NSIP
MCID: NNS002
MIFTS: 48

Nonspecific Interstitial Pneumonia (NSIP)

Categories: Rare diseases, Respiratory 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
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 pulmonary fibrosis. 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 Endothelin Receptor Antagonists 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 101)
# Related Disease Score Top Affiliating Genes
1 pneumonia 31.5 CCL2 IL13 SFTPC
2 pulmonary fibrosis 30.5 CCL2 HPS1 IL13 SFTPC
3 interstitial lung disease 30.3 CCL2 IFNG SFTPC
4 lung disease 30.3 CCL2 CXCR3 IL13 SFTPC
5 idiopathic interstitial pneumonia 30.2 CCL2 IFNG IL13 SFTPC
6 rheumatoid arthritis 29.9 CCL2 CXCL5 IFNG SERPINH1
7 pulmonary fibrosis, idiopathic 29.5 CCL2 CXCL5 CXCR3 FGF2 IFNG IL13
8 surfactant metabolism dysfunction, pulmonary, 1 11.1
9 vascular disease 10.2
10 paragonimiasis 10.2 CXCR3 IL13
11 diffuse pulmonary fibrosis 10.2 SERPINH1 SFTPC
12 neonatal respiratory failure 10.2 FOXF1 SFTPC
13 vernal keratoconjunctivitis 10.2 CXCR3 IL13
14 african tick-bite fever 10.2 IFNG IL13
15 nickel allergic contact dermatitis 10.2 IFNG IL13
16 penicillin allergy 10.2 IFNG IL13
17 strongyloidiasis 10.2 IFNG IL13
18 ascaris lumbricoides infection 10.2 IFNG IL13
19 intermediate uveitis 10.2 CXCR3 IFNG
20 orofacial granulomatosis 10.2 CXCR3 IFNG
21 urinary schistosomiasis 10.2 IFNG IL13
22 peanut allergy 10.2 IFNG IL13
23 scleroderma, familial progressive 10.2
24 pulmonary hypertension 10.2
25 arthritis 10.2
26 connective tissue disease 10.2
27 mycoplasma pneumoniae pneumonia 10.2 IFNG IL13
28 lichen disease 10.2 CXCR3 IFNG
29 erythema multiforme 10.2 CXCR3 IFNG
30 vacterl association 10.1 FOXF1 SHH
31 paranasal sinus disease 10.1 IFNG IL13
32 chronic eosinophilic pneumonia 10.1 CCL2 CXCR3
33 stevens-johnson syndrome/toxic epidermal necrolysis 10.1 CXCR3 IFNG IL13
34 scleritis 10.1 CCL2 IFNG
35 allergic conjunctivitis 10.1 IFNG IL13
36 fungal meningitis 10.1 CCL2 IFNG
37 autoimmune disease 10.1
38 lymphoma 10.1
39 dermatomyositis 10.1
40 acute interstitial pneumonia 10.1
41 undifferentiated connective tissue disease 10.1
42 radiculopathy 10.1 CCL2 CXCR3
43 viral meningitis 10.1 CCL2 IFNG
44 esophageal atresia 10.1 FOXF1 SHH
45 pleural tuberculosis 10.0 CCL2 IFNG
46 lipoid nephrosis 10.0 CCL2 IL13
47 dupuytren contracture 10.0 FGF2 SERPINH1
48 extrapulmonary tuberculosis 10.0 CCL2 IFNG
49 brachydactyly, type b2 10.0 FGF2 IFNG
50 proliferative glomerulonephritis 10.0 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 10.01 CXCR3 FGF2 FOXF1 HPS1 IFNG SERPINH1
2 mortality/aging MP:0010768 9.91 CXCR3 FGF2 FOXF1 HPS1 IFNG IL13
3 immune system MP:0005387 9.87 CCL2 CXCR3 HPS1 IFNG IL13 SFTPC
4 digestive/alimentary MP:0005381 9.77 FOXF1 IFNG IL13 SERPINH1 SHH
5 neoplasm MP:0002006 9.55 CXCR3 FGF2 IFNG SFTPC SHH
6 no phenotypic analysis MP:0003012 9.35 HPS1 IFNG IL13 SFTPC SHH
7 respiratory system MP:0005388 9.23 CXCR3 FOXF1 HPS1 IFNG IL13 SERPINH1

Drugs & Therapeutics for Nonspecific Interstitial Pneumonia

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

(show all 20)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Bosentan Approved, Investigational Phase 4 147536-97-8 104865
2 Endothelin Receptor Antagonists Phase 4
3 Antihypertensive Agents Phase 4
4
rituximab Approved Phase 3 174722-31-7 10201696
5 Antirheumatic Agents Phase 3
6 Immunologic Factors Phase 3
7
Nitric Oxide Approved 10102-43-9 145068
8 Vasodilator Agents
9 Neurotransmitter Agents
10 Anti-Asthmatic Agents
11 Free Radical Scavengers
12 Antioxidants
13 Peripheral Nervous System Agents
14 Bronchodilator Agents
15 Respiratory System Agents
16 Protective Agents
17 Autonomic Agents
18 Endothelium-Dependent Relaxing Factors
19 Immunoglobulins
20 Antibodies

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
8 Idiopathic Pulmonary Fibrosis and Interstitial Lung Disease Prospective Outcomes Registry Active, not recruiting NCT01915511
9 Skeletal Muscle Function in Interstitial Lung Disease Not yet recruiting NCT03800017 Not Applicable

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, Skin, Brain, Heart

Publications for Nonspecific Interstitial Pneumonia

Articles related to Nonspecific Interstitial Pneumonia:

(show top 50) (show all 164)
# 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
Fatal Invasive Pulmonary Aspergillosis Associated with Nonspecific Interstitial Pneumonia: An Autopsy Case Report. ( 30555119 )
2018
7
Expression Pattern of the Hippo Pathway Effector TAZ in Cellular and Fibrotic Nonspecific Interstitial Pneumonia. ( 29483403 )
2018
8
Reprint of: Nonspecific interstitial pneumonia: pathologic features and clinical implications. ( 30249370 )
2018
9
A Patient with Non-Hodgkin Lymphoma and Nonspecific Interstitial Pneumonia during Ibrutinib Therapy. ( 29259834 )
2017
10
Lower expression of platelet derived growth factor is associated with better overall survival rate of patients with idiopathic nonspecific interstitial pneumonia. ( 28449458 )
2017
11
Factors affecting treatment outcome in patients with idiopathic nonspecific interstitial pneumonia: a nationwide cohort study. ( 29212510 )
2017
12
Texture analysis using proton density and T2 relaxation in patients with histological usual interstitial pneumonia (UIP) or nonspecific interstitial pneumonia (NSIP). ( 28520778 )
2017
13
Diffuse large B-cell lymphoma of the lung in a patient with nonspecific interstitial pneumonia. ( 28867466 )
2017
14
Association between nonspecific interstitial pneumonia and presence of CD20+a88B lymphocytes within pulmonary lymphoid follicles. ( 29208971 )
2017
15
Coexistence of Autoantibodies against the Golgi Complex and Ro52 Antigen in a Patient with Nonspecific Interstitial Pneumonia. ( 26831023 )
2016
16
FDG-PET/CT in the prediction of pulmonary function improvement in nonspecific interstitial pneumonia. A Pilot Study. ( 27842667 )
2016
17
Confluent Fibrosis and Fibroblast Foci in Fibrotic Nonspecific Interstitial Pneumonia. ( 26864142 )
2016
18
Nonspecific interstitial pneumonia: time to be more specific? ( 27428647 )
2016
19
Nonspecific Interstitial Pneumonia: What Is the Optimal Approach to Management? ( 27231862 )
2016
20
Nonspecific interstitial pneumonia preceding diagnosis of collagen vascular disease. ( 27492512 )
2016
21
Organizing pneumonia components in non-specific interstitial pneumonia (NSIP): a clinicopathological study of 33 NSIP cases. ( 26100347 )
2016
22
Pulmonary Vein Stenosis Mimicking Nonspecific Interstitial Pneumonia. ( 26779359 )
2015
23
Efficacy of thrombomodulin for acute exacerbation of idiopathic pulmonary fibrosis and nonspecific interstitial pneumonia: a nonrandomized prospective study. ( 26566367 )
2015
24
Improvement in idiopathic nonspecific interstitial pneumonia after smoking cessation. ( 26029566 )
2015
25
Ultrasound in Rheumatologic Interstitial Lung Disease: A Case Report of Nonspecific Interstitial Pneumonia in Rheumatoid Arthritis. ( 26240772 )
2015
26
Nonspecific interstitial pneumonia overlaps organizing pneumonia in lung-dominant connective tissue disease. ( 26617847 )
2015
27
Nonspecific interstitial pneumonia: survival is influenced by the underlying cause. ( 25537566 )
2015
28
Prognosis of nonspecific interstitial pneumonia correlates with perivascular CD4+ T lymphocyte infiltration of the lung. ( 26496721 )
2015
29
Nonspecific interstitial pneumonia and usual interstitial pneumonia: comparison of the clinicopathologic features and prognosis. ( 25364525 )
2014
30
Prognostic relevance of histological variants in nonspecific interstitial pneumonia. ( 24621097 )
2014
31
Nonspecific interstitial pneumonia: clinical associations and outcomes. ( 25380997 )
2014
32
Hermansky-Pudlak syndrome with nonspecific interstitial pneumonia. ( 24583434 )
2014
33
Nonspecific Interstitial pneumonia (NSIP)/ Overlap or Distinct Entity: A case report from the National Research Institute of Tuberculosis and Lung Disease (NRITLD). ( 24778789 )
2014
34
Nonspecific interstitial pneumonia and usual interstitial pneumonia: is differentiation possible by high-resolution computed tomography? ( 24480140 )
2014
35
Pathologically proved nonspecific interstitial pneumonia: CT pattern analysis as compared with usual interstitial pneumonia CT pattern. ( 24661246 )
2014
36
Proteomic analysis in usual and nonspecific interstitial pneumonia. ( 24048960 )
2014
37
Serum analysis of coagulation factors in IPF and NSIP. ( 23912648 )
2014
38
Airspace enlargement with fibrosis shows characteristic histology and immunohistology different from usual interstitial pneumonia, nonspecific interstitial pneumonia and centrilobular emphysema. ( 23692421 )
2013
39
Pathological characteristics in idiopathic nonspecific interstitial pneumonia with emphysema and pulmonary hypertension. ( 25473539 )
2013
40
The difference of neovascularization in early intra-alveolar fibrosis between nonspecific interstitial pneumonia and usual interstitial pneumonia. ( 23714250 )
2013
41
Quadriceps weakness contributes to exercise capacity in nonspecific interstitial pneumonia. ( 23312985 )
2013
42
CD4+ and CD8+ T lymphocytes in lung tissue of NSIP: correlation with T lymphocytes in BALF. ( 23085212 )
2013
43
Treatment response and long term follow-up results of nonspecific interstitial pneumonia. ( 22690098 )
2012
44
Increased lymphatic vessel length is associated with the fibroblast reticulum and disease severity in usual interstitial pneumonia and nonspecific interstitial pneumonia. ( 22797508 )
2012
45
A case of isolated IgG4-related interstitial pneumonia: a new consideration for the cause of idiopathic nonspecific interstitial pneumonia. ( 22796844 )
2012
46
Current status of idiopathic nonspecific interstitial pneumonia. ( 23001799 )
2012
47
Usual interstitial pneumonia coexisted with nonspecific interstitial pneumonia, What's the diagnosis? ( 23206520 )
2012
48
Nonspecific interstitial pneumonia. ( 22365250 )
2012
49
Infliximab-induced nonspecific interstitial pneumonia. ( 22688498 )
2012
50
Usual interstitial pneumonia and nonspecific interstitial pneumonia: correlation between CT findings at the site of biopsy with pathological diagnoses. ( 22169358 )
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 20)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
13.13 CCL2 CXCL5 CXCR3 FGF2 IL13
2
Show member pathways
13.02 CCL2 CXCL5 CXCR3 FGF2 IL13
3 12.5 FGF2 IFNG IL13 SHH
4 11.76 FGF2 FOXF1 SHH
5
Show member pathways
11.68 CCL2 FGF2 IFNG IL13
6
Show member pathways
11.67 CCL2 CXCL5 CXCR3
7 11.64 CCL2 FGF2 IL13
8
Show member pathways
11.59 CCL2 CXCL5 IFNG IL13
9 11.45 CCL2 CXCL5 IFNG
10 11.34 CXCR3 IFNG IL13
11 11.25 IFNG IL13
12 11.18 CCL2 IFNG IL13
13 11.08 IFNG IL13
14 11.03 IFNG IL13
15 10.99 IFNG IL13
16 10.93 CCL2 CXCL5 IL13
17 10.93 IFNG IL13
18 10.89 CCL2 IFNG
19 10.74 CCL2 CXCL5 FGF2 IL13
20 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 21)
# Name GO ID Score Top Affiliating Genes
1 positive regulation of cell proliferation GO:0008284 9.89 CXCL5 FGF2 IFNG SHH
2 inflammatory response GO:0006954 9.8 CCL2 CXCL5 CXCR3 IL13
3 animal organ morphogenesis GO:0009887 9.69 CCL2 FGF2 FOXF1
4 cell chemotaxis GO:0060326 9.63 CCL2 CXCL5 CXCR3
5 angiogenesis GO:0001525 9.62 CCL2 CXCR3 FGF2 SHH
6 branching involved in ureteric bud morphogenesis GO:0001658 9.6 FGF2 SHH
7 pancreas development GO:0031016 9.58 FOXF1 SHH
8 positive regulation of mesenchymal cell proliferation GO:0002053 9.58 FOXF1 SHH
9 renal system development GO:0072001 9.57 FOXF1 SHH
10 lung morphogenesis GO:0060425 9.56 FOXF1 SHH
11 embryonic digestive tract morphogenesis GO:0048557 9.55 FOXF1 SHH
12 immune response GO:0006955 9.55 CCL2 CXCL5 CXCR3 IFNG IL13
13 epithelial tube branching involved in lung morphogenesis GO:0060441 9.54 FOXF1 SHH
14 chemokine-mediated signaling pathway GO:0070098 9.54 CCL2 CXCL5 CXCR3
15 embryonic morphogenesis GO:0048598 9.52 FGF2 SHH
16 embryonic foregut morphogenesis GO:0048617 9.48 FOXF1 SHH
17 lung lobe morphogenesis GO:0060463 9.43 FOXF1 SHH
18 respiratory tube development GO:0030323 9.4 FOXF1 SHH
19 trachea development GO:0060438 9.26 FOXF1 SHH
20 chemotaxis GO:0006935 9.26 CCL2 CXCL5 CXCR3 FGF2
21 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 8.92 CCL2 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
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