NSIP
MCID: NNS002
MIFTS: 45

Nonspecific Interstitial Pneumonia (NSIP) malady

Categories: Respiratory diseases, Rare diseases

Aliases & Classifications for Nonspecific Interstitial Pneumonia

Aliases & Descriptions 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 PAK Pathway. The drugs Bosentan and Nintedanib have been mentioned in the context of this disorder. Affiliated tissues include lung, t cells and b cells, and related phenotypes are cardiovascular system and digestive/alimentary

Wikipedia : 71 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 72)
id Related Disease Score Top Affiliating Genes
1 rheumatoid arthritis 29.1 CCL2 CXCL5 IFNG SERPINH1 TGFB1
2 surfactant metabolism dysfunction, pulmonary, 1 10.8
3 pneumonia 10.8
4 pulmonary fibrosis 10.3
5 renal hypertension 10.2 CXCR3 IL13
6 osteochondritis dissecans 10.2 SERPINH1 SFTPC
7 israeli tick typhus 10.2 IFNG IL13
8 noonan syndrome 4 10.2 SERPINH1 TGFB1
9 crustacean allergy 10.2 IFNG IL13
10 papillary adenocarcinoma 10.2 IL13 TGFB1
11 acute gonococcal prostatitis 10.1 IFNG IL13
12 lung disease 10.1
13 osteodysplasty precocious of danks mayne and kozlowski 10.1 CXCR3 IFNG
14 distal trisomy 14q 10.1 IFNG TGFB1
15 visceral steatosis 10.1 CXCR3 IL13
16 behcet syndrome 10.1 FOXF1 SHH
17 plantar wart 10.1 IFNG TGFB1
18 myofibroma 10.1 IFNG IL13
19 idiopathic interstitial pneumonia 10.1
20 discharging ear 10.1 IFNG TGFB1
21 autosomal recessive congenital methemoglobinemia 10.1 CXCR3 IFNG IL13
22 cyclosporiasis 10.0 CXCR3 IFNG
23 epidermolysis bullosa 10.0 CXCR3 IFNG IL13
24 vascular disease 10.0
25 interstitial lung disease 10.0
26 burns 10.0 CXCR3 IFNG TGFB1
27 spastic diplegia 10.0 IFNG IL13 TGFB1
28 loiasis 10.0 IFNG IL13 TGFB1
29 erythematosquamous dermatosis 10.0 IFNG IL13 TGFB1
30 urethral intrinsic sphincter deficiency 10.0 CCL2 IFNG
31 connective tissue disease 10.0
32 arthritis 10.0
33 chronic thromboembolic pulmonary hypertension 9.9 IFNG TGFB1
34 venezuelan hemorrhagic fever 9.9 CXCR3 IFNG
35 smooth muscle tumor 9.9 CCL2 CXCR3 IL13
36 rectum squamous cell carcinoma 9.9 CCL2 IL13 SFTPC
37 immune-complex glomerulonephritis 9.9 CCL2 CXCR3 IFNG
38 proximal symphalangism 9.9 CCL2 CXCR3 IFNG
39 acute interstitial pneumonia 9.9
40 pulmonary hypertension 9.9
41 undifferentiated connective tissue disease 9.9
42 dermatomyositis 9.9
43 deafness, autosomal recessive 67 9.9 CCL2 CXCR3 IFNG
44 esophageal candidiasis 9.9 CCL2 CXCR3 IFNG
45 bare lymphocyte syndrome, type i 9.9 CCL2 IFNG IL13
46 glucose intolerance 9.9 CCL2 IFNG
47 substance-induced psychosis 9.8 CCL2 CXCR3 IFNG
48 thymus adenosquamous carcinoma 9.8 CCL2 IFNG IL13
49 epithelioid type angiomyolipoma 9.8 CCL2 CXCR3 IFNG
50 strawberry gallbladder 9.8 CCL2 IFNG TGFB1

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 9.91 SHH TGFB1 CXCR3 FOXF1 IFNG SERPINH1
2 digestive/alimentary MP:0005381 9.8 FOXF1 IFNG IL13 SERPINH1 SHH TGFB1
3 immune system MP:0005387 9.8 CCL2 CXCR3 IFNG IL13 SFTPC SHH
4 mortality/aging MP:0010768 9.76 CXCR3 FOXF1 IFNG IL13 SERPINH1 SFTPC
5 neoplasm MP:0002006 9.35 CXCR3 IFNG SFTPC SHH TGFB1
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 32)
id Name Status Phase Clinical Trials Cas Number PubChem Id
1
Bosentan Approved, Investigational Phase 4 147536-97-8 104865
2
Nintedanib Approved Phase 4,Phase 3 656247-17-5 56843413
3 Antihypertensive Agents Phase 4
4 Endothelin Receptor Antagonists Phase 4
5
rituximab Approved Phase 3 174722-31-7 10201696
6
Mycophenolate mofetil Approved, Investigational Phase 3 128794-94-5 5281078
7
Mycophenolic acid Approved Phase 3 24280-93-1 446541
8 Antirheumatic Agents Phase 3
9 Vasodilator Agents Phase 3
10 Phosphodiesterase 5 Inhibitors Phase 3
11 Phosphodiesterase Inhibitors Phase 3
12 Sildenafil Citrate Phase 3 171599-83-0
13 Anti-Bacterial Agents Phase 3
14 Antibiotics, Antitubercular Phase 3
15 Immunosuppressive Agents Phase 3
16 Pharmaceutical Solutions Phase 3
17 Citrate Nutraceutical Phase 3
18
Citric Acid Nutraceutical, Vet_approved Phase 3 77-92-9 311
19
Nitric Oxide Approved 10102-43-9 145068
20
Menthol Approved 2216-51-5 16666
21 Anti-Asthmatic Agents
22 Antioxidants
23 Autonomic Agents
24 Bronchodilator Agents
25 Endothelium-Dependent Relaxing Factors
26 Neurotransmitter Agents
27 Peripheral Nervous System Agents
28 Protective Agents
29 Respiratory System Agents
30 Antibodies
31 Immunoglobulins
32 Kava Nutraceutical 9000-38-8

Interventional clinical trials:

(show all 12)
id Name Status NCT ID Phase
1 Bosentan in Pulmonary Hypertension in Interstitial Lung Disease Treatment Study Unknown status NCT00637065 Phase 4
2 Effect of Nintedanib on Biomarkers of Extracellular Matrix Turnover in Patients With Idiopathic Pulmonary Fibrosis and Limited Forced Vital Capacity Impairment Recruiting NCT02788474 Phase 4
3 68Ga-DOTA-NOC PET/CT in Patients With Idiopathic Pulmonary Fibrosis Terminated NCT01321996 Phase 4
4 Rituximab in Rheumatoid Arthritis Lung Disease Completed NCT00578565 Phase 3
5 Efficacy and Safety of Nintedanib When Co-administered With Sildenafil in Idiopathic Pulmonary Fibrosis Patients With Advanced Lung Function Impairment Recruiting NCT02802345 Phase 3
6 Evaluation of Efficacy and Safety of Rituximab With Mycophenolate Mofetil in Patients With Interstitial Lung Diseases Recruiting NCT02990286 Phase 3
7 Case-Control Study of Fibrotic Interstitial Lung Disease (ILD) and Cardiac Disease / Interventions. Unknown status NCT01154998
8 Prospective Observation of Fibrosis in the Lung Clinical Endpoints Study Unknown status NCT01110694
9 Lung Diffusing Capacity for Nitric Oxide as a Marker of Fibrotic Changes in Idiopathic Interstitial Pneumonias Completed NCT02596841
10 A New Way of Looking at Your Lungs Completed NCT01949402
11 Evaluation of Novel Lung Function Parameters in Patients With Interstitial Lung Disease (ILD) Recruiting NCT02827734
12 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:

39
Lung, T Cells, B Cells

Publications for Nonspecific Interstitial Pneumonia

Articles related to Nonspecific Interstitial Pneumonia:

(show top 50) (show all 137)
id Title Authors Year
1
Nonspecific interstitial pneumonia preceding diagnosis of collagen vascular disease. ( 27492512 )
2016
2
FDG-PET/CT in the prediction of pulmonary function improvement in nonspecific interstitial pneumonia. A Pilot Study. ( 27842667 )
2016
3
Nonspecific interstitial pneumonia: time to be more specific? ( 27428647 )
2016
4
Coexistence of Autoantibodies against the Golgi Complex and Ro52 Antigen in a Patient with Nonspecific Interstitial Pneumonia. ( 26831023 )
2016
5
Nonspecific Interstitial Pneumonia: What Is the Optimal Approach to Management? ( 27231862 )
2016
6
Confluent Fibrosis and Fibroblast Foci in Fibrotic Nonspecific Interstitial Pneumonia. ( 26864142 )
2016
7
Nonspecific interstitial pneumonia overlaps organizing pneumonia in lung-dominant connective tissue disease. ( 26617847 )
2015
8
Nonspecific interstitial pneumonia: survival is influenced by the underlying cause. ( 25537566 )
2015
9
Ultrasound in Rheumatologic Interstitial Lung Disease: A Case Report of Nonspecific Interstitial Pneumonia in Rheumatoid Arthritis. ( 26240772 )
2015
10
Efficacy of thrombomodulin for acute exacerbation of idiopathic pulmonary fibrosis and nonspecific interstitial pneumonia: a nonrandomized prospective study. ( 26566367 )
2015
11
Pulmonary Vein Stenosis Mimicking Nonspecific Interstitial Pneumonia. ( 26779359 )
2015
12
Prognosis of nonspecific interstitial pneumonia correlates with perivascular CD4+ T lymphocyte infiltration of the lung. ( 26496721 )
2015
13
Improvement in idiopathic nonspecific interstitial pneumonia after smoking cessation. ( 26029566 )
2015
14
Nonspecific interstitial pneumonia: clinical associations and outcomes. ( 25380997 )
2014
15
Pathologically proved nonspecific interstitial pneumonia: CT pattern analysis as compared with usual interstitial pneumonia CT pattern. ( 24661246 )
2014
16
Prognostic relevance of histological variants in nonspecific interstitial pneumonia. ( 24621097 )
2014
17
Hermansky-Pudlak syndrome with nonspecific interstitial pneumonia. ( 24583434 )
2014
18
Nonspecific interstitial pneumonia and usual interstitial pneumonia: is differentiation possible by high-resolution computed tomography? ( 24480140 )
2014
19
Nonspecific Interstitial pneumonia (NSIP)/ Overlap or Distinct Entity: A case report from the National Research Institute of Tuberculosis and Lung Disease (NRITLD). ( 24778789 )
2014
20
Nonspecific interstitial pneumonia and usual interstitial pneumonia: comparison of the clinicopathologic features and prognosis. ( 25364525 )
2014
21
Proteomic analysis in usual and nonspecific interstitial pneumonia. ( 24048960 )
2013
22
Pathological characteristics in idiopathic nonspecific interstitial pneumonia with emphysema and pulmonary hypertension. ( 25473539 )
2013
23
The difference of neovascularization in early intra-alveolar fibrosis between nonspecific interstitial pneumonia and usual interstitial pneumonia. ( 23714250 )
2013
24
Airspace enlargement with fibrosis shows characteristic histology and immunohistology different from usual interstitial pneumonia, nonspecific interstitial pneumonia and centrilobular emphysema. ( 23692421 )
2013
25
Quadriceps weakness contributes to exercise capacity in nonspecific interstitial pneumonia. ( 23312985 )
2013
26
Nonspecific interstitial pneumonia. ( 22365250 )
2012
27
Nonspecific interstitial pneumonia: a study of 6 patients with progressive disease. ( 22173118 )
2012
28
A case of isolated IgG4-related interstitial pneumonia: a new consideration for the cause of idiopathic nonspecific interstitial pneumonia. ( 22796844 )
2012
29
Infliximab-induced nonspecific interstitial pneumonia. ( 22688498 )
2012
30
Idiopathic nonspecific interstitial pneumonia: changes in high-resolution computed tomography on long-term follow-up. ( 22446354 )
2012
31
Pulmonary dendritic cell accumulation in usual interstitial pneumonia and nonspecific interstitial pneumonia. ( 23311128 )
2012
32
Lymphatic fluctuation in the parenchymal remodeling stage of acute interstitial pneumonia, organizing pneumonia, nonspecific interstitial pneumonia and idiopathic pulmonary fibrosis. ( 22488224 )
2012
33
Comparative manifestations and diagnostic accuracy of high-resolution computed tomography in usual interstitial pneumonia and nonspecific interstitial pneumonia. ( 22759772 )
2012
34
Pneumocyte biomarkers KL-6 and surfactant protein D reflect the distinct findings of high-resolution computed tomography in nonspecific interstitial pneumonia. ( 21555868 )
2012
35
Usual interstitial pneumonia and nonspecific interstitial pneumonia: correlation between CT findings at the site of biopsy with pathological diagnoses. ( 22169358 )
2012
36
Treatment response and long term follow-up results of nonspecific interstitial pneumonia. ( 22690098 )
2012
37
Interstitial lung disease and antinuclear antibody: consider nonspecific interstitial pneumonia histology and newer antibodies. ( 22553272 )
2012
38
Current status of idiopathic nonspecific interstitial pneumonia. ( 23001799 )
2012
39
Severe pulmonary hypertension in idiopathic nonspecific interstitial pneumonia. ( 22558525 )
2012
40
Usual interstitial pneumonia coexisted with nonspecific interstitial pneumonia, What's the diagnosis? ( 23206520 )
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
Familial forms of nonspecific interstitial pneumonia/idiopathic pulmonary fibrosis: clinical course and genetic background. ( 22781209 )
2012
43
Propylthiouracil-induced nonspecific interstitial pneumonia. ( 21362656 )
2011
44
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
45
Nonspecific interstitial pneumonia: prognostic significance of high-resolution computed tomography in 59 patients. ( 21926853 )
2011
46
Idiopathic nonspecific interstitial pneumonia: an interstitial lung disease associated with autoimmune disorders? ( 21273390 )
2011
47
Thalidomide induced nonspecific interstitial pneumonia in patient with relapsed multiple myeloma. ( 21179284 )
2010
48
HSP47 in lung fibroblasts is a predictor of survival in fibrotic nonspecific interstitial pneumonia. ( 20129768 )
2010
49
Distinct prognosis of idiopathic nonspecific interstitial pneumonia (NSIP) fulfilling criteria for undifferentiated connective tissue disease (UCTD). ( 20483576 )
2010
50
Nonspecific interstitial pneumonia: histologic correlation with high-resolution CT in 29 patients. ( 18242033 )
2009

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)
id Super pathways Score Top Affiliating Genes
1
Show member pathways
13.37 CCL2 CXCL5 CXCR3 IFNG IL13 TGFB1
2
Show member pathways
13.03 CCL2 CXCL5 CXCR3 IL13 TGFB1
3
Show member pathways
12 CCL2 CXCL5 IFNG IL13
4 11.81 FOXF1 SHH TGFB1
5
Show member pathways
11.74 CCL2 CXCL5 CXCR3
6 11.71 CCL2 IFNG TGFB1
7 11.67 CCL2 IL13 TGFB1
8 11.48 CXCR3 IFNG IL13
9 11.21 CCL2 CXCL5 IFNG TGFB1
10
Show member pathways
11.19 IFNG TGFB1
11 11.19 CCL2 IFNG TGFB1
12 11.09 IFNG IL13
13 11.05 SHH TGFB1
14 10.95 CCL2 CXCL5 IL13
15 10.95 IFNG IL13
16 10.92 CCL2 IFNG
17 10.91 IFNG IL13 TGFB1
18 10.78 IFNG IL13 TGFB1
19 10.72 CCL2 IL13 SFTPC TGFB1
20 10.67 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 9.5 CCL2 CXCL5 IFNG IL13 SFTPC SHH
2 extracellular space GO:0005615 9.23 CCL2 CXCL5 IFNG IL13 SERPINH1 SFTPC

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

(show all 39)
id Name GO ID Score Top Affiliating Genes
1 positive regulation of transcription, DNA-templated GO:0045893 9.94 FOXF1 IFNG SHH TGFB1
2 positive regulation of cell proliferation GO:0008284 9.88 CXCL5 IFNG SHH TGFB1
3 immune response GO:0006955 9.87 CCL2 CXCL5 IFNG IL13
4 heart development GO:0007507 9.84 FOXF1 SHH TGFB1
5 angiogenesis GO:0001525 9.84 CCL2 CXCR3 SHH
6 response to lipopolysaccharide GO:0032496 9.81 CCL2 CXCL5 IL13
7 negative regulation of gene expression GO:0010629 9.77 IFNG SHH TGFB1
8 chemotaxis GO:0006935 9.72 CCL2 CXCL5 CXCR3
9 extrinsic apoptotic signaling pathway GO:0097191 9.68 IFNG TGFB1
10 response to progesterone GO:0032570 9.68 CCL2 TGFB1
11 positive regulation of protein secretion GO:0050714 9.67 IL13 TGFB1
12 negative regulation of T cell proliferation GO:0042130 9.67 SHH TGFB1
13 cellular response to dexamethasone stimulus GO:0071549 9.66 CCL2 TGFB1
14 digestive tract development GO:0048565 9.65 FOXF1 TGFB1
15 protein kinase B signaling GO:0043491 9.65 CCL2 TGFB1
16 lipopolysaccharide-mediated signaling pathway GO:0031663 9.65 CCL2 TGFB1
17 cellular response to cytokine stimulus GO:0071345 9.64 FOXF1 IL13
18 protein import into nucleus, translocation GO:0000060 9.63 IFNG TGFB1
19 pancreas development GO:0031016 9.62 FOXF1 SHH
20 positive regulation of mesenchymal cell proliferation GO:0002053 9.62 FOXF1 SHH
21 oligodendrocyte development GO:0014003 9.61 SHH TGFB1
22 positive regulation of collagen biosynthetic process GO:0032967 9.61 CCL2 TGFB1
23 movement of cell or subcellular component GO:0006928 9.61 CXCR3 IFNG IL13
24 positive regulation of protein complex assembly GO:0031334 9.59 IFNG TGFB1
25 renal system development GO:0072001 9.58 FOXF1 SHH
26 lung morphogenesis GO:0060425 9.56 FOXF1 SHH
27 embryonic digestive tract morphogenesis GO:0048557 9.54 FOXF1 SHH
28 positive regulation of protein import into nucleus GO:0042307 9.52 SHH TGFB1
29 epithelial tube branching involved in lung morphogenesis GO:0060441 9.51 FOXF1 SHH
30 cellular response to organic cyclic compound GO:0071407 9.5 CCL2 FOXF1 TGFB1
31 negative regulation of interleukin-17 production GO:0032700 9.49 IFNG TGFB1
32 embryonic foregut morphogenesis GO:0048617 9.48 FOXF1 SHH
33 morphogenesis of a branching structure GO:0001763 9.46 FOXF1 TGFB1
34 chemokine-mediated signaling pathway GO:0070098 9.43 CCL2 CXCL5 CXCR3
35 lung lobe morphogenesis GO:0060463 9.4 FOXF1 SHH
36 respiratory tube development GO:0030323 9.37 FOXF1 SHH
37 vasculogenesis GO:0001570 9.33 FOXF1 SHH TGFB1
38 inflammatory response GO:0006954 9.02 CCL2 CXCL5 CXCR3 IL13 TGFB1
39 trachea development GO:0060438 8.96 FOXF1 SHH

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 9.02 CCL2 CXCL5 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 Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
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