NSIP
MCID: NNS002
MIFTS: 42

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 70
Nsip 12 58
Non-Specific Idiopathic Interstitial Pneumonia 58
Non-Specific Interstitial Pneumonia Nos 70
Non-Specific Interstitial Pneumonia 58

Classifications:

Orphanet: 58  
Rare respiratory diseases


External Ids:

Disease Ontology 12 DOID:2801
NCIt 50 C35717
SNOMED-CT 67 129452008
ICD10 via Orphanet 33 J84.8
UMLS via Orphanet 71 C1290344
Orphanet 58 ORPHA91364
UMLS 70 C1290344 C3161437

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 surfactant metabolism dysfunction, pulmonary, 1 and idiopathic interstitial pneumonia. An important gene associated with Nonspecific Interstitial Pneumonia is SFTPC (Surfactant Protein C), and among its related pathways/superpathways are Akt Signaling and Cytokine Signaling in Immune system. Affiliated tissues include lung, myeloid and eye, and related phenotypes are immune system and mortality/aging

Wikipedia : 73 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 228)
# Related Disease Score Top Affiliating Genes
1 surfactant metabolism dysfunction, pulmonary, 1 31.7 SFTPC ABCA3
2 idiopathic interstitial pneumonia 31.5 TGFB1 SFTPD SFTPC MUC5B IL13 IFNG
3 cryptogenic organizing pneumonia 31.0 SFTPD SFTPC MUC1
4 bronchiolitis 30.8 SFTPD IL13 IFNG CCL2
5 pneumonia 30.8 SFTPD SFTPC MUC5B MUC1 IL13 IFNG
6 extrinsic allergic alveolitis 30.7 SFTPD CXCR3 CXCL5 CCL2
7 connective tissue disease 30.4 SFTPD SERPINH1 MUC1 MIR21 IFNG CCL2
8 bronchiolitis obliterans 30.4 TGFB1 SFTPD IL13 IFNG CXCR3
9 vascular disease 30.4 TGFB1 MIR21 IFNG FGF2 CCL2
10 pleuroparenchymal fibroelastosis 30.3 SFTPD MUC1
11 pulmonary hypertension 30.2 SFTPC MIR21 FOXF1 FGF2 CCL2
12 pulmonary fibrosis 30.2 TGFB1 SFTPD SFTPC MUC5B IL13 HPS1
13 arthritis 30.1 TGFB1 SERPINH1 IL13 IFNG CXCL5 CCL2
14 interstitial pneumonitis, desquamative, familial 30.1 SFTPD SFTPC MUC5B MUC1 ABCA3
15 eosinophilic pneumonia 29.9 SFTPD MUC1 IL13 CCR4
16 respiratory failure 29.9 SFTPD SFTPC IL13 CCL2 ABCA3
17 acute interstitial pneumonia 29.9 SFTPD SFTPC MUC1 IFIH1 ABCA3
18 sarcoidosis 1 29.8 SFTPD IL13 IFNG CXCR3 CXCL5 CCL2
19 rheumatoid arthritis 29.7 TGFB1 SERPINH1 IL13 IFNG CXCR3 CXCL5
20 systemic scleroderma 29.7 TRIM21 TGFB1 SFTPD IL13 IFNG
21 exanthem 29.5 TRIM21 IFNG IFIH1
22 autoimmune disease 29.3 TRIM21 TGFB1 IL13 IFNG IFIH1 CXCR3
23 antisynthetase syndrome 29.2 TRIM21 MUC5B IFIH1
24 psoriasis 29.2 MIR21 IL13 IFNG IFIH1 CXCR3 CCR4
25 lung disease 28.9 TGFB1 SFTPD SFTPC MUC5B MUC1 MIR21
26 pulmonary fibrosis, idiopathic 28.7 TGFB1 SFTPD SFTPC SERPINH1 MUC5B MUC1
27 interstitial lung disease 28.7 TRIM21 TGFB1 SFTPD SFTPC MUC5B MUC1
28 bronchiectasis 10.6
29 fibrosis of extraocular muscles, congenital, 1 10.4
30 dermatomyositis 10.4
31 keloid disorder 10.4 TGFB1 SERPINH1
32 fuchs' heterochromic uveitis 10.3 IFNG CCL2
33 fixed drug eruption 10.3 IFNG CCR4
34 conjunctivitis 10.3 IL13 IFNG CCL2
35 chronic beryllium disease 10.3 TGFB1 IFNG
36 viral meningitis 10.3 IFNG CXCR3 CCL2
37 proliferative glomerulonephritis 10.3 IFNG CXCR3 CCL2
38 strongyloidiasis 10.3 TGFB1 IL13 IFNG
39 scleroderma, familial progressive 10.3
40 polymyositis 10.3
41 pulmonary fibrosis predisposition 10.3 SFTPC MUC5B
42 paranasal sinus disease 10.3 TGFB1 IL13 IFNG
43 pelvic inflammatory disease 10.3 IL13 IFNG CCL2
44 brucellosis 10.3 TGFB1 IL13 IFNG
45 intermediate uveitis 10.3 IFNG CXCR3 CCR4
46 pleurisy 10.3 TGFB1 IFNG CCL2
47 cutaneous leishmaniasis 10.2 IL13 IFNG CCL2
48 pustulosis palmaris et plantaris 10.2 TGFB1 CXCR3 CCR4
49 localized pulmonary fibrosis 10.2 SFTPD MUC1
50 respiratory distress syndrome, infant 10.2 SFTPC ABCA3

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 immune system MP:0005387 10.1 CCL2 CCR4 CXCR3 FOXF1 HPS1 IFIH1
2 mortality/aging MP:0010768 10.03 ABCA3 CXCR3 FGF2 FOXF1 HPS1 IFIH1
3 renal/urinary system MP:0005367 9.56 ABCA3 CXCR3 HPS1 IFIH1 IFNG SHH
4 respiratory system MP:0005388 9.44 ABCA3 CCR4 CXCR3 FOXF1 HPS1 IFNG

Drugs & Therapeutics for Nonspecific Interstitial Pneumonia

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Case-Control Study of Fibrotic Interstitial Lung Disease and it's Association With Cardiac Disease or Cardiac Intervention. Unknown status NCT01154998
2 Creation of a Biospecimen Repository From Patients With Interstitial Lung Diseases (ILD) Recruiting NCT03478826
3 Molecular Diagnosis of Idiopathic Interstitial Pneumonias: a Prospective Study Recruiting NCT03836417

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:

40
Lung, Myeloid, Eye, Lymph Node, Monocytes, Endothelial, Bone Marrow

Publications for Nonspecific Interstitial Pneumonia

Articles related to Nonspecific Interstitial Pneumonia:

(show top 50) (show all 981)
# Title Authors PMID Year
1
Usual interstitial pneumonia progressing to nonspecific interstitial pneumonia-like pattern on high-resolution CT with histologic confirmation. 61
33680269 2021
2
A comprehensive assessment of environmental exposures and the medical history guides multidisciplinary discussion in interstitial lung disease. 61
33676119 2021
3
Analysis of Clinical-Analytical Characteristics in Patients with Rheumatoid Arthritis and Interstitial Lung Disease: Case-Control Study. 61
31474500 2021
4
Radiographic features and poor prognostic factors of interstitial lung disease with nivolumab for non-small cell lung cancer. 61
33098725 2021
5
The Significance of Subpleural Sparing in CT Chest: A State-of-the-Art Review. 61
33487401 2021
6
[Hypersensitivity pneumonia diagnosed by excisional lung biopsy: a clinicopathological features of 47 cases]. 61
33677886 2021
7
Chest CT Diagnosis and Clinical Management of Drug-Related Pneumonitis in Patients Receiving Molecular Targeting Agents and Immune Checkpoint Inhibitors: A Position Paper From the Fleischner Society. 61
33450293 2021
8
Chest CT Diagnosis and Clinical Management of Drug-related Pneumonitis in Patients Receiving Molecular Targeting Agents and Immune Checkpoint Inhibitors: A Position Paper from the Fleischner Society. 61
33434111 2021
9
Rheumatoid arthritis related interstitial lung disease. 61
33779447 2021
10
[Connective tissue disease-related interstitial lung disease]. 61
33689246 2021
11
Effectiveness of pirfenidone for idiopathic pulmonary fibrosis associated with pleuroparenchymal fibroelastosis-like lesions and nonspecific interstitial pneumonia. 61
33058535 2021
12
Multidisciplinary team obviates biopsy in most patients with diffuse parenchymal lung diseases-A retrospective study from India. 61
33721404 2021
13
Myositis-associated Interstitial Lung Disease: Clinical Characteristics and Factors Related to Pulmonary Function Improvement: A Latin-American Multicenter Cohort Study. 61
33602594 2021
14
Predictive Features and Clinical Presentation of Interstitial Lung Disease in Inflammatory Myositis. 61
33141387 2021
15
Impact of interstitial lung disease associated with immune checkpoint inhibitors on prognosis in patients with non-small-cell lung cancer. 61
33394102 2021
16
Single Nucleotide Polymorphism in the IL17A Gene Is Associated with Interstitial Lung Disease Positive to Anti-Jo1 Antisynthetase Autoantibodies. 61
33672430 2021
17
Content-Based Image Retrieval of Chest CT with Convolutional Neural Network for Diffuse Interstitial Lung Disease: Performance Assessment in Three Major Idiopathic Interstitial Pneumonias. 61
33169547 2021
18
[Drug-induced interstitial lung diseases]. 61
33415347 2021
19
Central paradiaphragmatic middle lobe involvement in nonspecific interstitial pneumonia. 61
33624164 2021
20
[Radiological features of interstitial lung diseases]. 61
33496812 2021
21
Hairspray Inhalation-induced Interstitial Pneumonitis Evaluated by a Transbronchial Lung Cryobiopsy. 61
33612669 2021
22
Low dose computed tomography of the lung for detection and grading of interstitial lung disease: A systematic simulation study. 61
32591280 2021
23
Interstitial lung abnormality (ILA) and nonspecific interstitial pneumonia (NSIP). 61
33796637 2021
24
Computed tomography findings of current nonspecific interstitial pneumonia based on the 2013 updated classification of idiopathic interstitial pneumonias: What is a characteristic of previously diagnosed nonspecific interstitial pneumonia excluded from the updated classification. 61
32875470 2021
25
Interstitial lung disease in patients with antisynthetase syndrome: a retrospective case series study. 61
32876818 2021
26
Better characterization of operation for ulcerative colitis through the National surgical quality improvement program: A 2-year audit of NSQIP-IBD. 61
32928540 2021
27
Development of Two Types of Skin Cancer in a Patient with Systemic Sclerosis: a Case Report and Overview of the Literature. 61
33728079 2021
28
Duration of rheumatoid arthritis and the risk of developing interstitial lung disease. 61
33644223 2021
29
Predictors of mortality in rheumatoid arthritis-associated lung disease: A retrospective study on ten years. 61
33484860 2021
30
Asymptomatic necrotizing myositis in a young male with progressive interstitial lung disease. 61
33747761 2021
31
Connective tissue disease-related interstitial lung disease (CTD-ILD) and interstitial lung abnormality (ILA): Evolving concept of CT findings, pathology and management. 61
33364263 2021
32
Exhaled breath analysis by use of eNose technology: a novel diagnostic tool for interstitial lung disease. 61
32732331 2021
33
Pathological Insight into 5-HT2B Receptor Activation in Fibrosing Interstitial Lung Diseases. 61
33379351 2020
34
Spectrum of Pulmonary Fibrosis from Interstitial Lung Abnormality to Usual Interstitial Pneumonia: Importance of Identification and Quantification of Traction Bronchiectasis in Patient Management. 61
33543848 2020
35
Characterization of Interstitial Lung Disease Associated With Anti-Ribonucleoprotein Antibodies. 61
31415476 2020
36
Abatacept in interstitial lung disease associated with rheumatoid arthritis: national multicenter study of 263 patients. 61
33068439 2020
37
Clinicopathological characteristics of lung cancer in patients with systemic sclerosis. 61
32770829 2020
38
[New developments in systemic sclerosis-associated interstitial lung disease]. 61
33206479 2020
39
Galactin-1, 3 and 9: Potential biomarkers in idiopathic pulmonary fibrosis and other interstitial lung diseases. 61
32920057 2020
40
Assessment and Management of Occupational Hypersensitivity Pneumonitis. 61
33161960 2020
41
[Day/Night Variability of Coughing Events in Interstitial Lung Disease]. 61
33147638 2020
42
Clinical, radiological, and pathological evaluation of "NSIP with OP overlap" pattern compared with NSIP in patients with idiopathic interstitial pneumonias. 61
33120192 2020
43
Diverse Pathological Findings of Interstitial Lung Disease in a Patient with Dyskeratosis Congenita. 61
33191321 2020
44
Evaluation of efficacy and safety of rituximab in combination with mycophenolate mofetil in patients with nonspecific interstitial pneumonia non-responding to a first-line immunosuppressive treatment (EVER-ILD): A double-blind placebo-controlled randomized trial. 61
32777737 2020
45
The Trouble With Group 3 Pulmonary Hypertension in Interstitial Lung Disease: Dilemmas in Diagnosis and the Conundrum of Treatment. 61
32387520 2020
46
Clinical utility of a composite scoring system including Charlson Comorbidity Index score in patients with interstitial lung disease. 61
33209409 2020
47
[Pirfenidone Inhibits Cytokines/chemokines Release from Alveolar Macrophages]. 61
33131513 2020
48
Clinical significance of radiological patterns of HRCT and their association with macrophage activation in dermatomyositis. 61
32065646 2020
49
The potential utility of anterior upper lobe honeycomb-like lesion in interstitial lung disease associated with connective tissue disease. 61
32911135 2020
50
Heme oxygenase-1 deficiency presenting with interstitial lung disease and hemophagocytic flares. 61
33066778 2020

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 21)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
13.28 TGFB1 IL13 FGF2 CXCR3 CXCL5 CCR4
2
Show member pathways
13.2 TRIM21 TGFB1 MUC1 IL13 IFNG FGF2
3
Show member pathways
13.11 TGFB1 IL13 FGF2 CXCR3 CXCL5 CCR4
4 12.7 TGFB1 SHH IL13 IFNG FGF2
5
Show member pathways
12.17 CXCR3 CXCL5 CCR4 CCL2
6 12.12 TGFB1 SHH MIR21 FGF2
7
Show member pathways
11.89 MUC5B IL13 IFNG CXCL5 CCL2
8 11.83 TGFB1 SHH FOXF1 FGF2
9 11.71 TGFB1 FGF2 CCL2
10 11.64 IL13 IFNG CXCR3
11 11.53 TGFB1 IFNG CXCL5 CCL2
12 11.51 TGFB1 SERPINH1 FGF2
13 11.41 TGFB1 MUC1 IL13 FGF2 CCL2
14 11.35 TGFB1 IFNG CCL2
15 11.31 TGFB1 SHH FGF2
16
Show member pathways
11.24 SFTPD SFTPC ABCA3
17 11.01 TGFB1 IL13 IFNG
18 10.94 TGFB1 IL13 IFNG
19 10.9 TGFB1 IL13 FGF2 CXCL5 CCL2
20 10.9 TGFB1 SFTPC MUC5B IL13 FGF2 CCL2
21 10.65 TGFB1 IL13

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 9.7 TGFB1 SHH SFTPD SFTPC MUC5B MUC1
2 extracellular space GO:0005615 9.47 TGFB1 SHH SFTPD SFTPC SERPINH1 MUC5B
3 alveolar lamellar body GO:0097208 9.16 SFTPC ABCA3

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

(show all 27)
# Name GO ID Score Top Affiliating Genes
1 angiogenesis GO:0001525 9.93 SHH FGF2 CXCR3 CCL2
2 positive regulation of gene expression GO:0010628 9.93 TGFB1 SHH MIR21 IL13 IFNG FGF2
3 positive regulation of ERK1 and ERK2 cascade GO:0070374 9.91 TGFB1 MIR21 FGF2 CCL2
4 negative regulation of gene expression GO:0010629 9.91 TGFB1 SHH MIR21 IFNG FGF2
5 immune response GO:0006955 9.91 IL13 IFNG CXCR3 CXCL5 CCR4 CCL2
6 positive regulation of protein phosphorylation GO:0001934 9.9 TGFB1 MIR21 IFNG FGF2
7 cytokine-mediated signaling pathway GO:0019221 9.88 TGFB1 MUC1 IL13 FGF2 CCL2
8 inflammatory response GO:0006954 9.8 TGFB1 IL13 CXCR3 CXCL5 CCR4 CCL2
9 cellular response to organic cyclic compound GO:0071407 9.79 TGFB1 FOXF1 CCL2
10 vasculogenesis GO:0001570 9.77 TGFB1 SHH FOXF1
11 positive regulation of cell division GO:0051781 9.73 TGFB1 SHH FGF2
12 negative regulation of T cell proliferation GO:0042130 9.72 TGFB1 SHH SFTPD
13 positive regulation of protein import into nucleus GO:0042307 9.71 TGFB1 SHH IFNG
14 lung development GO:0030324 9.71 SHH FOXF1 FGF2 ABCA3
15 lung alveolus development GO:0048286 9.67 TGFB1 SFTPD FOXF1
16 cell chemotaxis GO:0060326 9.67 CXCR3 CXCL5 CCR4 CCL2
17 macrophage chemotaxis GO:0048246 9.63 SFTPD CCL2
18 morphogenesis of a branching structure GO:0001763 9.62 TGFB1 FOXF1
19 embryonic foregut morphogenesis GO:0048617 9.61 SHH FOXF1
20 positive regulation of NMDA glutamate receptor activity GO:1904783 9.58 IFNG CCL2
21 lung lobe morphogenesis GO:0060463 9.58 SHH FOXF1
22 respiratory tube development GO:0030323 9.57 SHH FOXF1
23 chemokine-mediated signaling pathway GO:0070098 9.56 CXCR3 CXCL5 CCR4 CCL2
24 chemotaxis GO:0006935 9.55 FGF2 CXCR3 CXCL5 CCR4 CCL2
25 trachea development GO:0060438 9.52 SHH FOXF1
26 positive regulation of cell proliferation GO:0008284 9.5 TGFB1 SHH MIR21 IFNG FGF2 CXCR3
27 surfactant homeostasis GO:0043129 8.8 TGFB1 SFTPD ABCA3

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 9.1 TGFB1 IL13 IFNG FGF2 CXCL5 CCL2
2 chemokine binding GO:0019956 8.96 FGF2 CXCR3

Sources for Nonspecific Interstitial Pneumonia

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
20 GARD
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
53 NINDS
54 Novoseek
56 OMIM via Orphanet
57 OMIM® (Updated 20-May-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 Tocris
70 UMLS
71 UMLS via Orphanet
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