LIP
MCID: LYM004
MIFTS: 51

Lymphoid Interstitial Pneumonia (LIP)

Categories: Immune diseases, Oral diseases, Rare diseases, Respiratory diseases

Aliases & Classifications for Lymphoid Interstitial Pneumonia

MalaCards integrated aliases for Lymphoid Interstitial Pneumonia:

Name: Lymphoid Interstitial Pneumonia 56 12 58 15 71
Lip Diseases 43 71
Lip Disease 12 15
Lymphocytic Interstitial Pneumonia 58
Disease of Lips 12
Lip 56

Characteristics:

Orphanet epidemiological data:

58
lymphoid interstitial pneumonia
Inheritance: Not applicable; Age of onset: All ages;

OMIM:

56
Inheritance:
autosomal recessive


HPO:

31
lymphoid interstitial pneumonia:
Inheritance autosomal recessive inheritance


Classifications:

Orphanet: 58  
Rare respiratory diseases


External Ids:

Disease Ontology 12 DOID:0050159 DOID:9297
OMIM 56 247610
ICD9CM 34 528.5
MeSH 43 D008047
NCIt 49 C26818
SNOMED-CT 67 90678009
ICD10 32 K13.0
ICD10 via Orphanet 33 J84.1
UMLS via Orphanet 72 C0264511
Orphanet 58 ORPHA79128
MedGen 41 C0264511
UMLS 71 C0023760 C0264511

Summaries for Lymphoid Interstitial Pneumonia

Disease Ontology : 12 An idiopathic interstitial pneumonia which involves diffuse interstitial infiltration of involved areas mostly with T lymphocytes, plasma cells, and macrophages. Lymphoid hyperplasia is frequently seen. Onset is often slow with gradually increasing cough and breathlessness over 3 or more years. Fever, weight loss, chest pain, and arthralgia are occasionally found. Crackles may be detected as the disease progresses.

MalaCards based summary : Lymphoid Interstitial Pneumonia, also known as lip diseases, is related to van der woude syndrome 1 and cleft lip and alveolus, and has symptoms including oral manifestations and cheilodynia. An important gene associated with Lymphoid Interstitial Pneumonia is CRP (C-Reactive Protein), and among its related pathways/superpathways are Innate Immune System and Cytokine Signaling in Immune system. The drugs Midazolam and Lidocaine have been mentioned in the context of this disorder. Affiliated tissues include lung, salivary gland and skin, and related phenotypes are aortic valve stenosis and clubbing

More information from OMIM: 247610

Related Diseases for Lymphoid Interstitial Pneumonia

Diseases related to Lymphoid Interstitial Pneumonia via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 2094)
# Related Disease Score Top Affiliating Genes
1 van der woude syndrome 1 34.7 NHLH1 MSX1 IRF6
2 cleft lip and alveolus 34.6 MSX1 IRF6
3 popliteal pterygium syndrome 34.2 NHLH1 MSX1 IRF6
4 branchiooculofacial syndrome 34.2 NHLH1 MSX1 IRF6
5 cleft palate, isolated 33.8 TMEM54 NHLH1 MSX1 LIG3 IRF6
6 kawasaki disease 33.2 TNF IL6 CRP CD40LG CD4
7 stomatitis 32.8 TNF IL6 CD40LG CD4
8 ectodermal dysplasia 10b, hypohidrotic/hair/tooth type, autosomal recessive 32.7 TNF TGFB1 IL6 CD8A CD4
9 orofacial cleft 32.7 TMEM54 NHLH1 MSX1 MIR3200 IRF6
10 syphilis 32.4 TNF PRTN3 IL6 CRP CD8A CD40LG
11 raynaud disease 32.2 TRIM21 CRP CD40LG
12 sebaceous gland disease 32.1 TNF IL6 CRP
13 pneumonia 32.0 TNF IL6 CRP CD8A CD40LG
14 hair disease 32.0 TNF IL6 CD8A CD40LG CD4
15 ectodermal dysplasia 1, hypohidrotic, x-linked 32.0 TNF TGFB1 IL6 CD8A CD4
16 potocki-shaffer syndrome 31.8 TRIM21 TNF IL6 CRP CD8A CD40LG
17 behcet syndrome 31.8 TNF PRTN3 IL6 CRP CD8A CD40LG
18 antiphospholipid syndrome 31.7 TNF CRP CD40LG
19 otitis media 31.4 TNF IL6 CRP CD40LG
20 facial paralysis 31.3 TGFB1 PRTN3 CRP CD8A CD40LG CD4
21 tooth agenesis 31.3 TNF TMEM54 NHLH1 MSX1 IRF6
22 contact dermatitis 31.3 TNF IL6 CD4
23 leishmaniasis 31.2 TNF TGFB1 IL6 CRP
24 castleman disease 31.1 PPL IL6 CRP
25 sarcoidosis 1 31.1 TNF CRP CD8A CD40LG CD4
26 bronchiolitis 31.1 TNF IL6 CRP CD40LG
27 allergic contact dermatitis 31.1 TNF IL6 CD4
28 sialadenitis 31.1 TRIM21 TNF CD8A CD4
29 pemphigus 31.0 TNF PPL IL6 CD40LG
30 urticaria 31.0 TNF IL6 CRP CD40LG
31 acquired immunodeficiency syndrome 31.0 TNF IL6 CRP CD40LG CD4
32 fasciitis 30.9 TNF IL6 CRP
33 respiratory failure 30.9 TNF IL6 CRP CD40LG CD4
34 igg4-related disease 30.9 PRTN3 CRP CD4
35 autoimmune disease 30.9 TRIM21 TNF TGFB1 PRTN3 IL6 CRP
36 bell's palsy 30.9 CD8A CD40LG CD4
37 cellulitis 30.9 TNF IL6 CRP CD8A CD40LG CD4
38 anemia, autoimmune hemolytic 30.9 CD8A CD40LG CD4
39 polyclonal hypergammaglobulinemia 30.8 TRIM21 IL6 CRP CD40LG CD4 CD1E
40 neuroretinitis 30.8 CRP CD40LG CD4
41 funisitis 30.8 TNF IL6 CRP
42 lung disease 30.8 TNF TGFB1 TERT PRTN3 IL6 CRP
43 miliary tuberculosis 30.8 TNF CRP CD8A CD40LG CD4
44 secondary syphilis 30.8 CD8A CD40LG CD4
45 rheumatoid arthritis 30.8 TNF TGFB1 PRTN3 IL6 CRP CD40LG
46 skin disease 30.8 TNF IL6 CRP CD8A CD40LG CD4
47 chlamydia 30.8 TNF IL6 CRP CD40LG
48 middle ear disease 30.8 TNF PRTN3 IL6 CRP CD8A CD40LG
49 gastroesophageal reflux 30.7 TNF TGFB1 TERT IL6
50 maxillary sinusitis 30.7 PRTN3 CRP CD40LG

Comorbidity relations with Lymphoid Interstitial Pneumonia via Phenotypic Disease Network (PDN):


Acute Cystitis Deficiency Anemia

Graphical network of the top 20 diseases related to Lymphoid Interstitial Pneumonia:



Diseases related to Lymphoid Interstitial Pneumonia

Symptoms & Phenotypes for Lymphoid Interstitial Pneumonia

Human phenotypes related to Lymphoid Interstitial Pneumonia:

31
# Description HPO Frequency HPO Source Accession
1 aortic valve stenosis 31 HP:0001650
2 clubbing 31 HP:0001217
3 lymphoid interstitial pneumonia 31 HP:0006527
4 cor pulmonale 31 HP:0001648

Symptoms via clinical synopsis from OMIM:

56
Pulmonary:
lymphoid interstitial pneumonia

Limbs:
digital clubbing

Cardiac:
cor pulmonale
aortic stenosis

Clinical features from OMIM:

247610

UMLS symptoms related to Lymphoid Interstitial Pneumonia:


oral manifestations, cheilodynia

MGI Mouse Phenotypes related to Lymphoid Interstitial Pneumonia:

45
# Description MGI Source Accession Score Top Affiliating Genes
1 immune system MP:0005387 9.93 CD4 CD40LG CD8A CRP IL6 NHLH1
2 integument MP:0010771 9.7 CD4 CD40LG CD8A IGFBP3 IL6 IRF6
3 nervous system MP:0003631 9.36 CD4 CD40LG CD8A IGFBP3 IL6 LIG3

Drugs & Therapeutics for Lymphoid Interstitial Pneumonia

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

(show all 7)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Midazolam Approved, Illicit 59467-70-8 4192
2
Lidocaine Approved, Vet_approved 137-58-6 3676
3 Immunoglobulins
4 Antibodies
5 Anesthetics
6 Pharmaceutical Solutions
7 Calamus

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 A Dose-Ranging Study of the Efficacy and Safety of Bardoxolone Methyl in Patients With Pulmonary Hypertension Completed NCT02036970 Phase 2 Bardoxolone methyl;Placebo
2 The European IPF Registry - an Internet-based, Pan-European Registry Linked to the European IPF Biobank (eurIPFbank) Recruiting NCT02951416
3 Granulomatous-Lymphocytic Interstitial Lung Disease (GLILD) Diagnosed in Children and Young Adults With Common Variable Immunodeficiency Recruiting NCT03648567
4 Telemonitoring as a Tool for the Assessment of Treatment Effects of Connective Tissue Disease-associated Interstitial Lung Disease (TEL-CTD-ILD) Recruiting NCT04428957
5 CXCL10 As a Biomarker Of Interstitial Lung Disease in Patients With Rheumatoid Arthritis Not yet recruiting NCT04356066
6 Incidence, Clinical Characteristics and Evaluation of Prognostic and Diagnostic Markers of Interstitial Pneumonia With Autoimmune Features (IPAF) - a Multicenter Prospective Study Not yet recruiting NCT03870828

Search NIH Clinical Center for Lymphoid Interstitial Pneumonia

Cochrane evidence based reviews: lip diseases

Genetic Tests for Lymphoid Interstitial Pneumonia

Anatomical Context for Lymphoid Interstitial Pneumonia

MalaCards organs/tissues related to Lymphoid Interstitial Pneumonia:

40
Lung, Salivary Gland, Skin, Bone, T Cells, Bone Marrow, B Cells

Publications for Lymphoid Interstitial Pneumonia

Articles related to Lymphoid Interstitial Pneumonia:

(show top 50) (show all 235)
# Title Authors PMID Year
1
Familial lymphoid interstitial pneumonia: a long-term follow-up. 61 56
7360539 1980
2
Dermatologist-level classification of malignant lip diseases using a deep convolutional neural network. 61
31449661 2020
3
Rituximab effect in severe progressive connective tissue disease-related lung disease: preliminary data. 61
32157369 2020
4
Lung disease in patients with common variable immunodeficiency. 61
32446785 2020
5
EBV-associated lymphoid interstitial pneumonia in IBD patient: Case report and literature review. 61
32373453 2020
6
Aspergilloma Superimposed Infection on Lymphoid Interstitial Pneumonia. 61
32082646 2020
7
Diffuse Cystic Lung Diseases. 61
31615921 2020
8
Characterization of Interstitial Lung Disease Associated With Anti-Ribonucleoprotein Antibodies. 61
31415476 2019
9
Thoracic Manifestations of Connective Tissue Diseases. 61
31200872 2019
10
Lymphoid interstitial pneumonia in a patient with rheumatoid arthritis. 61
31506703 2019
11
Pulmonary and Bronchiolar Involvement in Sjogren's Syndrome. 61
31137063 2019
12
Successful treatment of isolated oral lichen planus on lower lip with traditional Chinese medicine and topical wet dressing: A case report. 61
30558050 2018
13
Lung histopathology of non-infectious pulmonary complications after allogeneic haematopoietic stem cell transplantation. 61
29953629 2018
14
Idiopathic interstitial pneumonias: review of the latest American Thoracic Society/European Respiratory Society classification. 61
30369660 2018
15
Complete Resolution of Lymphoid Interstitial Pneumonia in a Patient With Juvenile Myelomonocytic Leukemia Treated With Allogeneic Bone Marrow Transplant: Killing 2 Birds With 1 Stone. 61
29023302 2018
16
Lymphoid Interstitial Pneumonia in Common Variable Immune Deficiency - Case Report With Disease Monitoring in Various Therapeutic Options: Pleiotropic Effects of Rituximab Regimens. 61
30713498 2018
17
A case report of lymphoid intestitial pneumonia in common variable immunodeficiency: Oligoclonal expansion of effector lymphocytes with preferential cytomegalovirus-specific immune response and lymphoproliferative disease promotion. 61
28591035 2017
18
Verruciform Xanthoma of a Lower Lip Lesion: A New Case and Review of the Literature. 61
29033816 2017
19
Additive Manufacturing Techniques for the Reconstruction of 3D Fetal Faces. 61
29410600 2017
20
Imaging of Pulmonary Manifestations of Connective Tissue Diseases. 61
27719973 2016
21
Cystic Lung Diseases: Algorithmic Approach. 61
27180915 2016
22
Diffuse Cystic Lung Diseases: Diagnostic Considerations. 61
27231867 2016
23
Lymphoid Interstitial Pneumonia and Other Benign Lymphoid Disorders. 61
27231864 2016
24
Rare idiopathic interstitial pneumonias: LIP and PPFE and rare histologic patterns of interstitial pneumonias: AFOP and BPIP. 61
26627191 2016
25
Diffuse Cystic Lung Disease as the Presenting Manifestation of Sjögren Syndrome. 61
26741500 2016
26
Early Bronchus-Associated Lymphoid Tissue Lymphoma Diagnosed with Immunoglobulin Heavy Chain Molecular Testing. 61
27445561 2016
27
Primary Pulmonary Lymphoid Lesions: Radiologic and Pathologic Findings. 61
26761531 2016
28
[Lung disease in adult common variable immunodeficiency]. 61
26071128 2015
29
A 15-year retrospective study of 160 cases of benign lip lesions. 61
26654640 2015
30
American Thoracic Society-European Respiratory Society Classification of the Idiopathic Interstitial Pneumonias: Advances in Knowledge since 2002. 61
26452110 2015
31
Rare idiopathic intestinal pneumonias (IIPs) and histologic patterns in new ATS/ERS multidisciplinary classification of the IIPs. 61
25591580 2015
32
Pathology of Idiopathic Interstitial Pneumonias. 61
26949346 2015
33
Idiopathic interstitial pneumonias: a radiology-pathology correlation based on the revised 2013 American Thoracic Society-European Respiratory Society classification system. 61
25512168 2015
34
Ultrasound in Rheumatologic Interstitial Lung Disease: A Case Report of Nonspecific Interstitial Pneumonia in Rheumatoid Arthritis. 61
26240772 2015
35
Prognostic relevance of histological variants in nonspecific interstitial pneumonia. 61
24621097 2014
36
Late-onset noninfectious interstitial lung disease after allogeneic hematopoietic stem cell transplantation. 61
25260979 2014
37
Chronic Epstein-Barr virus infection causing both benign and malignant lymphoproliferative disorders. 61
25324869 2014
38
The lip in lupus erythematosus. 61
24934909 2014
39
The pulmonary histopathologic manifestations of the anti-PL7/antithreonyl transfer RNA synthetase syndrome. 61
24767252 2014
40
Clinical and radiological features of idiopathic interstitial pneumonias (IIPs): a pictorial review. 61
24844883 2014
41
Spatial fuzzy c-means algorithm with adaptive fuzzy exponent selection for robust vermilion border detection in healthy and diseased lower lips. 61
24661607 2014
42
Clinical manifestations and pulmonary histopathological analysis related to different diseases in patients with fatal pulmonary thromboembolism: an autopsy study. 61
27147874 2014
43
Lymphangioleiomyomatosis: differential diagnosis and optimal management. 61
25187723 2014
44
CT findings of thoracic manifestations of primary Sjögren syndrome: radiologic-pathologic correlation. 61
24224588 2013
45
Imaging manifestations of autoimmune disease-associated lymphoproliferative disorders of the lung. 61
23728499 2013
46
Diffuse cystic lung diseases. 61
23666611 2013
47
Lung miliary micro-nodules in human T-cell leukemia virus type I carriers. 61
23464968 2013
48
A Case of Multicentric Castleman's Disease Presenting with Follicular Bronchiolitis. 61
23390449 2013
49
Rare case of idiopathic lymphocytic interstitial pneumonia exhibits good response to Mycophenolate Mofetil. 61
26029625 2013
50
Update for pathologists on idiopathic interstitial pneumonias. 61
23020729 2012

Variations for Lymphoid Interstitial Pneumonia

Expression for Lymphoid Interstitial Pneumonia

Search GEO for disease gene expression data for Lymphoid Interstitial Pneumonia.

Pathways for Lymphoid Interstitial Pneumonia

Pathways related to Lymphoid Interstitial Pneumonia according to GeneCards Suite gene sharing:

(show all 19)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
13.92 TRIM21 TNF TGFB1 PRTN3 PPL IRF6
2
Show member pathways
13.25 TRIM21 TNF TGFB1 PRTN3 IRF6 IL6
3
Show member pathways
12.73 TNF TGFB1 IL6 CD8A CD40LG CD4
4
Show member pathways
12.45 TNF TGFB1 IL6 CD8A CD40LG CD4
5
Show member pathways
12.17 TNF TGFB1 TERT IRF6 IL6
6 12.07 TNF TGFB1 TERT MSX1 IL6 CD4
7 11.89 TNF IL6 CD8A CD4
8 11.84 TGFB1 TERT MSX1 IRF6 IL6
9
Show member pathways
11.82 TNF TGFB1 IL6 CD4
10 11.79 TNF TGFB1 IL6 CD1E
11 11.66 TNF CD8A CD4
12 11.55 TNF IL6 CD8A CD4 CD1E
13
Show member pathways
11.54 TNF IL6 CD40LG
14 11.5 TNF TGFB1 TERT IL6
15 11.37 TNF TGFB1 IL6 CD40LG
16 11.3 CD8A CD40LG CD4
17 10.91 TNF IL6 CRP
18 10.89 TNF TGFB1 IL6 CD8A CD4
19 10.72 TNF TGFB1 IL6 CD4

GO Terms for Lymphoid Interstitial Pneumonia

Cellular components related to Lymphoid Interstitial Pneumonia according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 external side of plasma membrane GO:0009897 9.35 TNF CD8A CD40LG CD4 CD1E
2 telomerase catalytic core complex GO:0000333 8.62 TERT TERC

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

(show all 17)
# Name GO ID Score Top Affiliating Genes
1 negative regulation of cell proliferation GO:0008285 9.92 TGFB1 MSX1 IRF6 IL6 IGFBP3
2 inflammatory response GO:0006954 9.89 TNF TGFB1 IL6 CRP CD40LG
3 positive regulation of T cell proliferation GO:0042102 9.69 IL6 CD40LG CD4
4 cytokine-mediated signaling pathway GO:0019221 9.65 TNF TGFB1 PRTN3 IL6 CD4
5 negative regulation of fat cell differentiation GO:0045599 9.63 TNF TGFB1 IL6
6 immune response GO:0006955 9.63 TNF IL6 CD8A CD40LG CD4 CD1E
7 T cell activation GO:0042110 9.61 TGFB1 CD8A CD4
8 positive regulation of production of miRNAs involved in gene silencing by miRNA GO:1903800 9.57 TGFB1 IL6
9 negative regulation of production of miRNAs involved in gene silencing by miRNA GO:1903799 9.56 TNF TGFB1
10 positive regulation of viral entry into host cell GO:0046598 9.55 TRIM21 CD4
11 cellular response to nicotine GO:0071316 9.54 TNF MSX1
12 positive regulation of pri-miRNA transcription by RNA polymerase II GO:1902895 9.54 TNF TGFB1 TERT
13 DNA biosynthetic process GO:0071897 9.5 TERT TERC LIG3
14 positive regulation of mononuclear cell migration GO:0071677 9.48 TNF TGFB1
15 regulation of immunoglobulin secretion GO:0051023 9.4 TNF CD40LG
16 positive regulation of NF-kappaB transcription factor activity GO:0051092 9.35 TRIM21 TNF TGFB1 IL6 CD40LG
17 negative regulation of lipid storage GO:0010888 8.8 TNF IL6 CRP

Molecular functions related to Lymphoid Interstitial Pneumonia according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 cytokine activity GO:0005125 9.56 TNF TGFB1 IL6 CD40LG
2 RNA-directed DNA polymerase activity GO:0003964 9.16 TERT TERC
3 telomerase activity GO:0003720 8.96 TERT TERC
4 telomerase RNA reverse transcriptase activity GO:0003721 8.62 TERT TERC

Sources for Lymphoid Interstitial Pneumonia

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
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
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
53 NINDS
54 Novoseek
56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
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