Details of Disease

General Information of Disease (ID: DIS1XQCN)

• Disease Name: Pulmonary sarcoidosis
• Synonyms: sarcoidosis of lung; lung sarcoidosis
• Disease Class: 4B20: Sarcoidosis
• Definition: Sarcoidosis affecting the lung parenchyma. It is characterized by the presence of non-necrotizing granulomas in the lung tissues. It is manifested with dyspnea, cough, fever, night sweats, fatigue, and weight loss.
• Disease Hierarchy:
  DISRH1XK: Interstitial lung disease specific to adulthood
  DISE5B8Z: Sarcoidosis
  DIS1XQCN: Pulmonary sarcoidosis
• ICD Code:
  ICD-11: ICD-11: 4B20.0
  ICD-10: ICD-10: D86.0
• Disease Identifiers:
  MONDO ID: MONDO_0001708
  MESH ID: D017565
  UMLS CUI: C0036205
  MedGen ID: 19873
  SNOMED CT ID: 24369008

Drug-Interaction Atlas (DIA) of This Disease

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

Drug Name	Drug ID	Highest Status	Drug Type	REF
ATYR1923	DM98NZR	Phase 3	Fusion protein	[1]

Molecular Interaction Atlas (MIA) of This Disease

This Disease Is Related to 4 DTT Molecule(s)

Gene Name	DTT ID	Evidence Level	Mode of Inheritance	REF
CCR8	TTE836A	Strong	Altered Expression	[2]
CEL	TTTRNQW	Strong	Biomarker	[3]
ICOSLG	TTB9Z8R	Strong	Altered Expression	[4]
ITGAE	TTH0Z37	Strong	Biomarker	[5]

This Disease Is Related to 1 DTP Molecule(s)

Gene Name	DTP ID	Evidence Level	Mode of Inheritance	REF
SLC27A5	DT0TQS3	Strong	Biomarker	[3]

This Disease Is Related to 7 DOT Molecule(s)

Gene Name	DOT ID	Evidence Level	Mode of Inheritance	REF
ACKR4	OTZPWT2N	Strong	Altered Expression	[6]
ADAMDEC1	OTMT7ZMG	Strong	Altered Expression	[7]
BTNL2	OTTTEMZA	Strong	Biomarker	[8]
CUL9	OTPUCLZT	Strong	Altered Expression	[9]
GPR162	OTK4LT3K	Strong	Altered Expression	[10]
HLA-DRB3	OT5PM9N7	Strong	Biomarker	[11]
PARP9	OT7K4494	Strong	Biomarker	[3]

References

• [1] ClinicalTrials.gov (NCT05415137) A Phase 3, Randomized, Double-Blind, Placebo-Controlled Study to Evaluate the Efficacy and Safety of Intravenous Efzofitimod in Patients With Pulmonary Sarcoidosis. U.S.National Institutes of Health.
• [2] The C-C chemokine receptors CCR4 and CCR8 identify airway T cells of allergen-challenged atopic asthmatics.J Clin Invest. 2001 Jun;107(11):1357-64. doi: 10.1172/JCI12655.
• [3] Elevated levels of interferon gamma-inducible protein-10 and epithelial neutrophil-activating peptide-78 in patients with pulmonary sarcoidosis.Respirology. 2006 Nov;11(6):708-14. doi: 10.1111/j.1440-1843.2006.00933.x.
• [4] Pulmonary sarcoidosis is associated with high-level inducible co-stimulator (ICOS) expression on lung regulatory T cells--possible implications for the ICOS/ICOS-ligand axis in disease course and resolution.Clin Exp Immunol. 2016 Feb;183(2):294-306. doi: 10.1111/cei.12715. Epub 2015 Nov 24.
• [5] Effect of variation in ITGAE on risk of sarcoidosis, CD103 expression, and chest radiography.Clin Immunol. 2009 Oct;133(1):117-25. doi: 10.1016/j.clim.2009.06.007. Epub 2009 Jul 14.
• [6] Expression of CCX CKR in pulmonary sarcoidosis.Inflamm Res. 2006 Oct;55(10):441-5. doi: 10.1007/s00011-006-6019-9.
• [7] Gene expression profiling identifies MMP-12 and ADAMDEC1 as potential pathogenic mediators of pulmonary sarcoidosis.Am J Respir Crit Care Med. 2009 May 15;179(10):929-38. doi: 10.1164/rccm.200803-490OC. Epub 2009 Feb 12.
• [8] Butyrophilin-like 2 in pulmonary sarcoidosis: a factor for susceptibility and progression?.Hum Immunol. 2011 Apr;72(4):342-7. doi: 10.1016/j.humimm.2011.01.011. Epub 2011 Jan 20.
• [9] Expression of the chemokine PARC mRNA in bronchoalveolar cells of patients with sarcoidosis.Immunol Lett. 2002 Oct 21;84(1):17-22. doi: 10.1016/s0165-2478(02)00130-x.
• [10] Angiotensin II receptor on BALF macrophages from Japanese patients with active sarcoidosis.Sarcoidosis Vasc Diffuse Lung Dis. 1999 Mar;16(1):67-74.
• [11] Lung T-helper cells expressing T-cell receptor AV2S3 associate with clinical features of pulmonary sarcoidosis.Am J Respir Crit Care Med. 2000 Mar;161(3 Pt 1):814-8. doi: 10.1164/ajrccm.161.3.9906001.