 27 Clinical Trial Drug(s)

Drug Name	Drug ID	Highest Status	Drug Type	REF
BI 1015550	DM6DO2P	Phase 3	Small molecule	[4]
Lebrikizumab	DMQP0X5	Phase 3	Monoclonal antibody	[5]
Zinpentraxin alfa	DMNJAVV	Phase 3	Protein	[6]
AMB-05X	DM958C5	Phase 2	Antibody	[7]
CC-930	DMHVEGJ	Phase 2	Small molecular drug	[8]
Cudetaxestat	DMYLDN9	Phase 2	Small molecule	[9]
Dectrekumab	DM1H7UK	Phase 2	Antibody	[10]
ENV-101	DMFOI3T	Phase 2	Small molecule	[11]
GB0139	DMA8KQR	Phase 2	Small molecule	[12]
LYT-100	DMX0GK1	Phase 2	Small molecule	[13]
Ocaperidone	DMP6B59	Phase 2	Small molecular drug	[15]
PBI-4050	DMPXK27	Phase 2	Small molecular drug	[16]
PLN-74809	DMQDN46	Phase 2	Small molecule	[17]
PRM-151	DMK8PTH	Phase 2	NA	[5]
Romilkimab	DMYEZ5J	Phase 2	Antibody	[18]
SAR-156597	DMUICJD	Phase 2	Antibody	[19]
STX-100	DMR9COG	Phase 2	NA	[20]
Tipelukast	DMS9BDQ	Phase 2	Small molecular drug	[5]
Brilaroxazine	DM90J3J	Phase 1	Small molecule	[21]
CC-90001	DMHPZFD	Phase 1	NA	[5]
GC-1008	DM6R4DO	Phase 1	Small molecular drug	[22]
ISM001-05	DMLEF70	Phase 1	Small molecule	[23]
IW-001	DM0U073	Phase 1	NA	[24]
IW001	DM1ZY4U	Phase 1	Recombinant protein	[25]
LTI-03	DMBGM88	Phase 1	Peptide	[26]
RXC006	DM9LUTV	Phase 1	Small molecule	[27]
Vixarelimab	DMB1NE2	Phase 1	Antibody	[28]

This Disease is Treated as An Indication in 1 Drugs in Phase 2 Trial

Drug Name	Drug ID	Highest Status	Drug Type	REF
Nintedanib	DMMQ2OW	Phase 2 Trial	Small molecular drug	[14]

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

Drug Name	Drug ID	Highest Status	Drug Type	REF
Batimastat	DM92VRP	Preclinical	Small molecular drug	[29]
BMS-986202	DM8TAI9	Preclinical	Small molecular drug	[30]

Molecular Interaction Atlas (MIA) of This Disease

This Disease Is Related to 1 DOT Molecule(s)

Gene Name	DOT ID	Evidence Level	Mode of Inheritance	REF
SFTPA2	OT6SFOMU	Definitive	Autosomal dominant	[31]

References

• [1] Drugs@FDA. U.S. Food and Drug Administration. U.S. Department of Health Human Services. 2007
• [2] Drugs@FDA. U.S. Food and Drug Administration. U.S. Department of Health & Human Services.
• [3] URL: http://www.guidetopharmacology.org Nucleic Acids Res. 2015 Oct 12. pii: gkv1037. The IUPHAR/BPS Guide to PHARMACOLOGY in 2016: towards curated quantitative interactions between 1300 protein targets and 6000 ligands. (Ligand id: 7532).
• [4] ClinicalTrials.gov (NCT05321069) A Double Blind, Randomized, Placebo-controlled Trial Evaluating the Efficacy and Safety of BI 1015550 Over at Least 52 Weeks in Patients With Idiopathic Pulmonary Fibrosis (IPF). U.S.National Institutes of Health.
• [5] Clinical pipeline report, company report or official report of the Pharmaceutical Research and Manufacturers of America (PhRMA)
• [6] ClinicalTrials.gov (NCT04552899) A Phase III Randomized, Double-blind, Placebo Controlled Trial to Evaluate the Efficacy and Safety of PRM-151 in Patients With Idiopathic Pulmonary Fibrosis. U.S.National Institutes of Health.
• [7] ClinicalTrials.gov (NCT05349760) A Multicenter, Randomized, Double-Blind, Placebo-Controlled Study to Evaluate the Safety, Tolerability, Pharmacokinetics, Pharmacodynamics, and Preliminary Efficacy of AMB-05X in Subjects With Idiopathic Pulmonary Fibrosis. U.S.National Institutes of Health.
• [8] ClinicalTrials.gov (NCT01203943) A Study to Characterize the Safety, PK and Biological Activity of CC-930 in Idiopathic Pulmonary Fibrosis (IPF). U.S. National Institutes of Health.
• [9] ClinicalTrials.gov (NCT05373914) RESPIRARE - A Phase 2, Randomized, Double-blinded, Placebo-controlled, Efficacy and Safety Study of Cudetaxestat (BLD-0409) Assessed Across Three Dose Ranges With or Without Standard of Care (Nintedanib or Pirfenidone) in Patients With Idiopathic Pulmonary Fibrosis (IPF). U.S.National Institutes of Health.
• [10] ClinicalTrials.gov (NCT01266135) Safety and Efficacy of QAX576 in Patients With Idiopathic Pulmonary Fibrosis (IPF). U.S. National Institutes of Health.
• [11] ClinicalTrials.gov (NCT04968574) A Phase 2, Multi-Center Study Evaluating the Safety and Efficacy of ENV-101 (Taladegib) in Subjects With Idiopathic Pulmonary Fibrosis (IPF). U.S.National Institutes of Health.
• [12] ClinicalTrials.gov (NCT03832946) GALACTIC-1 -A Randomized, Double-blind, Multicentre, Parallel, Placebo-controlled Phase 2b Study in Subjects With Idiopathic Pulmonary Fibrosis (IPF) Investigating the Efficacy and Safety of GB0139, an Inhaled Galectin-3 Inhibitor Administered Via a Dry Powder Inhaler Over 52 Weeks. U.S.National Institutes of Health.
• [13] ClinicalTrials.gov (NCT05321420) A Randomized Double-blind, Four-Arm Active and Placebo-controlled Dose-Finding Trial to Evaluate the Efficacy, Tolerability, Safety and Dose Response of LYT-100 in Patients With Idiopathic Pulmonary Fibrosis (IPF). U.S.National Institutes of Health.
• [14] FDA Approved Drug Products from FDA Official Website. 2019. Application Number: (NDA) 205832
• [15] Agents in development for the treatment of diabetic nephropathy. Expert Opin Emerg Drugs. 2008 Sep;13(3):447-63.
• [16] Clinical pipeline report, company report or official report of Liminal BioSciences.
• [17] ClinicalTrials.gov (NCT05621252) A Phase 2a, Randomized, Double-Blind, Placebo-Controlled, Evaluation of PLN-74809 on Type 1 Collagen Deposition Using 68Ga-CBP8 PET/MRI Imaging in Participants With Idiopathic Pulmonary Fibrosis (IPF). U.S.National Institutes of Health.
• [18] ClinicalTrials.gov (NCT02345070) Efficacy and Safety of SAR156597 in the Treatment of Idiopathic Pulmonary Fibrosis (ESTAIR). U.S. National Institutes of Health.
• [19] ClinicalTrials.gov (NCT02345070) Efficacy and Safety of SAR156597 in the Treatment of Idiopathic Pulmonary Fibrosis
• [20] ClinicalTrials.gov (NCT01371305) STX-100 in Patients With Idiopathic Pulmonary Fibrosis (IPF). U.S. National Institutes of Health.
• [21] Clinical pipeline report, company report or official report of Reviva Pharmaceuticals
• [22] Current and emerging drugs for idiopathic pulmonary fibrosis. Expert Opin Emerg Drugs. 2007 Nov;12(4):627-46.
• [23] Clinical pipeline report, company report or official report of InSilico Medicine
• [24] ClinicalTrials.gov (NCT01199887) Trial Of IW001 in Patients With Idiopathic Pulmonary Fibrosis. U.S. National Institutes of Health.
• [25] ClinicalTrials.gov (NCT01199887) Trial Of IW001 in Patients With Idiopathic Pulmonary Fibrosis. U.S. National Institutes of Health.
• [26] ClinicalTrials.gov (NCT04233814) A Randomized, Double-Blind, Placebo-Controlled, Ph 1a, First-in-Man, Single Ascending Dose & Multiple Ascending Dose Safety, Tolerability and PK Study of a Caveolin-1-Scaffolding-Protein-Derived Peptide (LTI-03) in Healthy Adult Subjects. U.S.National Institutes of Health.
• [27] ClinicalTrials.gov (NCT05134727) A Double-blind, Randomized, Placebo-controlled Study in Healthy Volunteers to Investigate the Safety, Tolerability and Pharmacokinetics of Oral AZD5055 Following Single and Multiple Ascending Doses. U.S.National Institutes of Health.
• [28] ClinicalTrials.gov (NCT05785624) A Two-Cohort, Phase II, Multicenter, Randomized, Double-Blind, Parallel-Group, Placebo-Controlled Study Evaluating the Efficacy and Safety of Vixarelimab Compared With Placebo in Patients With Idiopathic Pulmonary Fibrosis and in Patients With Systemic Sclerosis-Associated Interstitial Lung Disease. U.S.National Institutes of Health.
• [29] Drugs and Targets in Fibrosis. Front Pharmacol. 2017 Nov 23;8:855.
• [30] URL: http://www.guidetopharmacology.org Nucleic Acids Res. 2015 Oct 12. pii: gkv1037. The IUPHAR/BPS Guide to PHARMACOLOGY in 2016: towards curated quantitative interactions between 1300 protein targets and 6000 ligands. (Ligand id: 6988).
• [31] Technical standards for the interpretation and reporting of constitutional copy-number variants: a joint consensus recommendation of the American College of Medical Genetics and Genomics (ACMG) and the Clinical Genome Resource (ClinGen). Genet Med. 2020 Feb;22(2):245-257. doi: 10.1038/s41436-019-0686-8. Epub 2019 Nov 6.