MCID: NNS002
MIFTS: 46

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 55
Non-Specific Idiopathic Interstitial Pneumonia 55
Non-Specific Interstitial Pneumonia Nos 69
Non-Specific Interstitial Pneumonia 55

Classifications:

Orphanet: 55  
Rare respiratory diseases


External Ids:

Disease Ontology 12 DOID:2801
NCIt 46 C35717
SNOMED-CT 64 129452008
Orphanet 55 ORPHA91364
UMLS via Orphanet 70 C1290344
ICD10 via Orphanet 33 J84.8

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 pneumonia and lung disease. An important gene associated with Nonspecific Interstitial Pneumonia is SFTPC (Surfactant Protein C), and among its related pathways/superpathways are PAK Pathway and Pathways in cancer. 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 78)
# Related Disease Score Top Affiliating Genes
1 pneumonia 31.4 CCL2 IL13 SFTPC
2 lung disease 30.3 CCL2 IL13 SFTPC
3 interstitial lung disease 30.2 CCL2 IFNG SFTPC
4 pulmonary fibrosis 30.0 CCL2 HPS1 IL13 SFTPC
5 idiopathic interstitial pneumonia 29.9 CCL2 IFNG IL13 SFTPC
6 arthritis 29.6 CCL2 IFNG SERPINH1
7 rheumatoid arthritis 29.4 CCL2 CXCL5 IFNG SERPINH1
8 pulmonary fibrosis, idiopathic 28.0 CCL2 CXCL5 CXCR3 FGF2 IFNG IL13
9 surfactant metabolism dysfunction, pulmonary, 1 10.9
10 fibrosis of extraocular muscles, congenital, 1 10.5
11 neonatal respiratory failure 10.4 FOXF1 SFTPC
12 diffuse pulmonary fibrosis 10.4 SERPINH1 SFTPC
13 paragonimiasis 10.4 CXCR3 IL13
14 vernal keratoconjunctivitis 10.4 CXCR3 IL13
15 african tick-bite fever 10.3 IFNG IL13
16 orofacial granulomatosis 10.3 CXCR3 IFNG
17 penicillin allergy 10.3 IFNG IL13
18 strongyloidiasis 10.3 IFNG IL13
19 intermediate uveitis 10.3 CXCR3 IFNG
20 urinary schistosomiasis 10.3 IFNG IL13
21 peanut allergy 10.3 IFNG IL13
22 erythema multiforme 10.3 CXCR3 IFNG
23 lichen disease 10.3 CXCR3 IFNG
24 vacterl association 10.2 FOXF1 SHH
25 esophageal atresia 10.1 FOXF1 SHH
26 lipoid nephrosis 10.1 CCL2 IL13
27 paranasal sinus disease 10.1 IFNG IL13
28 pleural tuberculosis 10.1 CCL2 IFNG
29 vascular disease 10.1
30 dermatitis, atopic 10.1 CXCR3 IFNG IL13
31 pulmonary alveolar proteinosis 10.1 CCL2 SFTPC
32 scleroderma, familial progressive 10.0
33 alpha/beta t-cell lymphopenia with gamma/delta t-cell expansion, severe cytomegalovirus infection, and autoimmunity 10.0
34 connective tissue disease 10.0
35 extrapulmonary tuberculosis 10.0 CCL2 IFNG
36 radiculopathy 10.0 CCL2 CXCR3 IL13
37 viral meningitis 10.0 CCL2 IFNG
38 dupuytren contracture 10.0 FGF2 SERPINH1
39 proliferative glomerulonephritis 10.0 CCL2 CXCR3 IFNG
40 uveitis 10.0 CCL2 CXCR3 IFNG
41 central nervous system disease 10.0 CCL2 CXCR3 IFNG
42 autoimmune disease 10.0
43 autoimmune disease 1 10.0
44 pulmonary hypertension 10.0
45 lymphoma 10.0
46 dermatomyositis 10.0
47 allergic hypersensitivity disease 10.0
48 acute interstitial pneumonia 10.0
49 undifferentiated connective tissue disease 10.0
50 autoinflammation, lipodystrophy, and dermatosis syndrome 10.0 CCL2 IFNG IL13

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:

43
# Description MGI Source Accession Score Top Affiliating Genes
1 cardiovascular system MP:0005385 9.92 IFNG SERPINH1 SFTPC CXCR3 SHH FGF2
2 mortality/aging MP:0010768 9.91 HPS1 IFNG IL13 SERPINH1 SFTPC CXCR3
3 neoplasm MP:0002006 9.55 IFNG SFTPC CXCR3 SHH FGF2
4 no phenotypic analysis MP:0003012 9.35 HPS1 IFNG IL13 SFTPC SHH
5 respiratory system MP:0005388 9.23 IFNG IL13 SERPINH1 SFTPC CXCR3 SHH

Drugs & Therapeutics for Nonspecific Interstitial Pneumonia

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

(show all 18)
# 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:


# 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

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:

38
Lung, B Cells, T Cells

Publications for Nonspecific Interstitial Pneumonia

Articles related to Nonspecific Interstitial Pneumonia:

(show top 50) (show all 144)
# Title Authors Year
1
Short-term Automated Quantification of Radiologic Changes in the Characterization of Idiopathic Pulmonary Fibrosis Versus Nonspecific Interstitial Pneumonia and Prediction of Long-term Survival. ( 29219887 )
2018
2
Factors affecting treatment outcome in patients with idiopathic nonspecific interstitial pneumonia: a nationwide cohort study. ( 29212510 )
2017
3
Diffuse large B-cell lymphoma of the lung in a patient with nonspecific interstitial pneumonia. ( 28867466 )
2017
4
Association between nonspecific interstitial pneumonia and presence of CD20+a88B lymphocytes within pulmonary lymphoid follicles. ( 29208971 )
2017
5
Texture analysis using proton density and T2 relaxation in patients with histological usual interstitial pneumonia (UIP) or nonspecific interstitial pneumonia (NSIP). ( 28520778 )
2017
6
A Patient with Non-Hodgkin Lymphoma and Nonspecific Interstitial Pneumonia during Ibrutinib Therapy. ( 29259834 )
2017
7
Lower expression of platelet derived growth factor is associated with better overall survival rate of patients with idiopathic nonspecific interstitial pneumonia. ( 28449458 )
2017
8
Nonspecific interstitial pneumonia preceding diagnosis of collagen vascular disease. ( 27492512 )
2016
9
Coexistence of Autoantibodies against the Golgi Complex and Ro52 Antigen in a Patient with Nonspecific Interstitial Pneumonia. ( 26831023 )
2016
10
FDG-PET/CT in the prediction of pulmonary function improvement in nonspecific interstitial pneumonia. A Pilot Study. ( 27842667 )
2016
11
Nonspecific interstitial pneumonia: time to be more specific? ( 27428647 )
2016
12
Confluent Fibrosis and Fibroblast Foci in Fibrotic Nonspecific Interstitial Pneumonia. ( 26864142 )
2016
13
Nonspecific Interstitial Pneumonia: What Is the Optimal Approach to Management? ( 27231862 )
2016
14
Improvement in idiopathic nonspecific interstitial pneumonia after smoking cessation. ( 26029566 )
2015
15
Nonspecific interstitial pneumonia: survival is influenced by the underlying cause. ( 25537566 )
2015
16
Pulmonary Vein Stenosis Mimicking Nonspecific Interstitial Pneumonia. ( 26779359 )
2015
17
Prognosis of nonspecific interstitial pneumonia correlates with perivascular CD4+ T lymphocyte infiltration of the lung. ( 26496721 )
2015
18
Nonspecific interstitial pneumonia overlaps organizing pneumonia in lung-dominant connective tissue disease. ( 26617847 )
2015
19
Efficacy of thrombomodulin for acute exacerbation of idiopathic pulmonary fibrosis and nonspecific interstitial pneumonia: a nonrandomized prospective study. ( 26566367 )
2015
20
Ultrasound in Rheumatologic Interstitial Lung Disease: A Case Report of Nonspecific Interstitial Pneumonia in Rheumatoid Arthritis. ( 26240772 )
2015
21
Nonspecific interstitial pneumonia and usual interstitial pneumonia: is differentiation possible by high-resolution computed tomography? ( 24480140 )
2014
22
Nonspecific interstitial pneumonia: clinical associations and outcomes. ( 25380997 )
2014
23
Hermansky-Pudlak syndrome with nonspecific interstitial pneumonia. ( 24583434 )
2014
24
Prognostic relevance of histological variants in nonspecific interstitial pneumonia. ( 24621097 )
2014
25
Nonspecific Interstitial pneumonia (NSIP)/ Overlap or Distinct Entity: A case report from the National Research Institute of Tuberculosis and Lung Disease (NRITLD). ( 24778789 )
2014
26
Nonspecific interstitial pneumonia and usual interstitial pneumonia: comparison of the clinicopathologic features and prognosis. ( 25364525 )
2014
27
Pathologically proved nonspecific interstitial pneumonia: CT pattern analysis as compared with usual interstitial pneumonia CT pattern. ( 24661246 )
2014
28
Airspace enlargement with fibrosis shows characteristic histology and immunohistology different from usual interstitial pneumonia, nonspecific interstitial pneumonia and centrilobular emphysema. ( 23692421 )
2013
29
Quadriceps weakness contributes to exercise capacity in nonspecific interstitial pneumonia. ( 23312985 )
2013
30
Proteomic analysis in usual and nonspecific interstitial pneumonia. ( 24048960 )
2013
31
Pathological characteristics in idiopathic nonspecific interstitial pneumonia with emphysema and pulmonary hypertension. ( 25473539 )
2013
32
The difference of neovascularization in early intra-alveolar fibrosis between nonspecific interstitial pneumonia and usual interstitial pneumonia. ( 23714250 )
2013
33
Usual interstitial pneumonia coexisted with nonspecific interstitial pneumonia, What's the diagnosis? ( 23206520 )
2012
34
Nonspecific interstitial pneumonia: a study of 6 patients with progressive disease. ( 22173118 )
2012
35
Increased lymphatic vessel length is associated with the fibroblast reticulum and disease severity in usual interstitial pneumonia and nonspecific interstitial pneumonia. ( 22797508 )
2012
36
Severe pulmonary hypertension in idiopathic nonspecific interstitial pneumonia. ( 22558525 )
2012
37
Comparative manifestations and diagnostic accuracy of high-resolution computed tomography in usual interstitial pneumonia and nonspecific interstitial pneumonia. ( 22759772 )
2012
38
Pulmonary dendritic cell accumulation in usual interstitial pneumonia and nonspecific interstitial pneumonia. ( 23311128 )
2012
39
Current status of idiopathic nonspecific interstitial pneumonia. ( 23001799 )
2012
40
Familial forms of nonspecific interstitial pneumonia/idiopathic pulmonary fibrosis: clinical course and genetic background. ( 22781209 )
2012
41
Interstitial lung disease and antinuclear antibody: consider nonspecific interstitial pneumonia histology and newer antibodies. ( 22553272 )
2012
42
Nonspecific interstitial pneumonia. ( 22365250 )
2012
43
Infliximab-induced nonspecific interstitial pneumonia. ( 22688498 )
2012
44
Idiopathic nonspecific interstitial pneumonia: changes in high-resolution computed tomography on long-term follow-up. ( 22446354 )
2012
45
Treatment response and long term follow-up results of nonspecific interstitial pneumonia. ( 22690098 )
2012
46
Pneumocyte biomarkers KL-6 and surfactant protein D reflect the distinct findings of high-resolution computed tomography in nonspecific interstitial pneumonia. ( 21555868 )
2012
47
Lymphatic fluctuation in the parenchymal remodeling stage of acute interstitial pneumonia, organizing pneumonia, nonspecific interstitial pneumonia and idiopathic pulmonary fibrosis. ( 22488224 )
2012
48
A case of isolated IgG4-related interstitial pneumonia: a new consideration for the cause of idiopathic nonspecific interstitial pneumonia. ( 22796844 )
2012
49
Usual interstitial pneumonia and nonspecific interstitial pneumonia: correlation between CT findings at the site of biopsy with pathological diagnoses. ( 22169358 )
2012
50
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

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

GO Terms for Nonspecific Interstitial Pneumonia

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

# Name GO ID Score Top Affiliating Genes
1 extracellular region GO:0005576 9.5 CCL2 CXCL5 FGF2 IFNG IL13 SFTPC
2 extracellular space GO:0005615 9.23 CCL2 CXCL5 FGF2 IFNG IL13 SERPINH1

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

(show all 22)
# Name GO ID Score Top Affiliating Genes
1 positive regulation of cell proliferation GO:0008284 9.88 CXCL5 FGF2 IFNG SHH
2 immune response GO:0006955 9.87 CCL2 CXCL5 IFNG IL13
3 inflammatory response GO:0006954 9.8 CCL2 CXCL5 CXCR3 IL13
4 animal organ morphogenesis GO:0009887 9.65 CCL2 FGF2 FOXF1
5 branching involved in ureteric bud morphogenesis GO:0001658 9.61 FGF2 SHH
6 movement of cell or subcellular component GO:0006928 9.61 CXCR3 IFNG IL13
7 cellular response to cytokine stimulus GO:0071345 9.6 FOXF1 IL13
8 positive regulation of mesenchymal cell proliferation GO:0002053 9.59 FOXF1 SHH
9 pancreas development GO:0031016 9.58 FOXF1 SHH
10 renal system development GO:0072001 9.56 FOXF1 SHH
11 angiogenesis GO:0001525 9.56 CCL2 CXCR3 FGF2 SHH
12 lung morphogenesis GO:0060425 9.55 FOXF1 SHH
13 embryonic digestive tract morphogenesis GO:0048557 9.54 FOXF1 SHH
14 epithelial tube branching involved in lung morphogenesis GO:0060441 9.52 FOXF1 SHH
15 embryonic morphogenesis GO:0048598 9.51 FGF2 SHH
16 chemokine-mediated signaling pathway GO:0070098 9.5 CCL2 CXCL5 CXCR3
17 embryonic foregut morphogenesis GO:0048617 9.46 FOXF1 SHH
18 lung lobe morphogenesis GO:0060463 9.4 FOXF1 SHH
19 respiratory tube development GO:0030323 9.37 FOXF1 SHH
20 chemotaxis GO:0006935 9.26 CCL2 CXCL5 CXCR3 FGF2
21 trachea development GO:0060438 9.16 FOXF1 SHH
22 regulation of receptor activity GO:0010469 9.1 CCL2 CXCL5 FGF2 IFNG IL13 SHH

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

# Name GO ID Score Top Affiliating Genes
1 cytokine activity GO:0005125 9.02 CCL2 CXCL5 FGF2 IFNG IL13

Sources for Nonspecific Interstitial Pneumonia

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
16 ExPASy
18 FMA
27 GO
28 GTR
29 HGMD
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 MedGen
41 MeSH
42 MESH via Orphanet
43 MGI
45 NCI
46 NCIt
47 NDF-RT
50 NINDS
51 Novoseek
53 OMIM
54 OMIM via Orphanet
58 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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