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 74
Nsip 12 60
Non-Specific Idiopathic Interstitial Pneumonia 60
Non-Specific Interstitial Pneumonia Nos 74
Non-Specific Interstitial Pneumonia 60

Classifications:

Orphanet: 60  
Rare respiratory diseases


External Ids:

Disease Ontology 12 DOID:2801
NCIt 51 C35717
ICD10 via Orphanet 35 J84.8
UMLS via Orphanet 75 C1290344
Orphanet 60 ORPHA91364

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 arthritis. 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 Endothelin Receptor Antagonists have been mentioned in the context of this disorder. Affiliated tissues include lung, skeletal muscle and b cells, and related phenotypes are cardiovascular system and immune system

Wikipedia : 77 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 106)
# Related Disease Score Top Affiliating Genes
1 pneumonia 31.5 CCL2 IL13 SFTPC
2 arthritis 30.0 CCL2 IFNG TGFB1
3 pulmonary fibrosis 29.9 CCL2 HPS1 IL13 SFTPC TGFB1
4 interstitial lung disease 29.8 CCL2 IFNG SFTPC TGFB1
5 lung disease 29.7 CCL2 CXCR3 IL13 SFTPC TGFB1
6 idiopathic interstitial pneumonia 29.6 CCL2 IFNG IL13 SFTPC TGFB1
7 rheumatoid arthritis 29.0 CCL2 CXCL5 IFNG SERPINH1 TGFB1
8 pulmonary fibrosis, idiopathic 28.7 CCL2 CXCL5 CXCR3 IFNG IL13 SERPINH1
9 surfactant metabolism dysfunction, pulmonary, 1 11.1
10 neonatal respiratory failure 10.3 FOXF1 SFTPC
11 paragonimiasis 10.3 CXCR3 IL13
12 diffuse pulmonary fibrosis 10.3 SERPINH1 SFTPC
13 vernal keratoconjunctivitis 10.3 CXCR3 IL13
14 african tick-bite fever 10.3 IFNG IL13
15 nickel allergic contact dermatitis 10.3 IFNG IL13
16 penicillin allergy 10.2 IFNG IL13
17 radiculopathy 10.2 CXCR3 IL13
18 intermediate uveitis 10.2 CXCR3 IFNG
19 orofacial granulomatosis 10.2 CXCR3 IFNG
20 urinary schistosomiasis 10.2 IFNG IL13
21 peanut allergy 10.2 IFNG IL13
22 vascular disease 10.2
23 fibromatosis, gingival, 1 10.2 SERPINH1 TGFB1
24 mycoplasma pneumoniae pneumonia 10.2 IFNG IL13
25 lichen disease 10.2 CXCR3 IFNG
26 erythema multiforme 10.2 CXCR3 IFNG
27 dupuytren contracture 10.2 SERPINH1 TGFB1
28 scleroderma, familial progressive 10.2
29 pulmonary hypertension 10.2
30 connective tissue disease 10.2
31 vacterl association 10.2 FOXF1 SHH
32 eosinophilia-myalgia syndrome 10.1 IFNG TGFB1
33 clonorchiasis 10.1 IFNG TGFB1
34 tuberculoid leprosy 10.1 IFNG TGFB1
35 chronic active epstein-barr virus infection 10.1 IFNG TGFB1
36 oligoarticular juvenile idiopathic arthritis 10.1 CXCR3 TGFB1
37 pustulosis palmaris et plantaris 10.1 CXCR3 TGFB1
38 hematopoietic stem cell transplantation 10.1
39 pleuroparenchymal fibroelastosis 10.1
40 stevens-johnson syndrome/toxic epidermal necrolysis 10.1 CXCR3 IFNG IL13
41 fungal meningitis 10.1 CCL2 IFNG
42 sarcoidosis 1 10.1 CXCR3 IFNG IL13
43 autoimmune disease 10.1
44 lymphoma 10.1
45 dermatomyositis 10.1
46 acute interstitial pneumonia 10.1
47 undifferentiated connective tissue disease 10.1
48 esophageal atresia 10.1 FOXF1 SHH
49 viral meningitis 10.0 CCL2 IFNG
50 ocular cicatricial pemphigoid 10.0 SERPINH1 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:

47
# Description MGI Source Accession Score Top Affiliating Genes
1 cardiovascular system MP:0005385 10.06 CXCR3 FOXF1 HPS1 IFNG SERPINH1 SFTPC
2 immune system MP:0005387 9.97 CCL2 CXCR3 HPS1 IFNG IL13 SFTPC
3 mortality/aging MP:0010768 9.97 CXCR3 FOXF1 HPS1 IFNG IL13 SERPINH1
4 digestive/alimentary MP:0005381 9.88 FOXF1 IFNG IL13 SERPINH1 SHH TGFB1
5 craniofacial MP:0005382 9.83 HPS1 IFNG SERPINH1 SHH TGFB1
6 neoplasm MP:0002006 9.65 CXCR3 IFNG SFTPC SHH TGFB1
7 no phenotypic analysis MP:0003012 9.55 HPS1 IFNG IL13 SFTPC SHH
8 renal/urinary system MP:0005367 9.35 CXCR3 HPS1 IFNG SHH TGFB1
9 respiratory system MP:0005388 9.28 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 26)
# 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 Antineoplastic Agents, Immunological Phase 3
8
Nitric Oxide Approved 10102-43-9 145068
9
Midazolam Approved, Illicit 59467-70-8 4192
10
Lidocaine Approved, Vet_approved 137-58-6 3676
11 Free Radical Scavengers
12 Anti-Asthmatic Agents
13 Autonomic Agents
14 Neurotransmitter Agents
15 Vasodilator Agents
16 Antioxidants
17 Peripheral Nervous System Agents
18 Respiratory System Agents
19 Bronchodilator Agents
20 Endothelium-Dependent Relaxing Factors
21 Protective Agents
22 Antibodies
23 Immunoglobulins
24 Anesthetics
25 Calamus
26 Pharmaceutical Solutions

Interventional clinical trials:

(show all 11)
# 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 Idiopathic Pulmonary Fibrosis and Interstitial Lung Disease Prospective Outcomes Registry Recruiting NCT01915511
8 Clinical Course of Interstitial Lung Diseases: European IPF Registry and Biobank Recruiting NCT02951416
9 Molecular Diagnosis of Idiopathic Interstitial Pneumonias: a Prospective Study Not yet recruiting NCT03836417
10 Skeletal Muscle Function in Interstitial Lung Disease Not yet recruiting NCT03800017 Not Applicable
11 Clinical Characteristics of Interstitial Pneumonia With Autoimmune Features (IPAF) - a Multicenter Prospective Study Not yet recruiting NCT03870828

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:

42
Lung, Skeletal Muscle, B Cells, T Cells

Publications for Nonspecific Interstitial Pneumonia

Articles related to Nonspecific Interstitial Pneumonia:

(show top 50) (show all 165)
# Title Authors Year
1
Calgranulin B and KL-6 in Bronchoalveolar Lavage of Patients with IPF and NSIP. ( 30680696 )
2019
2
Prevalence and impact of WHO group 3 pulmonary hypertension in advanced idiopathic nonspecific interstitial pneumonia. ( 29773690 )
2018
3
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
4
Differentiating Usual Interstitial Pneumonia From Nonspecific Interstitial Pneumonia Using High-resolution Computed Tomography: The "Straight-edge Sign". ( 29683868 )
2018
5
Expanded acceptance of acute exacerbation of nonspecific interstitial pneumonia, including 7 additional cases with detailed clinical pathologic correlation. ( 29726062 )
2018
6
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
7
Expression Pattern of the Hippo Pathway Effector TAZ in Cellular and Fibrotic Nonspecific Interstitial Pneumonia. ( 29483403 )
2018
8
Fatal Invasive Pulmonary Aspergillosis Associated with Nonspecific Interstitial Pneumonia: An Autopsy Case Report. ( 30555119 )
2018
9
Reprint of: Nonspecific interstitial pneumonia: pathologic features and clinical implications. ( 30249370 )
2018
10
A Patient with Non-Hodgkin Lymphoma and Nonspecific Interstitial Pneumonia during Ibrutinib Therapy. ( 29259834 )
2017
11
Lower expression of platelet derived growth factor is associated with better overall survival rate of patients with idiopathic nonspecific interstitial pneumonia. ( 28449458 )
2017
12
Factors affecting treatment outcome in patients with idiopathic nonspecific interstitial pneumonia: a nationwide cohort study. ( 29212510 )
2017
13
Texture analysis using proton density and T2 relaxation in patients with histological usual interstitial pneumonia (UIP) or nonspecific interstitial pneumonia (NSIP). ( 28520778 )
2017
14
Diffuse large B-cell lymphoma of the lung in a patient with nonspecific interstitial pneumonia. ( 28867466 )
2017
15
Association between nonspecific interstitial pneumonia and presence of CD20+a88B lymphocytes within pulmonary lymphoid follicles. ( 29208971 )
2017
16
Coexistence of Autoantibodies against the Golgi Complex and Ro52 Antigen in a Patient with Nonspecific Interstitial Pneumonia. ( 26831023 )
2016
17
FDG-PET/CT in the prediction of pulmonary function improvement in nonspecific interstitial pneumonia. A Pilot Study. ( 27842667 )
2016
18
Confluent Fibrosis and Fibroblast Foci in Fibrotic Nonspecific Interstitial Pneumonia. ( 26864142 )
2016
19
Nonspecific interstitial pneumonia: time to be more specific? ( 27428647 )
2016
20
Nonspecific Interstitial Pneumonia: What Is the Optimal Approach to Management? ( 27231862 )
2016
21
Nonspecific interstitial pneumonia preceding diagnosis of collagen vascular disease. ( 27492512 )
2016
22
Organizing pneumonia components in non-specific interstitial pneumonia (NSIP): a clinicopathological study of 33 NSIP cases. ( 26100347 )
2016
23
Pulmonary Vein Stenosis Mimicking Nonspecific Interstitial Pneumonia. ( 26779359 )
2015
24
Efficacy of thrombomodulin for acute exacerbation of idiopathic pulmonary fibrosis and nonspecific interstitial pneumonia: a nonrandomized prospective study. ( 26566367 )
2015
25
Improvement in idiopathic nonspecific interstitial pneumonia after smoking cessation. ( 26029566 )
2015
26
Ultrasound in Rheumatologic Interstitial Lung Disease: A Case Report of Nonspecific Interstitial Pneumonia in Rheumatoid Arthritis. ( 26240772 )
2015
27
Nonspecific interstitial pneumonia overlaps organizing pneumonia in lung-dominant connective tissue disease. ( 26617847 )
2015
28
Nonspecific interstitial pneumonia: survival is influenced by the underlying cause. ( 25537566 )
2015
29
Prognosis of nonspecific interstitial pneumonia correlates with perivascular CD4+ T lymphocyte infiltration of the lung. ( 26496721 )
2015
30
Nonspecific interstitial pneumonia and usual interstitial pneumonia: comparison of the clinicopathologic features and prognosis. ( 25364525 )
2014
31
Prognostic relevance of histological variants in nonspecific interstitial pneumonia. ( 24621097 )
2014
32
Nonspecific interstitial pneumonia: clinical associations and outcomes. ( 25380997 )
2014
33
Hermansky-Pudlak syndrome with nonspecific interstitial pneumonia. ( 24583434 )
2014
34
Nonspecific Interstitial pneumonia (NSIP)/ Overlap or Distinct Entity: A case report from the National Research Institute of Tuberculosis and Lung Disease (NRITLD). ( 24778789 )
2014
35
Nonspecific interstitial pneumonia and usual interstitial pneumonia: is differentiation possible by high-resolution computed tomography? ( 24480140 )
2014
36
Pathologically proved nonspecific interstitial pneumonia: CT pattern analysis as compared with usual interstitial pneumonia CT pattern. ( 24661246 )
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
Proteomic analysis in usual and nonspecific interstitial pneumonia. ( 24048960 )
2013
43
CD4+ and CD8+ T lymphocytes in lung tissue of NSIP: correlation with T lymphocytes in BALF. ( 23085212 )
2013
44
Treatment response and long term follow-up results of nonspecific interstitial pneumonia. ( 22690098 )
2012
45
Increased lymphatic vessel length is associated with the fibroblast reticulum and disease severity in usual interstitial pneumonia and nonspecific interstitial pneumonia. ( 22797508 )
2012
46
A case of isolated IgG4-related interstitial pneumonia: a new consideration for the cause of idiopathic nonspecific interstitial pneumonia. ( 22796844 )
2012
47
Current status of idiopathic nonspecific interstitial pneumonia. ( 23001799 )
2012
48
Usual interstitial pneumonia coexisted with nonspecific interstitial pneumonia, What's the diagnosis? ( 23206520 )
2012
49
Nonspecific interstitial pneumonia. ( 22365250 )
2012
50
Infliximab-induced nonspecific interstitial pneumonia. ( 22688498 )
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 22)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
13.42 CCL2 CXCL5 CXCR3 IFNG IL13 TGFB1
2
Show member pathways
13.05 CCL2 CXCL5 CXCR3 IL13 TGFB1
3 12.56 IFNG IL13 SHH TGFB1
4
Show member pathways
12.04 CCL2 CXCL5 IFNG IL13
5 11.82 FOXF1 SHH TGFB1
6 11.77 CCL2 IFNG TGFB1
7
Show member pathways
11.77 CCL2 IFNG IL13 TGFB1
8
Show member pathways
11.74 CCL2 CXCL5 CXCR3
9 11.71 CCL2 IL13 TGFB1
10 11.51 CXCR3 IFNG IL13
11 11.42 CCL2 CXCL5 IFNG TGFB1
12 11.23 CCL2 IFNG TGFB1
13 11.11 CCL2 IFNG IL13 TGFB1
14 11.1 IFNG IL13
15 11.07 SHH TGFB1
16 10.97 CCL2 CXCL5 IL13
17 10.97 IFNG IL13
18 10.96 IFNG IL13 TGFB1
19 10.95 CCL2 IFNG
20 10.88 IFNG IL13 TGFB1
21 10.78 CCL2 CXCL5 IL13 TGFB1
22 10.72 CCL2 IL13 SFTPC TGFB1

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 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 33)
# Name GO ID Score Top Affiliating Genes
1 positive regulation of cell proliferation GO:0008284 9.92 CXCL5 IFNG SHH TGFB1
2 positive regulation of gene expression GO:0010628 9.85 IFNG IL13 SHH TGFB1
3 heart development GO:0007507 9.83 FOXF1 SHH TGFB1
4 angiogenesis GO:0001525 9.82 CCL2 CXCR3 SHH
5 chemotaxis GO:0006935 9.76 CCL2 CXCL5 CXCR3
6 negative regulation of T cell proliferation GO:0042130 9.66 SHH TGFB1
7 positive regulation of protein secretion GO:0050714 9.65 IL13 TGFB1
8 cell chemotaxis GO:0060326 9.65 CCL2 CXCL5 CXCR3
9 protein kinase B signaling GO:0043491 9.64 CCL2 TGFB1
10 lipopolysaccharide-mediated signaling pathway GO:0031663 9.64 CCL2 TGFB1
11 digestive tract development GO:0048565 9.63 FOXF1 TGFB1
12 positive regulation of protein import into nucleus GO:0042307 9.63 SHH TGFB1
13 pancreas development GO:0031016 9.62 FOXF1 SHH
14 oligodendrocyte development GO:0014003 9.61 SHH TGFB1
15 positive regulation of mesenchymal cell proliferation GO:0002053 9.61 FOXF1 SHH
16 positive regulation of protein complex assembly GO:0031334 9.6 IFNG TGFB1
17 renal system development GO:0072001 9.59 FOXF1 SHH
18 lung morphogenesis GO:0060425 9.58 FOXF1 SHH
19 chemokine-mediated signaling pathway GO:0070098 9.58 CCL2 CXCL5 CXCR3
20 embryonic digestive tract morphogenesis GO:0048557 9.57 FOXF1 SHH
21 epithelial tube branching involved in lung morphogenesis GO:0060441 9.56 FOXF1 SHH
22 immune response GO:0006955 9.55 CCL2 CXCL5 CXCR3 IFNG IL13
23 regulation of regulatory T cell differentiation GO:0045589 9.54 IFNG TGFB1
24 cellular response to organic cyclic compound GO:0071407 9.54 CCL2 FOXF1 TGFB1
25 negative regulation of interleukin-17 production GO:0032700 9.52 IFNG TGFB1
26 morphogenesis of a branching structure GO:0001763 9.51 FOXF1 TGFB1
27 vasculogenesis GO:0001570 9.5 FOXF1 SHH TGFB1
28 embryonic foregut morphogenesis GO:0048617 9.49 FOXF1 SHH
29 lung lobe morphogenesis GO:0060463 9.46 FOXF1 SHH
30 respiratory tube development GO:0030323 9.43 FOXF1 SHH
31 inflammatory response GO:0006954 9.35 CCL2 CXCL5 CXCR3 IL13 TGFB1
32 trachea development GO:0060438 9.26 FOXF1 SHH
33 regulation of signaling receptor activity GO:0010469 9.1 CCL2 CXCL5 IFNG IL13 SHH TGFB1

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 IFNG IL13 TGFB1

Sources for Nonspecific Interstitial Pneumonia

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
20 FMA
29 GO
30 GTR
31 HGMD
32 HMDB
33 HPO
34 ICD10
35 ICD10 via Orphanet
36 ICD9CM
37 IUPHAR
38 KEGG
39 LifeMap
41 LOVD
43 MedGen
45 MeSH
46 MESH via Orphanet
47 MGI
50 NCI
51 NCIt
52 NDF-RT
55 NINDS
56 Novoseek
58 OMIM
59 OMIM via Orphanet
63 PubMed
65 QIAGEN
70 SNOMED-CT via HPO
71 SNOMED-CT via Orphanet
72 TGDB
73 Tocris
74 UMLS
75 UMLS via Orphanet
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