MCID: LYM004
MIFTS: 45

Lymphoid Interstitial Pneumonia

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

Aliases & Classifications for Lymphoid Interstitial Pneumonia

MalaCards integrated aliases for Lymphoid Interstitial Pneumonia:

Name: Lymphoid Interstitial Pneumonia 57 12 59 15 73
Lip Diseases 44 73
Lip Disease 12 15
Lymphocytic Interstitial Pneumonia 59
Disease of Lips 12
Lip 57

Characteristics:

Orphanet epidemiological data:

59
lymphoid interstitial pneumonia
Inheritance: Not applicable; Age of onset: All ages;

OMIM:

57
Inheritance:
autosomal recessive


HPO:

32
lymphoid interstitial pneumonia:
Inheritance autosomal recessive inheritance


Classifications:

Orphanet: 59  
Rare respiratory diseases


External Ids:

OMIM 57 247610
Disease Ontology 12 DOID:0050159 DOID:9297
ICD10 33 K13.0
ICD9CM 35 528.5
MeSH 44 D008047
NCIt 50 C26818
SNOMED-CT 68 196563002 90678009
Orphanet 59 ORPHA79128
UMLS via Orphanet 74 C0264511
ICD10 via Orphanet 34 J84.1
MedGen 42 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 oral cancer and vulva cancer, and has symptoms including oral manifestations and cheilodynia. An important gene associated with Lymphoid Interstitial Pneumonia is CD8A (CD8a Molecule), and among its related pathways/superpathways is p75(NTR)-mediated signaling. The drugs Zidovudine and Anti-HIV Agents have been mentioned in the context of this disorder. Affiliated tissues include lung, t cells and bone, and related phenotypes are clubbing and cor pulmonale

Description 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 425)
# Related Disease Score Top Affiliating Genes
1 oral cancer 30.9 KRT13 TP53
2 vulva cancer 30.0 KRT13 TP53
3 tongue cancer 29.8 KRT13 TP53
4 cleft lip/palate 12.4
5 cleft lip 12.4
6 lip cancer 12.3
7 cleft lip/palate-ectodermal dysplasia syndrome 12.3
8 ankyloblepharon-ectodermal defects-cleft lip/palate 12.2
9 ectrodactyly, ectodermal dysplasia, and cleft lip/palate syndrome 1 12.2
10 blepharochalasis and double lip 12.2
11 van der woude syndrome 1 12.1
12 cleft, median, of upper lip with polyps of facial skin and nasal mucosa 12.1
13 arthrogryposis, ectodermal dysplasia, cleft lip/palate, and developmental delay 12.1
14 ectrodactyly, ectodermal dysplasia, and cleft lip/palate syndrome 3 12.1
15 coloboma, ocular, with or without hearing impairment, cleft lip/palate, and/or mental retardation 12.0
16 lower lip cancer 12.0
17 lip, median nodule of upper 12.0
18 brachial amelia, cleft lip, and holoprosencephaly 12.0
19 isolated cleft lip 12.0
20 polydactyly cleft lip palate psychomotor retardation 12.0
21 cleft lip and alveolus 12.0
22 upper lip cancer 11.9
23 lower lip fistula 11.9
24 commissural lip fistula 11.9
25 microcephaly, corpus callosum dysgenesis, and cleft lip/palate 11.9
26 uveal coloboma-cleft lip and palate-intellectual disability 11.9
27 microbrachycephaly ptosis cleft lip 11.9
28 lip and oral cavity cancer 11.9
29 lip carcinoma in situ 11.9
30 lip prints 11.8
31 median cleft lip/mandibule 11.8
32 commissural lip pits 11.8
33 maxillary double lip 11.8
34 popliteal pterygium syndrome 11.8
35 woolly hair, hypotrichosis, everted lower lip, and outstanding ears 11.8
36 cleft lip/palate with abnormal thumbs and microcephaly 11.8
37 ectrodactyly and ectodermal dysplasia without cleft lip/palate 11.8
38 kniest-like dysplasia with pursed lips and ectopia lentis 11.8
39 cleft lip-retinopathy syndrome 11.8
40 lip, hamartomatous 11.7
41 cleft lip/palate with characteristic facies, intestinal malrotation, and lethal congenital heart disease 11.7
42 cleft lip and palate-craniofacial dysmorphism-congenital heart defect-hearing loss syndrome 11.7
43 fountain syndrome 11.7
44 phlebectasia of lips 11.7
45 radius, aplasia of, with cleft lip/palate 11.7
46 split lower lip 11.7
47 hypotrichosis, progressive patterned scalp, with wiry hair, onycholysis, and cleft lip/palate 11.7
48 capillary malformation of the lower lip, lymphatic malformation of face and neck, asymmetry of face and limbs, and partial/generalized overgrowth 11.7
49 cleft lip and palate malrotation cardiopathy 11.7
50 cleft lip and/or palate with mucous cysts of lower 11.7

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

Symptoms via clinical synopsis from OMIM:

57
Pulmonary:
lymphoid interstitial pneumonia

Cardiac:
cor pulmonale
aortic stenosis

Limbs:
digital clubbing


Clinical features from OMIM:

247610

Human phenotypes related to Lymphoid Interstitial Pneumonia:

32
# Description HPO Frequency HPO Source Accession
1 clubbing 32 HP:0001217
2 cor pulmonale 32 HP:0001648
3 aortic valve stenosis 32 HP:0001650
4 lymphoid interstitial pneumonia 32 HP:0006527

UMLS symptoms related to Lymphoid Interstitial Pneumonia:


oral manifestations, cheilodynia

MGI Mouse Phenotypes related to Lymphoid Interstitial Pneumonia:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 hematopoietic system MP:0005397 9.86 CD8A NUMB SPEN TMEM79 TP53 TPPP
2 homeostasis/metabolism MP:0005376 9.76 NUMB TMEM79 TP53 TPPP TRIM21 UNC93B1
3 immune system MP:0005387 9.5 CD8A CRP SPEN TMEM79 TP53 TRIM21
4 integument MP:0010771 9.1 CD8A NDN SPEN TMEM79 TP53 TRIM21

Drugs & Therapeutics for Lymphoid Interstitial Pneumonia

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

(show all 10)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Zidovudine Approved Phase 2 30516-87-1 35370
2 Anti-HIV Agents Phase 2
3 Anti-Infective Agents Phase 2
4 Antimetabolites Phase 2
5 Anti-Retroviral Agents Phase 2
6 Antiviral Agents Phase 2
7 Nucleic Acid Synthesis Inhibitors Phase 2
8 Reverse Transcriptase Inhibitors Phase 2
9 Antibodies
10 Immunoglobulins

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 The Safety and Effectiveness of Zidovudine in the Treatment of HIV-Infected Children With Mild to Moderate Symptoms Completed NCT00000990 Phase 2 Zidovudine
2 Bardoxolone Methyl Evaluation in Patients With Pulmonary Hypertension (PH) - LARIAT Completed NCT02036970 Phase 2 Bardoxolone methyl;Placebo
3 Clinical Course of Interstitial Lung Diseases: European IPF Registry and Biobank Recruiting NCT02951416

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:

41
Lung, T Cells, Bone, Bone Marrow

Publications for Lymphoid Interstitial Pneumonia

Articles related to Lymphoid Interstitial Pneumonia:

(show top 50) (show all 55)
# Title Authors Year
1
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. ( 29023302 )
2017
2
Lymphoid Interstitial Pneumonia and Other Benign Lymphoid Disorders. ( 27231864 )
2016
3
Cystic amyloidosis or lymphoid interstitial pneumonia associated with amyloidosis? A diagnostic challenge. ( 22916778 )
2012
4
HIV-associated lymphoid interstitial pneumonia mimicking miliary tuberculosis. ( 22145281 )
2011
5
Lymphoid interstitial pneumonia associated with common variable immunoglobulin deficiency. ( 18421195 )
2008
6
Lymphoid interstitial pneumonia: clinical features, associations and prognosis. ( 16571614 )
2006
7
Diffuse lung cysts in lymphoid interstitial pneumonia: high-resolution CT and pathologic findings. ( 16915074 )
2006
8
CR10/80--Lymphoid interstitial pneumonia and Kabuki-Syndrome in a young man. ( 17036410 )
2006
9
BCL-6 mutations in pulmonary lymphoproliferative disorders: demonstration of an aberrant immunological reaction in HIV-related lymphoid interstitial pneumonia. ( 15153535 )
2004
10
Improvement of symptomatic human immunodeficiency virus-related lymphoid interstitial pneumonia in patients receiving highly active antiretroviral therapy. ( 12746792 )
2003
11
Oligoclonal T cell expansions in pulmonary lymphoproliferative disorders: demonstration of the frequent occurrence of oligoclonal T cells in human immunodeficiency virus-related lymphoid interstitial pneumonia. ( 11790664 )
2002
12
Lymphoid interstitial pneumonia: a narrative review. ( 12475860 )
2002
13
Aberrant expression of immunoglobulin heavy chain genes in Epstein-Barr virus-negative, human immunodeficiency virus-related lymphoid interstitial pneumonia. ( 11140701 )
2000
14
Gadolinium chloride removes pulmonary intravascular macrophages and curtails the degree of ovine lentivirus-induced lymphoid interstitial pneumonia. ( 9741357 )
1998
15
Transforming growth factor-beta 1 (TGF-beta1) expression in ovine lentivirus-induced lymphoid interstitial pneumonia. ( 9566793 )
1998
16
A case of lymphoid interstitial pneumonia in a 3-month-old boy not associated with HIV infection: immunohistochemistry of lung biopsy specimens and serum transforming growth factor-beta 1 assay. ( 9361104 )
1997
17
Distribution and quantitation of lung parenchymal contractile tissue in ovine lentivirus-induced lymphoid interstitial pneumonia. Do tissue forces limit lung distensibility? ( 7564278 )
1995
18
Lymphoid interstitial pneumonia as a pulmonary lesion of idiopathic plasmacytic lymphadenopathy with hyperimmunoglobulinemia. ( 8069020 )
1994
19
Pulmonary pathology in AIDS: atypical Pneumocystis carinii infection and lymphoid interstitial pneumonia. ( 7974327 )
1994
20
Bronchoalveolar carcinoma, lymphoid interstitial pneumonia, and retroviruses. ( 7888571 )
1994
21
Pathophysiologic correlations in lymphoid interstitial pneumonia. ( 8004316 )
1994
22
[Multicentric Castleman's disease accompanied with both lymphoid interstitial pneumonia and interstitial nephritis]. ( 7823399 )
1994
23
Treatment of lymphoid interstitial pneumonia with chloroquine. ( 8489636 )
1993
24
Pathogenesis of lymphoid interstitial pneumonia in natural and experimental ovine lentivirus infection. ( 8399922 )
1993
25
Effects on lung compliance, lung volume, and single-breath transfer factor for carbon monoxide in sheep with lentivirus-induced lymphoid interstitial pneumonia. ( 8388674 )
1993
26
Multicentric lymphosarcoma associated with pulmonary adenomatosis (Jaagsiekte), pulmonary lymphoid hyperplasia, and lymphoid interstitial pneumonia in a ewe. ( 1621339 )
1992
27
Effects of virus load in the pathogenesis of lentivirus-induced lymphoid interstitial pneumonia. ( 1323619 )
1992
28
Lymphoid interstitial pneumonia not associated with HIV infection: role of Epstein-Barr virus. ( 1344810 )
1992
29
Primary pulmonary lymphoma: a re-appraisal of its histogenesis and its relationship to pseudolymphoma and lymphoid interstitial pneumonia. ( 3209184 )
1988
30
Retrovirus-associated ovine pulmonary carcinoma (sheep pulmonary adenomatosis) and lymphoid interstitial pneumonia. I. Lesion development and age susceptibility. ( 3212891 )
1988
31
Spontaneous interferon production by pulmonary leukocytes is associated with lentivirus-induced lymphoid interstitial pneumonia. ( 2448376 )
1988
32
Lymphoid interstitial pneumonia: clinicopathological and immunopathological findings in 18 cases. ( 3453998 )
1987
33
Suppressor T cell activity in lymphoid interstitial pneumonia. ( 2949115 )
1987
34
Experimental coinduction of type D retrovirus-associated pulmonary carcinoma and lentivirus-associated lymphoid interstitial pneumonia in lambs. ( 3474445 )
1987
35
Ovine lentivirus lymphoid interstitial pneumonia. Rapid induction in neonatal lambs. ( 3022591 )
1986
36
Lymphoid interstitial pneumonia and rheumatoid arthritis. ( 4032144 )
1985
37
Lymphoid interstitial pneumonia: findings at bronchoalveolar lavage. ( 3876947 )
1985
38
Lymphoid interstitial pneumonia after allogeneic bone marrow transplantation. A possible manifestation of chronic graft-versus-host disease. ( 3880651 )
1985
39
Lymphoid interstitial pneumonia associated with depressed cellular immunity and polyclonal gammopathy. ( 6476599 )
1984
40
Lymphoid interstitial pneumonia in juvenile rheumatoid arthritis. ( 6502346 )
1984
41
Familial lymphoid interstitial pneumonia: a long-term follow-up. ( 7360539 )
1980
42
Lymphoid interstitial pneumonia and dysproteinemia associated with lymphoma. ( 6967792 )
1980
43
Lymphoid interstitial pneumonia. ( 6445495 )
1980
44
Ultrastructure of lymphoid interstitial pneumonia: virus-like particles in bronchiolar epithelium of a patient with SjAPgren's syndrome. ( 851093 )
1977
45
Utrastructure lymphoid interstitial pneumonia. ( 602919 )
1977
46
Lymphoid interstitial pneumonia with associated chronic active hepatitis and renal tubular acidosis. ( 835885 )
1977
47
Coexistent lymphoid interstitial pneumonia, pernicious anemia, and agammaglobulinemia. ( 1082325 )
1976
48
Lymphoid interstitial pneumonia. ( 1268792 )
1976
49
Lymphoid interstitial pneumonia and autoerythrocyte sensitization syndrome. A case with deposition of immunoglobulins on the alveolar basement membrane. ( 4604713 )
1974
50
Diffuse lymphoid interstitial pneumonia. ( 4787989 )
1973

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:

# Super pathways Score Top Affiliating Genes
1
Show member pathways
10.44 NDN TP53

GO Terms for Lymphoid Interstitial Pneumonia

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

# Name GO ID Score Top Affiliating Genes
1 positive regulation of neurogenesis GO:0050769 8.62 NUMB SPEN

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

# Name GO ID Score Top Affiliating Genes
1 RNA polymerase II transcription factor binding GO:0001085 8.62 SPEN TP53

Sources for Lymphoid 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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