MCID: NNS002
MIFTS: 44

Nonspecific Interstitial Pneumonia

Categories: Respiratory diseases, Rare diseases

Aliases & Classifications for Nonspecific Interstitial Pneumonia

MalaCards integrated aliases for Nonspecific Interstitial Pneumonia:

Name: Nonspecific Interstitial Pneumonia 12 14 69
Nsip 12 56
Non-Specific Idiopathic Interstitial Pneumonia 56
Non-Specific Interstitial Pneumonia Nos 69
Non-Specific Interstitial Pneumonia 56

Classifications:

Orphanet: 56  
Rare respiratory diseases


External Ids:

Disease Ontology 12 DOID:2801
NCIt 47 C35717
SNOMED-CT 64 129452008
Orphanet 56 ORPHA91364
UMLS via Orphanet 70 C1290344
ICD10 via Orphanet 34 J84.8
UMLS 69 C1290344

Summaries for Nonspecific Interstitial Pneumonia

Disease Ontology : 12 An idiopathic interstitial pneumonia that occurs mainly in women, people who do not smoke, and people younger than 50 years with no known cause or risk factors. Lung biopsies may show predominantly interstitial inflammation or fibrosis or a combination of inflammation and fibrosis. A dry cough and shortness of breath develop over 6 to 18 months. Low-grade fever and a feeling of illness (malaise) may occur.

MalaCards based summary : Nonspecific Interstitial Pneumonia, also known as nsip, is related to rheumatoid arthritis and surfactant metabolism dysfunction, pulmonary, 1. An important gene associated with Nonspecific Interstitial Pneumonia is SFTPC (Surfactant Protein C), and among its related pathways/superpathways are PEDF Induced Signaling and Akt Signaling. The drugs Bosentan and Antihypertensive Agents have been mentioned in the context of this disorder. Affiliated tissues include lung, b cells and t cells, and related phenotypes are cardiovascular system and mortality/aging

Wikipedia : 72 Non-specific interstitial pneumonia (NSIP) is a form of idiopathic interstitial... more...

Related Diseases for Nonspecific Interstitial Pneumonia

Diseases related to Nonspecific Interstitial Pneumonia via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 76)
id Related Disease Score Top Affiliating Genes
1 rheumatoid arthritis 28.0 CCL2 CXCL5 IFNG SERPINH1 TGFB1
2 surfactant metabolism dysfunction, pulmonary, 1 10.8
3 pneumonia 10.8
4 osteoarthritis 10.6 SERPINH1 SFTPC
5 toxic diffuse goiter 10.6 CXCR3 IL13
6 tricuspid valve prolapse 10.5 SERPINH1 SFTPC
7 indian tick typhus 10.5 IFNG IL13
8 viljoen kallis voges syndrome 10.5 CXCR3 IL13
9 goat milk allergy 10.5 IFNG IL13
10 oropharyngeal cancer, childhood 10.5 CXCR3 IFNG
11 acute gonococcal prostatitis 10.4 IFNG IL13
12 papillary squamous carcinoma 10.4 IFNG IL13
13 cyclosporiasis 10.4 CXCR3 IFNG
14 bolivian hemorrhagic fever 10.4 CXCR3 IFNG
15 pulmonary fibrosis 10.3
16 hypereosinophilic syndrome 10.3 IFNG TGFB1
17 branchiootorenal syndrome 10.3 FOXF1 SHH
18 plantar wart 10.3 IFNG TGFB1
19 strawberry gallbladder 10.3 IFNG TGFB1
20 acute vascular insufficiency of intestine 10.2 CXCR3 IFNG
21 chronic graft versus host disease 10.2 IFNG TGFB1
22 acute necrotizing encephalopathy type 1 10.2 CXCR3 IFNG IL13
23 pyridoxine deficiency 10.2 CXCR3 TGFB1
24 lung disease 10.1
25 scrotal carcinoma 10.1 FGF2 TGFB1
26 idiopathic interstitial pneumonia 10.1
27 interstitial lung disease 10.0
28 vascular disease 10.0
29 cholesteatoma of middle ear 10.0 IFNG IL13 TGFB1
30 loiasis 10.0 IFNG IL13 TGFB1
31 prolymphocytic leukemia 10.0 FGF2 SHH
32 discoid lupus erythematosus of eyelid 10.0 IFNG IL13 TGFB1
33 arthritis 10.0
34 connective tissue disease 10.0
35 acute interstitial pneumonia 9.9
36 pulmonary hypertension 9.9
37 undifferentiated connective tissue disease 9.9
38 dermatomyositis 9.9
39 ohdo syndrome, maat-kievit-brunner type 9.8 FGF2 SERPINH1 TGFB1
40 myofascial pain syndrome 9.8 CCL2 CXCR3 IL13
41 steatorrhea 9.8 CCL2 IFNG
42 adenoid squamous cell carcinoma 9.8 CCL2 IL13 SFTPC
43 diffuse glomerulonephritis 9.7 CCL2 CXCR3 IFNG
44 bronchiolitis 9.7
45 thymoma 9.7
46 hermansky-pudlak syndrome 9.7
47 multiple myeloma 9.7
48 tuberculosis 9.7
49 b-cell lymphomas 9.7
50 diffuse large b-cell lymphoma 9.7

Graphical network of the top 20 diseases related to Nonspecific Interstitial Pneumonia:



Diseases related to Nonspecific Interstitial Pneumonia

Symptoms & Phenotypes for Nonspecific Interstitial Pneumonia

MGI Mouse Phenotypes related to Nonspecific Interstitial Pneumonia:

44
id Description MGI Source Accession Score Top Affiliating Genes
1 cardiovascular system MP:0005385 9.97 CXCR3 FGF2 FOXF1 IFNG SERPINH1 SFTPC
2 mortality/aging MP:0010768 9.91 IL13 SERPINH1 SFTPC SHH TGFB1 CXCR3
3 digestive/alimentary MP:0005381 9.8 FOXF1 IFNG IL13 SERPINH1 SHH TGFB1
4 muscle MP:0005369 9.63 FGF2 FOXF1 IFNG IL13 SHH TGFB1
5 neoplasm MP:0002006 9.43 IFNG SFTPC SHH TGFB1 CXCR3 FGF2
6 respiratory system MP:0005388 9.23 CXCR3 FOXF1 IFNG IL13 SERPINH1 SFTPC

Drugs & Therapeutics for Nonspecific Interstitial Pneumonia

Drugs for Nonspecific Interstitial Pneumonia (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 18)
id Name Status Phase Clinical Trials Cas Number PubChem Id
1
Bosentan Approved, Investigational Phase 4 147536-97-8 104865
2 Antihypertensive Agents Phase 4
3 Endothelin Receptor Antagonists Phase 4
4
rituximab Approved Phase 3 174722-31-7 10201696
5 Antirheumatic Agents Phase 3
6
Nitric Oxide Approved 10102-43-9 145068
7 Anti-Asthmatic Agents
8 Antioxidants
9 Autonomic Agents
10 Bronchodilator Agents
11 Endothelium-Dependent Relaxing Factors
12 Neurotransmitter Agents
13 Peripheral Nervous System Agents
14 Protective Agents
15 Respiratory System Agents
16 Vasodilator Agents
17 Antibodies
18 Immunoglobulins

Interventional clinical trials:


id Name Status NCT ID Phase Drugs
1 Bosentan in Pulmonary Hypertension in Interstitial Lung Disease Treatment Study Unknown status NCT00637065 Phase 4 Bosentan;Placebo
2 68Ga-DOTA-NOC PET/CT in Patients With Idiopathic Pulmonary Fibrosis Terminated NCT01321996 Phase 4
3 Rituximab in Rheumatoid Arthritis Lung Disease Completed NCT00578565 Phase 3 Rituximab
4 Case-Control Study of Fibrotic Interstitial Lung Disease (ILD) and Cardiac Disease / Interventions. Unknown status NCT01154998
5 Lung Diffusing Capacity for Nitric Oxide as a Marker of Fibrotic Changes in Idiopathic Interstitial Pneumonias Completed NCT02596841
6 Evaluation of Novel Lung Function Parameters in Patients With Interstitial Lung Disease (ILD) Recruiting NCT02827734
7 Clinical Course of Interstitial Lung Diseases: European IPF Registry and Biobank Recruiting NCT02951416
8 Prospective Observation of Fibrosis in the Lung Clinical Endpoints Study Active, not recruiting NCT01110694

Search NIH Clinical Center for Nonspecific Interstitial Pneumonia

Genetic Tests for Nonspecific Interstitial Pneumonia

Anatomical Context for Nonspecific Interstitial Pneumonia

MalaCards organs/tissues related to Nonspecific Interstitial Pneumonia:

39
Lung, B Cells, T Cells

Publications for Nonspecific Interstitial Pneumonia

Articles related to Nonspecific Interstitial Pneumonia:

(show top 50) (show all 140)
id Title Authors Year
1
Texture analysis using proton density and T2 relaxation in patients with histological usual interstitial pneumonia (UIP) or nonspecific interstitial pneumonia (NSIP). ( 28520778 )
2017
2
Lower expression of platelet derived growth factor is associated with better overall survival rate of patients with idiopathic nonspecific interstitial pneumonia. ( 28449458 )
2017
3
Diffuse large B-cell lymphoma of the lung in a patient with nonspecific interstitial pneumonia. ( 28867466 )
2017
4
Confluent Fibrosis and Fibroblast Foci in Fibrotic Nonspecific Interstitial Pneumonia. ( 26864142 )
2016
5
FDG-PET/CT in the prediction of pulmonary function improvement in nonspecific interstitial pneumonia. A Pilot Study. ( 27842667 )
2016
6
Nonspecific interstitial pneumonia preceding diagnosis of collagen vascular disease. ( 27492512 )
2016
7
Nonspecific interstitial pneumonia: time to be more specific? ( 27428647 )
2016
8
Nonspecific Interstitial Pneumonia: What Is the Optimal Approach to Management? ( 27231862 )
2016
9
Coexistence of Autoantibodies against the Golgi Complex and Ro52 Antigen in a Patient with Nonspecific Interstitial Pneumonia. ( 26831023 )
2016
10
Prognosis of nonspecific interstitial pneumonia correlates with perivascular CD4+ T lymphocyte infiltration of the lung. ( 26496721 )
2015
11
Nonspecific interstitial pneumonia: survival is influenced by the underlying cause. ( 25537566 )
2015
12
Ultrasound in Rheumatologic Interstitial Lung Disease: A Case Report of Nonspecific Interstitial Pneumonia in Rheumatoid Arthritis. ( 26240772 )
2015
13
Improvement in idiopathic nonspecific interstitial pneumonia after smoking cessation. ( 26029566 )
2015
14
Pulmonary Vein Stenosis Mimicking Nonspecific Interstitial Pneumonia. ( 26779359 )
2015
15
Efficacy of thrombomodulin for acute exacerbation of idiopathic pulmonary fibrosis and nonspecific interstitial pneumonia: a nonrandomized prospective study. ( 26566367 )
2015
16
Nonspecific interstitial pneumonia overlaps organizing pneumonia in lung-dominant connective tissue disease. ( 26617847 )
2015
17
Prognostic relevance of histological variants in nonspecific interstitial pneumonia. ( 24621097 )
2014
18
Nonspecific Interstitial pneumonia (NSIP)/ Overlap or Distinct Entity: A case report from the National Research Institute of Tuberculosis and Lung Disease (NRITLD). ( 24778789 )
2014
19
Nonspecific interstitial pneumonia: clinical associations and outcomes. ( 25380997 )
2014
20
Hermansky-Pudlak syndrome with nonspecific interstitial pneumonia. ( 24583434 )
2014
21
Nonspecific interstitial pneumonia and usual interstitial pneumonia: is differentiation possible by high-resolution computed tomography? ( 24480140 )
2014
22
Nonspecific interstitial pneumonia and usual interstitial pneumonia: comparison of the clinicopathologic features and prognosis. ( 25364525 )
2014
23
Pathologically proved nonspecific interstitial pneumonia: CT pattern analysis as compared with usual interstitial pneumonia CT pattern. ( 24661246 )
2014
24
Airspace enlargement with fibrosis shows characteristic histology and immunohistology different from usual interstitial pneumonia, nonspecific interstitial pneumonia and centrilobular emphysema. ( 23692421 )
2013
25
The difference of neovascularization in early intra-alveolar fibrosis between nonspecific interstitial pneumonia and usual interstitial pneumonia. ( 23714250 )
2013
26
Pathological characteristics in idiopathic nonspecific interstitial pneumonia with emphysema and pulmonary hypertension. ( 25473539 )
2013
27
Quadriceps weakness contributes to exercise capacity in nonspecific interstitial pneumonia. ( 23312985 )
2013
28
Proteomic analysis in usual and nonspecific interstitial pneumonia. ( 24048960 )
2013
29
Severe pulmonary hypertension in idiopathic nonspecific interstitial pneumonia. ( 22558525 )
2012
30
Usual interstitial pneumonia and nonspecific interstitial pneumonia: correlation between CT findings at the site of biopsy with pathological diagnoses. ( 22169358 )
2012
31
Familial forms of nonspecific interstitial pneumonia/idiopathic pulmonary fibrosis: clinical course and genetic background. ( 22781209 )
2012
32
Treatment response and long term follow-up results of nonspecific interstitial pneumonia. ( 22690098 )
2012
33
Idiopathic nonspecific interstitial pneumonia: changes in high-resolution computed tomography on long-term follow-up. ( 22446354 )
2012
34
Comparative manifestations and diagnostic accuracy of high-resolution computed tomography in usual interstitial pneumonia and nonspecific interstitial pneumonia. ( 22759772 )
2012
35
Pulmonary dendritic cell accumulation in usual interstitial pneumonia and nonspecific interstitial pneumonia. ( 23311128 )
2012
36
Interstitial lung disease and antinuclear antibody: consider nonspecific interstitial pneumonia histology and newer antibodies. ( 22553272 )
2012
37
Pneumocyte biomarkers KL-6 and surfactant protein D reflect the distinct findings of high-resolution computed tomography in nonspecific interstitial pneumonia. ( 21555868 )
2012
38
Current status of idiopathic nonspecific interstitial pneumonia. ( 23001799 )
2012
39
Infliximab-induced nonspecific interstitial pneumonia. ( 22688498 )
2012
40
A case of isolated IgG4-related interstitial pneumonia: a new consideration for the cause of idiopathic nonspecific interstitial pneumonia. ( 22796844 )
2012
41
Increased lymphatic vessel length is associated with the fibroblast reticulum and disease severity in usual interstitial pneumonia and nonspecific interstitial pneumonia. ( 22797508 )
2012
42
Lymphatic fluctuation in the parenchymal remodeling stage of acute interstitial pneumonia, organizing pneumonia, nonspecific interstitial pneumonia and idiopathic pulmonary fibrosis. ( 22488224 )
2012
43
Usual interstitial pneumonia coexisted with nonspecific interstitial pneumonia, What's the diagnosis? ( 23206520 )
2012
44
Nonspecific interstitial pneumonia. ( 22365250 )
2012
45
Nonspecific interstitial pneumonia: a study of 6 patients with progressive disease. ( 22173118 )
2012
46
Propylthiouracil-induced nonspecific interstitial pneumonia. ( 21362656 )
2011
47
Idiopathic nonspecific interstitial pneumonia: an interstitial lung disease associated with autoimmune disorders? ( 21273390 )
2011
48
Comparison of usual interstitial pneumonia and nonspecific interstitial pneumonia: quantification of disease severity and discrimination between two diseases on HRCT using a texture-based automated system. ( 21603289 )
2011
49
Nonspecific interstitial pneumonia: prognostic significance of high-resolution computed tomography in 59 patients. ( 21926853 )
2011
50
HSP47 in lung fibroblasts is a predictor of survival in fibrotic nonspecific interstitial pneumonia. ( 20129768 )
2010

Variations for Nonspecific Interstitial Pneumonia

Expression for Nonspecific Interstitial Pneumonia

Search GEO for disease gene expression data for Nonspecific Interstitial Pneumonia.

Pathways for Nonspecific Interstitial Pneumonia

Pathways related to Nonspecific Interstitial Pneumonia according to GeneCards Suite gene sharing:

(show all 26)
id Super pathways Score Top Affiliating Genes
1
Show member pathways
13.44 CCL2 CXCL5 CXCR3 IFNG IL13 TGFB1
2
Show member pathways
13.17 CCL2 CXCL5 CXCR3 FGF2 IL13 TGFB1
3
Show member pathways
13.04 CCL2 CXCL5 CXCR3 FGF2 IL13 TGFB1
4 12.09 FGF2 SHH TGFB1
5
Show member pathways
12.09 CCL2 CXCL5 IFNG IL13
6 11.82 CCL2 IFNG TGFB1
7
Show member pathways
11.82 CCL2 CXCL5 CXCR3
8 11.75 FGF2 FOXF1 SHH TGFB1
9 11.64 CCL2 FGF2 TGFB1
10 11.59 CXCR3 IFNG IL13
11 11.51 CCL2 FGF2 IL13 TGFB1
12 11.47 FGF2 SERPINH1 TGFB1
13 11.3 CCL2 IFNG TGFB1
14 11.26 FGF2 SHH TGFB1
15
Show member pathways
11.23 IFNG TGFB1
16 11.21 CCL2 CXCL5 IFNG TGFB1
17 11.12 IFNG IL13
18 11.08 SHH TGFB1
19 11.02 IFNG IL13 TGFB1
20 11.01 FGF2 TGFB1
21 11 CCL2 CXCL5 IL13
22 11 IFNG IL13
23 10.97 CCL2 IFNG
24 10.88 IFNG IL13 TGFB1
25 10.82 CCL2 FGF2 IL13 SFTPC TGFB1
26 10.71 IL13 TGFB1

GO Terms for Nonspecific Interstitial Pneumonia

Cellular components related to Nonspecific Interstitial Pneumonia according to GeneCards Suite gene sharing:

id Name GO ID Score Top Affiliating Genes
1 extracellular region GO:0005576 9.56 CCL2 CXCL5 FGF2 IFNG IL13 SFTPC
2 extracellular space GO:0005615 9.28 CCL2 CXCL5 FGF2 IFNG IL13 SERPINH1

Biological processes related to Nonspecific Interstitial Pneumonia according to GeneCards Suite gene sharing:

(show all 44)
id Name GO ID Score Top Affiliating Genes
1 immune response GO:0006955 9.94 CCL2 CXCL5 IFNG IL13
2 negative regulation of gene expression GO:0010629 9.86 IFNG SHH TGFB1
3 positive regulation of ERK1 and ERK2 cascade GO:0070374 9.85 CCL2 FGF2 TGFB1
4 response to lipopolysaccharide GO:0032496 9.85 CCL2 CXCL5 IL13
5 positive regulation of transcription, DNA-templated GO:0045893 9.83 FGF2 FOXF1 IFNG SHH TGFB1
6 animal organ morphogenesis GO:0009887 9.74 CCL2 FGF2 FOXF1
7 positive regulation of cell proliferation GO:0008284 9.72 CXCL5 FGF2 IFNG SHH TGFB1
8 angiogenesis GO:0001525 9.71 CCL2 CXCR3 FGF2 SHH
9 movement of cell or subcellular component GO:0006928 9.7 CXCR3 IFNG IL13
10 cellular response to dexamethasone stimulus GO:0071549 9.69 CCL2 TGFB1
11 digestive tract development GO:0048565 9.69 FOXF1 TGFB1
12 protein kinase B signaling GO:0043491 9.69 CCL2 TGFB1
13 cellular response to organic cyclic compound GO:0071407 9.69 CCL2 FOXF1 TGFB1
14 lipopolysaccharide-mediated signaling pathway GO:0031663 9.68 CCL2 TGFB1
15 positive regulation of phosphatidylinositol 3-kinase activity GO:0043552 9.68 FGF2 TGFB1
16 cellular response to cytokine stimulus GO:0071345 9.67 FOXF1 IL13
17 positive regulation of mesenchymal cell proliferation GO:0002053 9.67 FOXF1 SHH
18 chemokine-mediated signaling pathway GO:0070098 9.67 CCL2 CXCL5 CXCR3
19 protein import into nucleus, translocation GO:0000060 9.66 IFNG TGFB1
20 pancreas development GO:0031016 9.65 FOXF1 SHH
21 oligodendrocyte development GO:0014003 9.65 SHH TGFB1
22 positive regulation of collagen biosynthetic process GO:0032967 9.65 CCL2 TGFB1
23 positive regulation of protein complex assembly GO:0031334 9.64 IFNG TGFB1
24 positive regulation of blood vessel endothelial cell migration GO:0043536 9.63 FGF2 TGFB1
25 renal system development GO:0072001 9.63 FOXF1 SHH
26 lung morphogenesis GO:0060425 9.62 FOXF1 SHH
27 embryonic digestive tract morphogenesis GO:0048557 9.62 FOXF1 SHH
28 positive regulation of protein import into nucleus GO:0042307 9.61 SHH TGFB1
29 negative regulation of blood vessel endothelial cell migration GO:0043537 9.61 FGF2 TGFB1
30 epithelial tube branching involved in lung morphogenesis GO:0060441 9.6 FOXF1 SHH
31 embryonic morphogenesis GO:0048598 9.59 FGF2 SHH
32 hyaluronan catabolic process GO:0030214 9.58 FGF2 TGFB1
33 negative regulation of interleukin-17 production GO:0032700 9.56 IFNG TGFB1
34 morphogenesis of a branching structure GO:0001763 9.55 FOXF1 TGFB1
35 embryonic foregut morphogenesis GO:0048617 9.54 FOXF1 SHH
36 vasculogenesis GO:0001570 9.54 FOXF1 SHH TGFB1
37 lung lobe morphogenesis GO:0060463 9.48 FOXF1 SHH
38 respiratory tube development GO:0030323 9.46 FOXF1 SHH
39 positive regulation of cell division GO:0051781 9.33 FGF2 SHH TGFB1
40 regulation of regulatory T cell differentiation GO:0045589 9.27 TGFB1
41 trachea development GO:0060438 9.26 FOXF1 SHH
42 chemotaxis GO:0006935 9.26 CCL2 CXCL5 CXCR3 FGF2
43 inflammatory response GO:0006954 9.02 CCL2 CXCL5 CXCR3 IL13 TGFB1
44 positive regulation of transcription from RNA polymerase II promoter GO:0045944 10.06 FGF2 FOXF1 IFNG SHH TGFB1

Molecular functions related to Nonspecific Interstitial Pneumonia according to GeneCards Suite gene sharing:

id Name GO ID Score Top Affiliating Genes
1 cytokine activity GO:0005125 9.1 CCL2 CXCL5 FGF2 IFNG IL13 TGFB1

Sources for Nonspecific Interstitial Pneumonia

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
16 ExPASy
18 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 MedGen
42 MeSH
43 MESH via Orphanet
44 MGI
46 NCI
47 NCIt
48 NDF-RT
51 NINDS
52 Novoseek
54 OMIM
55 OMIM via Orphanet
59 PubMed
60 QIAGEN
65 SNOMED-CT via HPO
66 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
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