MCID: PLM015
MIFTS: 24

Pulmonary Systemic Sclerosis

Categories: 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 34 517.2
SNOMED-CT 67 196133001
ICD10 32 M34.81
UMLS 70 C0339904

Summaries for Pulmonary Systemic Sclerosis

MalaCards based summary : Pulmonary Systemic Sclerosis, also known as lung involvement in systemic sclerosis, is related to systemic scleroderma and pulmonary hypertension, primary, 1. An important gene associated with Pulmonary Systemic Sclerosis is TMSB4X (Thymosin Beta 4 X-Linked). The drugs Methotrexate and Mycophenolic acid have been mentioned in the context of this disorder. Affiliated tissues include lung and 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 systemic scleroderma 10.4
2 pulmonary hypertension, primary, 1 10.3
3 scleroderma, familial progressive 10.2
4 interstitial lung disease 10.2
5 lung disease 10.2
6 fibrosis of extraocular muscles, congenital, 1 10.0
7 pulmonary hypertension 10.0
8 limited scleroderma 10.0
9 pulmonary fibrosis 10.0

Graphical network of the top 20 diseases related to Pulmonary Systemic Sclerosis:



Diseases related to Pulmonary Systemic Sclerosis

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):


# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Methotrexate Approved 1959-05-2, 59-05-2 126941
2
Mycophenolic acid Approved 24280-93-1 446541
3
Cyclophosphamide Approved, Investigational 50-18-0, 6055-19-2 2907
4
Azathioprine Approved 446-86-6 2265
5 Immunosuppressive Agents

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Prevention and Treatment of Interstitial Lung Disease in Systemic Sclerosis Unknown status NCT01858259

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:

40
Lung, Skin

Publications for Pulmonary Systemic Sclerosis

Articles related to Pulmonary Systemic Sclerosis:

(show all 22)
# Title Authors PMID Year
1
Lung involvement in systemic sclerosis is associated with adverse hospital outcomes: insights from the National Inpatient Sample. 61
33589444 2021
2
The Treatment of Lung Involvement in Systemic Sclerosis. 61
33668530 2021
3
Intravenous versus oral cyclophosphamide for lung and/or skin fibrosis in systemic sclerosis: an indirect comparison from EUSTAR and randomised controlled trials. 61
32865169 2020
4
Imatinib-loaded gold nanoparticles inhibit proliferation of fibroblasts and macrophages from systemic sclerosis patients and ameliorate experimental bleomycin-induced lung fibrosis. 61
31430501 2019
5
18F-fluorodeoxyglucose positron-emission tomography/CT and lung involvement in systemic sclerosis. 61
30337426 2019
6
Association of Serum Homocysteine Level and Interstitial Lung Disease in Systemic Sclerosis: A Case-control Study. 61
29956634 2019
7
Th-17 cytokines and interstitial lung involvement in systemic sclerosis. 61
27869103 2016
8
[Lung involvement in systemic sclerosis]. 61
26040160 2015
9
Lung involvement in systemic sclerosis: role of high resolution computed tomography and its relationship with other pulmonary and clinico-serological features. 61
25316135 2014
10
Growth differentiation factor 15, a marker of lung involvement in systemic sclerosis, is involved in fibrosis development but is not indispensable for fibrosis development. 61
24504814 2014
11
Angiotensin II type 1 and 2 receptors and lymphatic vessels modulate lung remodeling and fibrosis in systemic sclerosis and idiopathic pulmonary fibrosis. 61
24473559 2014
12
Lung involvement in systemic sclerosis. 61
21195581 2011
13
HRCT and scleroderma: semiquantitative evaluation of lung damage and functional abnormalities. 61
19266260 2009
14
[Lung involvement in systemic sclerosis]. 61
18051224 2007
15
Serum III procollagen, but not serum I procollagen, is predictive of lung involvement in systemic sclerosis. 61
9706450 1998
16
Lung involvement in systemic sclerosis (scleroderma): relation to classification based on extent of skin involvement or autoantibody status. 61
8736656 1996
17
[Pulmonary involvement in scleroderma assessed with high-resolution computerized tomography and functional tests]. 61
8008890 1994
18
Lung involvement in systemic sclerosis sine scleroderma treated by plasma exchange. 61
1516994 1992
19
Lung involvement in systemic sclerosis. 61
1600445 1992
20
Diffusing capacity of the lung and nifedipine in systemic sclerosis. 61
2242061 1990
21
[The correlations of respiratory function tests, chest roentgenographic manifestations and respiratory symptoms in the patients with systemic sclerosis, using a scoring-method for symptoms]. 61
2214236 1990
22
Lung involvement in systemic sclerosis. 61
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

Biological processes related to Pulmonary Systemic Sclerosis according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 activation of MAPK activity GO:0000187 8.62 GDF15 DUSP7

Molecular functions related to Pulmonary Systemic Sclerosis according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 mitogen-activated protein kinase binding GO:0051019 8.62 DUSP7 ATF7

Sources for Pulmonary Systemic Sclerosis

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
20 GARD
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
53 NINDS
54 Novoseek
56 OMIM via Orphanet
57 OMIM® (Updated 20-May-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 Tocris
70 UMLS
71 UMLS via Orphanet
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