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 15 73
Nsip 12 59
Non-Specific Idiopathic Interstitial Pneumonia 59
Non-Specific Interstitial Pneumonia Nos 73
Non-Specific Interstitial Pneumonia 59

Classifications:

Orphanet: 59  
Rare respiratory diseases


External Ids:

Disease Ontology 12 DOID:2801
NCIt 50 C35717
SNOMED-CT 68 129452008
Orphanet 59 ORPHA91364
UMLS via Orphanet 74 C1290344
ICD10 via Orphanet 34 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 Akt Signaling and PAK Pathway. 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 : 76 Non-specific interstitial pneumonia (also known as Non-specific interstitial pneumonitis) (NSIP) is a... 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 74)
# Related Disease Score Top Affiliating Genes
1 pneumonia 31.3 CCL2 IL13 SFTPC
2 lung disease 30.2 CCL2 IL13 SFTPC
3 interstitial lung disease 30.0 CCL2 IFNG SFTPC
4 idiopathic interstitial pneumonia 29.5 CCL2 IFNG IL13 SFTPC
5 pulmonary fibrosis 29.1 CCL2 HPS1 IL13 SFTPC
6 rheumatoid arthritis 28.8 CCL2 CXCL5 IFNG SERPINH1
7 pulmonary fibrosis, idiopathic 26.9 CCL2 CXCL5 CXCR3 FGF2 IFNG IL13
8 surfactant metabolism dysfunction, pulmonary, 1 10.9
9 newborn respiratory distress syndrome 10.7 SERPINH1 SFTPC
10 neonatal respiratory failure 10.7 FOXF1 SFTPC
11 diffuse pulmonary fibrosis 10.6 SERPINH1 SFTPC
12 paragonimiasis 10.6 CXCR3 IL13
13 vernal keratoconjunctivitis 10.6 CXCR3 IL13
14 african tick-bite fever 10.5 IFNG IL13
15 penicillin allergy 10.5 IFNG IL13
16 strongyloidiasis 10.5 IFNG IL13
17 intermediate uveitis 10.5 CXCR3 IFNG
18 urinary schistosomiasis 10.5 IFNG IL13
19 orofacial granulomatosis 10.5 CXCR3 IFNG
20 peanut allergy 10.5 IFNG IL13
21 erythema multiforme 10.5 CXCR3 IFNG
22 septic arthritis 10.4 CXCR3 IFNG
23 lichen disease 10.4 CXCR3 IFNG
24 dupuytren contracture 10.4 FGF2 SERPINH1
25 vacterl association 10.3 FOXF1 SHH
26 paranasal sinus disease 10.2 IFNG IL13
27 esophageal atresia 10.1 FOXF1 SHH
28 vascular disease 10.1
29 lipoid nephrosis 10.1 CCL2 IL13
30 pleural tuberculosis 10.1 CCL2 IFNG
31 disuse amblyopia 10.1 FGF2 SHH
32 scleroderma, familial progressive 10.0
33 pulmonary hypertension 10.0
34 arthritis 10.0
35 connective tissue disease 10.0
36 allergic conjunctivitis 10.0 IFNG IL13
37 mesangial proliferative glomerulonephritis 10.0 CCL2 FGF2
38 autoimmune disease 10.0
39 autoimmune disease 1 10.0
40 alpha/beta t-cell lymphopenia with gamma/delta t-cell expansion, severe cytomegalovirus infection, and autoimmunity 10.0
41 lymphoma 10.0
42 dermatomyositis 10.0
43 acute interstitial pneumonia 10.0
44 undifferentiated connective tissue disease 10.0
45 extrapulmonary tuberculosis 10.0 CCL2 IFNG
46 viral meningitis 9.9 CCL2 IFNG
47 pulmonary disease, chronic obstructive 9.9 CCL2 CXCR3 IL13
48 proliferative glomerulonephritis 9.8 CCL2 CXCR3 IFNG
49 diabetic macular edema 9.8 CCL2 FGF2
50 uveitis 9.8 CCL2 CXCR3 IFNG

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:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 cardiovascular system MP:0005385 9.97 SHH FOXF1 HPS1 IFNG SERPINH1 CXCR3
2 mortality/aging MP:0010768 9.91 SHH FOXF1 HPS1 IFNG IL13 SERPINH1
3 immune system MP:0005387 9.87 SHH HPS1 IFNG IL13 CCL2 CXCR3
4 neoplasm MP:0002006 9.55 FGF2 SHH IFNG CXCR3 SFTPC
5 no phenotypic analysis MP:0003012 9.35 SHH HPS1 IFNG IL13 SFTPC
6 respiratory system MP:0005388 9.23 FOXF1 HPS1 IFNG IL13 SERPINH1 CXCR3

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 160954
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:

41
Lung, B Cells, T Cells

Publications for Nonspecific Interstitial Pneumonia

Articles related to Nonspecific Interstitial Pneumonia:

(show top 50) (show all 149)
# Title Authors Year
1
Prevalence and impact of WHO group 3 pulmonary hypertension in advanced idiopathic nonspecific interstitial pneumonia. ( 29773690 )
2018
2
Association of serum high-mobility group box protein 1 level with outcomes of acute exacerbation of idiopathic pulmonary fibrosis and fibrosing nonspecific interstitial pneumonia. ( 29795561 )
2018
3
Differentiating Usual Interstitial Pneumonia From Nonspecific Interstitial Pneumonia Using High-resolution Computed Tomography: The "Straight-edge Sign". ( 29683868 )
2018
4
Expanded acceptance of acute exacerbation of nonspecific interstitial pneumonia, including 7 additional cases with detailed clinical pathologic correlation. ( 29726062 )
2018
5
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
6
Expression Pattern of the Hippo Pathway Effector TAZ in Cellular and Fibrotic Nonspecific Interstitial Pneumonia. ( 29483403 )
2018
7
A Patient with Non-Hodgkin Lymphoma and Nonspecific Interstitial Pneumonia during Ibrutinib Therapy. ( 29259834 )
2017
8
Lower expression of platelet derived growth factor is associated with better overall survival rate of patients with idiopathic nonspecific interstitial pneumonia. ( 28449458 )
2017
9
Factors affecting treatment outcome in patients with idiopathic nonspecific interstitial pneumonia: a nationwide cohort study. ( 29212510 )
2017
10
Texture analysis using proton density and T2 relaxation in patients with histological usual interstitial pneumonia (UIP) or nonspecific interstitial pneumonia (NSIP). ( 28520778 )
2017
11
Diffuse large B-cell lymphoma of the lung in a patient with nonspecific interstitial pneumonia. ( 28867466 )
2017
12
Association between nonspecific interstitial pneumonia and presence of CD20+a88B lymphocytes within pulmonary lymphoid follicles. ( 29208971 )
2017
13
Coexistence of Autoantibodies against the Golgi Complex and Ro52 Antigen in a Patient with Nonspecific Interstitial Pneumonia. ( 26831023 )
2016
14
FDG-PET/CT in the prediction of pulmonary function improvement in nonspecific interstitial pneumonia. A Pilot Study. ( 27842667 )
2016
15
Confluent Fibrosis and Fibroblast Foci in Fibrotic Nonspecific Interstitial Pneumonia. ( 26864142 )
2016
16
Nonspecific interstitial pneumonia: time to be more specific? ( 27428647 )
2016
17
Nonspecific Interstitial Pneumonia: What Is the Optimal Approach to Management? ( 27231862 )
2016
18
Nonspecific interstitial pneumonia preceding diagnosis of collagen vascular disease. ( 27492512 )
2016
19
Pulmonary Vein Stenosis Mimicking Nonspecific Interstitial Pneumonia. ( 26779359 )
2015
20
Efficacy of thrombomodulin for acute exacerbation of idiopathic pulmonary fibrosis and nonspecific interstitial pneumonia: a nonrandomized prospective study. ( 26566367 )
2015
21
Improvement in idiopathic nonspecific interstitial pneumonia after smoking cessation. ( 26029566 )
2015
22
Ultrasound in Rheumatologic Interstitial Lung Disease: A Case Report of Nonspecific Interstitial Pneumonia in Rheumatoid Arthritis. ( 26240772 )
2015
23
Nonspecific interstitial pneumonia overlaps organizing pneumonia in lung-dominant connective tissue disease. ( 26617847 )
2015
24
Nonspecific interstitial pneumonia: survival is influenced by the underlying cause. ( 25537566 )
2015
25
Prognosis of nonspecific interstitial pneumonia correlates with perivascular CD4+ T lymphocyte infiltration of the lung. ( 26496721 )
2015
26
Nonspecific interstitial pneumonia and usual interstitial pneumonia: comparison of the clinicopathologic features and prognosis. ( 25364525 )
2014
27
Prognostic relevance of histological variants in nonspecific interstitial pneumonia. ( 24621097 )
2014
28
Nonspecific interstitial pneumonia: clinical associations and outcomes. ( 25380997 )
2014
29
Hermansky-Pudlak syndrome with nonspecific interstitial pneumonia. ( 24583434 )
2014
30
Nonspecific Interstitial pneumonia (NSIP)/ Overlap or Distinct Entity: A case report from the National Research Institute of Tuberculosis and Lung Disease (NRITLD). ( 24778789 )
2014
31
Nonspecific interstitial pneumonia and usual interstitial pneumonia: is differentiation possible by high-resolution computed tomography? ( 24480140 )
2014
32
Pathologically proved nonspecific interstitial pneumonia: CT pattern analysis as compared with usual interstitial pneumonia CT pattern. ( 24661246 )
2014
33
Airspace enlargement with fibrosis shows characteristic histology and immunohistology different from usual interstitial pneumonia, nonspecific interstitial pneumonia and centrilobular emphysema. ( 23692421 )
2013
34
Pathological characteristics in idiopathic nonspecific interstitial pneumonia with emphysema and pulmonary hypertension. ( 25473539 )
2013
35
The difference of neovascularization in early intra-alveolar fibrosis between nonspecific interstitial pneumonia and usual interstitial pneumonia. ( 23714250 )
2013
36
Quadriceps weakness contributes to exercise capacity in nonspecific interstitial pneumonia. ( 23312985 )
2013
37
Proteomic analysis in usual and nonspecific interstitial pneumonia. ( 24048960 )
2013
38
Treatment response and long term follow-up results of nonspecific interstitial pneumonia. ( 22690098 )
2012
39
Increased lymphatic vessel length is associated with the fibroblast reticulum and disease severity in usual interstitial pneumonia and nonspecific interstitial pneumonia. ( 22797508 )
2012
40
A case of isolated IgG4-related interstitial pneumonia: a new consideration for the cause of idiopathic nonspecific interstitial pneumonia. ( 22796844 )
2012
41
Current status of idiopathic nonspecific interstitial pneumonia. ( 23001799 )
2012
42
Usual interstitial pneumonia coexisted with nonspecific interstitial pneumonia, What's the diagnosis? ( 23206520 )
2012
43
Nonspecific interstitial pneumonia. ( 22365250 )
2012
44
Infliximab-induced nonspecific interstitial pneumonia. ( 22688498 )
2012
45
Usual interstitial pneumonia and nonspecific interstitial pneumonia: correlation between CT findings at the site of biopsy with pathological diagnoses. ( 22169358 )
2012
46
Severe pulmonary hypertension in idiopathic nonspecific interstitial pneumonia. ( 22558525 )
2012
47
Interstitial lung disease and antinuclear antibody: consider nonspecific interstitial pneumonia histology and newer antibodies. ( 22553272 )
2012
48
Comparative manifestations and diagnostic accuracy of high-resolution computed tomography in usual interstitial pneumonia and nonspecific interstitial pneumonia. ( 22759772 )
2012
49
Idiopathic nonspecific interstitial pneumonia: changes in high-resolution computed tomography on long-term follow-up. ( 22446354 )
2012
50
Pneumocyte biomarkers KL-6 and surfactant protein D reflect the distinct findings of high-resolution computed tomography in nonspecific interstitial pneumonia. ( 21555868 )
2012

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 17)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
13.11 CCL2 CXCL5 CXCR3 FGF2 IL13
2
Show member pathways
12.99 CCL2 CXCL5 CXCR3 FGF2 IL13
3 12.5 FGF2 IFNG IL13 SHH
4 11.73 FGF2 FOXF1 SHH
5
Show member pathways
11.72 CCL2 FGF2 IFNG IL13
6
Show member pathways
11.61 CCL2 CXCL5 CXCR3
7
Show member pathways
11.59 CCL2 CXCL5 IFNG IL13
8 11.55 CCL2 FGF2 IL13
9 11.39 CCL2 CXCL5 IFNG
10 11.23 CXCR3 IFNG IL13
11 11.22 IFNG IL13
12 11.05 IFNG IL13
13 11.01 IFNG IL13
14 10.96 IFNG IL13
15 10.91 IFNG IL13
16 10.85 CCL2 IFNG
17 10.72 CCL2 FGF2 IL13 SFTPC

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 20)
# Name GO ID Score Top Affiliating Genes
1 positive regulation of cell proliferation GO:0008284 9.87 CXCL5 FGF2 IFNG SHH
2 immune response GO:0006955 9.86 CCL2 CXCL5 IFNG IL13
3 inflammatory response GO:0006954 9.78 CCL2 CXCL5 CXCR3 IL13
4 animal organ morphogenesis GO:0009887 9.63 CCL2 FGF2 FOXF1
5 branching involved in ureteric bud morphogenesis GO:0001658 9.59 FGF2 SHH
6 positive regulation of mesenchymal cell proliferation GO:0002053 9.58 FOXF1 SHH
7 pancreas development GO:0031016 9.58 FOXF1 SHH
8 angiogenesis GO:0001525 9.56 CCL2 CXCR3 FGF2 SHH
9 renal system development GO:0072001 9.55 FOXF1 SHH
10 lung morphogenesis GO:0060425 9.54 FOXF1 SHH
11 embryonic digestive tract morphogenesis GO:0048557 9.52 FOXF1 SHH
12 embryonic morphogenesis GO:0048598 9.51 FGF2 SHH
13 chemokine-mediated signaling pathway GO:0070098 9.5 CCL2 CXCL5 CXCR3
14 epithelial tube branching involved in lung morphogenesis GO:0060441 9.49 FOXF1 SHH
15 embryonic foregut morphogenesis GO:0048617 9.43 FOXF1 SHH
16 respiratory tube development GO:0030323 9.4 FOXF1 SHH
17 lung lobe morphogenesis GO:0060463 9.37 FOXF1 SHH
18 chemotaxis GO:0006935 9.26 CCL2 CXCL5 CXCR3 FGF2
19 trachea development GO:0060438 9.16 FOXF1 SHH
20 regulation of signaling 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
17 ExPASy
19 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 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 SNOMED-CT via Orphanet
71 TGDB
72 Tocris
73 UMLS
74 UMLS via Orphanet
Content
Loading form....