MCID: PLM015
MIFTS: 23

Pulmonary Systemic Sclerosis

Categories: Immune diseases, Respiratory diseases

Aliases & Classifications for Pulmonary Systemic Sclerosis

MalaCards integrated aliases for Pulmonary Systemic Sclerosis:

Name: Pulmonary Systemic Sclerosis 12 15
Lung Involvement in Systemic Sclerosis 12
Lung Disease with Systemic Sclerosis 12

Classifications:



External Ids:

Disease Ontology 12 DOID:1578
ICD9CM 36 517.2
ICD10 34 M34.81
UMLS 74 C0339904

Summaries for Pulmonary Systemic Sclerosis

MalaCards based summary : Pulmonary Systemic Sclerosis, also known as lung involvement in systemic sclerosis, is related to scleroderma, familial progressive and interstitial lung disease. An important gene associated with Pulmonary Systemic Sclerosis is GDF15 (Growth Differentiation Factor 15). The drugs Cyclophosphamide and Leuprolide have been mentioned in the context of this disorder. Affiliated tissues include skin.

Related Diseases for Pulmonary Systemic Sclerosis

Diseases related to Pulmonary Systemic Sclerosis via text searches within MalaCards or GeneCards Suite gene sharing:

# Related Disease Score Top Affiliating Genes
1 scleroderma, familial progressive 10.0
2 interstitial lung disease 10.0
3 lung disease 10.0

Symptoms & Phenotypes for Pulmonary Systemic Sclerosis

Drugs & Therapeutics for Pulmonary Systemic Sclerosis

Drugs for Pulmonary Systemic Sclerosis (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 9)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Cyclophosphamide Approved, Investigational Phase 3 50-18-0, 6055-19-2 2907
2
Leuprolide Approved, Investigational Phase 3 53714-56-0 657181 3911
3 Immunologic Factors Phase 3
4 Antirheumatic Agents Phase 3
5 Antineoplastic Agents, Alkylating Phase 3
6 Alkylating Agents Phase 3
7 Immunosuppressive Agents Phase 3
8 Antineoplastic Agents, Hormonal Phase 3
9 Fertility Agents Phase 3

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Scleroderma Lung Disease Completed NCT00004563 Phase 3 Cyclophosphamide;Placebo
2 GnRH-a for Ovarian Protection During CYC Therapy for Rheumatic Diseases Terminated NCT01257802 Phase 3 depot leuprolide acetate 3.75 mg;Placebo
3 Creation of a Biospecimen Repository From Patients With Interstitial Lung Diseases (ILD) Recruiting NCT03478826
4 Non-interventional Study Describing Epidemiology, Prognosis and Patient Healthcare Costs in France, 2010-2017 Not yet recruiting NCT03858842

Search NIH Clinical Center for Pulmonary Systemic Sclerosis

Genetic Tests for Pulmonary Systemic Sclerosis

Anatomical Context for Pulmonary Systemic Sclerosis

MalaCards organs/tissues related to Pulmonary Systemic Sclerosis:

42
Skin

Publications for Pulmonary Systemic Sclerosis

Articles related to Pulmonary Systemic Sclerosis:

# Title Authors Year
1
18F-fluorodeoxyglucose positron-emission tomography/CT and lung involvement in systemic sclerosis. ( 30337426 )
2019
2
Th-17 cytokines and interstitial lung involvement in systemic sclerosis. ( 27869103 )
2016
3
Lung involvement in systemic sclerosis: role of high resolution computed tomography and its relationship with other pulmonary and clinico-serological features. ( 25316135 )
2014
4
Growth differentiation factor 15, a marker of lung involvement in systemic sclerosis, is involved in fibrosis development but is not indispensable for fibrosis development. ( 24504814 )
2014
5
Lung involvement in systemic sclerosis. ( 21195581 )
2011
6
Serum III procollagen, but not serum I procollagen, is predictive of lung involvement in systemic sclerosis. ( 9706450 )
1998
7
Lung involvement in systemic sclerosis (scleroderma): relation to classification based on extent of skin involvement or autoantibody status. ( 8736656 )
1996
8
Lung involvement in systemic sclerosis sine scleroderma treated by plasma exchange. ( 1516994 )
1992
9
Lung involvement in systemic sclerosis. ( 1600445 )
1992
10
Lung involvement in systemic sclerosis. ( 2406808 )
1990

Variations for Pulmonary Systemic Sclerosis

Expression for Pulmonary Systemic Sclerosis

LifeMap Discovery
Genes differentially expressed in tissues of Pulmonary Systemic Sclerosis patients vs. healthy controls: 35
# Gene Description Tissue Up/Dn Fold Change (log2) P value
1 DEFA1 defensin, alpha 1 Lung - 3.15 0.000
Search GEO for disease gene expression data for Pulmonary Systemic Sclerosis.

Pathways for Pulmonary Systemic Sclerosis

GO Terms for Pulmonary Systemic Sclerosis

Sources for Pulmonary Systemic Sclerosis

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
20 FMA
29 GO
30 GTR
31 HGMD
32 HMDB
33 HPO
34 ICD10
35 ICD10 via Orphanet
36 ICD9CM
37 IUPHAR
38 KEGG
39 LifeMap
41 LOVD
43 MedGen
45 MeSH
46 MESH via Orphanet
47 MGI
50 NCI
51 NCIt
52 NDF-RT
55 NINDS
56 Novoseek
58 OMIM
59 OMIM via Orphanet
63 PubMed
65 QIAGEN
70 SNOMED-CT via HPO
71 SNOMED-CT via Orphanet
72 TGDB
73 Tocris
74 UMLS
75 UMLS via Orphanet
Content
Loading form....