MCID: PNB004
MIFTS: 35

Panbronchiolitis, Diffuse

Categories: Rare diseases, Respiratory diseases

Aliases & Classifications for Panbronchiolitis, Diffuse

MalaCards integrated aliases for Panbronchiolitis, Diffuse:

Name: Panbronchiolitis, Diffuse 57 53 13
Diffuse Panbronchiolitis 76 53 59 37 73
Pblt 57 53
Dpb 57 53

Characteristics:

Orphanet epidemiological data:

59
diffuse panbronchiolitis
Inheritance: Multigenic/multifactorial; Age of onset: Adult;

OMIM:

57
Inheritance:
multifactorial

Miscellaneous:
progressive disorder
onset in the second to fifth decade
fatal outcome if untreated
increased frequency among individuals of east asian descent


HPO:

32
panbronchiolitis, diffuse:
Onset and clinical course progressive


Classifications:

Orphanet: 59  
Rare respiratory diseases


External Ids:

OMIM 57 604809
Orphanet 59 ORPHA171700
MESH via Orphanet 45 C536174
UMLS via Orphanet 74 C0878555
ICD10 via Orphanet 34 J44.8
MedGen 42 C0878555
KEGG 37 H01713
UMLS 73 C0878555

Summaries for Panbronchiolitis, Diffuse

NIH Rare Diseases : 53 Diffuse panbronchiolitis (DPB) is a rare condition characterized by inflammation of the small airways of the lungs (bronchiolitis) and chronic sinusitis. It mainly occurs among the Japanese but has been reported in other populations. Symptoms typically develop anywhere from the teenage years to the fifth decade of life and are slowly progressive over months to years. Common symptoms include chronic sinusitis, a productive cough (producing mucus), breathlessness with exertion, wheezing, and weight loss (especially as symptoms worsen). The exact cause of DPB is not known, but a variety of genetic, environmental, and systemic factors appear to contribute to the condition. Treatment involves long-term use of macrolide antibiotics, which reportedly dramatically improves the long-term outcome. If untreated, DPB leads to bronchiectasis, progressive respiratory failure and death over the course of a few years.

MalaCards based summary : Panbronchiolitis, Diffuse, also known as diffuse panbronchiolitis, is related to bronchiolitis and rheumatoid arthritis, and has symptoms including coughing and wheezing. An important gene associated with Panbronchiolitis, Diffuse is MUCL3 (Mucin Like 3). The drugs Clarithromycin and Anti-Bacterial Agents have been mentioned in the context of this disorder. Affiliated tissues include lung, t cells and neutrophil, and related phenotypes are bronchiectasis and foam cells

OMIM : 57 Diffuse panbronchiolitis (DPB) is a rare chronic inflammatory obstructive pulmonary disease primarily affecting the respiratory bronchioles. 'Diffuse' refers to the distribution of the lesions throughout both lungs, and 'pan-' refers to the involvement of inflammation in all layers of the respiratory bronchioles. Onset of the disorder occurs in the second to fifth decade of life, and is clinically manifest by chronic cough, exertional dyspnea, and sputum production. Most patients also have chronic paranasal sinusitis. If untreated, the disorder progresses to bronchiectasis, respiratory failure, and death (summary by Poletti et al., 2006). (604809)

Wikipedia : 76 Diffuse panbronchiolitis (DPB) is an inflammatory lung disease of unknown cause. It is a severe,... more...

Related Diseases for Panbronchiolitis, Diffuse

Diseases related to Panbronchiolitis, Diffuse via text searches within MalaCards or GeneCards Suite gene sharing:

# Related Disease Score Top Affiliating Genes
1 bronchiolitis 10.1
2 rheumatoid arthritis 10.1
3 arthritis 10.1
4 cystic fibrosis 10.0
5 thymoma 10.0
6 bronchitis 10.0
7 asthma 9.9
8 pulmonary disease, chronic obstructive 9.9
9 nephrotic syndrome 9.9
10 iga glomerulonephritis 9.9

Graphical network of the top 20 diseases related to Panbronchiolitis, Diffuse:



Diseases related to Panbronchiolitis, Diffuse

Symptoms & Phenotypes for Panbronchiolitis, Diffuse

Symptoms via clinical synopsis from OMIM:

57
Laboratory Abnormalities:
hypoxemia

Respiratory Lung:
bronchiectasis
rhonchi
crackles
chronic inflammation in the bronchioles
infiltration of plasma cells, lymphocytes, and histiocytes
more
Respiratory:
cough
wheezing
obstructive respiratory impairment
sputum production

Respiratory Nasopharynx:
paranasal sinusitis, chronic


Clinical features from OMIM:

604809

Human phenotypes related to Panbronchiolitis, Diffuse:

32 (show all 7)
# Description HPO Frequency HPO Source Accession
1 bronchiectasis 32 HP:0002110
2 foam cells 32 HP:0003651
3 hypoxemia 32 HP:0012418
4 cough 32 HP:0012735
5 wheezing 32 HP:0030828
6 rales 32 HP:0030830
7 rhonchi 32 HP:0030831

UMLS symptoms related to Panbronchiolitis, Diffuse:


coughing, wheezing

Drugs & Therapeutics for Panbronchiolitis, Diffuse

Drugs for Panbronchiolitis, Diffuse (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 6)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Clarithromycin Approved Not Applicable 81103-11-9 84029
2 Anti-Bacterial Agents Not Applicable
3 Antibiotics, Antitubercular Not Applicable
4 Anti-Infective Agents Not Applicable
5 Cytochrome P-450 CYP3A Inhibitors Not Applicable
6 Cytochrome P-450 Enzyme Inhibitors Not Applicable

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Macrolide Antibiotic Therapy for Patients With Cystic Fibrosis Completed NCT00205634 Not Applicable Biaxin ( clarithromycin)

Search NIH Clinical Center for Panbronchiolitis, Diffuse

Genetic Tests for Panbronchiolitis, Diffuse

Anatomical Context for Panbronchiolitis, Diffuse

MalaCards organs/tissues related to Panbronchiolitis, Diffuse:

41
Lung, T Cells, Neutrophil, Testes, Whole Blood

Publications for Panbronchiolitis, Diffuse

Articles related to Panbronchiolitis, Diffuse:

(show top 50) (show all 200)
# Title Authors Year
1
High expression of diffuse panbronchiolitis critical region 1 gene promotes cell proliferation, migration and invasion in pancreatic ductal adenocarcinoma. ( 29242154 )
2018
2
Case of diffuse panbronchiolitis developed in a patient with epidermodysplasia verruciformis. ( 28836693 )
2017
3
Diffuse panbronchiolitis in a Samoan man. ( 28286653 )
2017
4
Successful treatment of diffuse panbronchiolitis in a child from Western China: A case report. ( 28565813 )
2017
5
Early-stage diffuse panbronchiolitis in a young patient confirmed by video-assisted lung biopsy: A case report. ( 29264074 )
2017
6
Diffuse panbronchiolitis in a 10-year-old boy. ( 25824890 )
2015
7
Macrolides for diffuse panbronchiolitis. ( 25618845 )
2015
8
Diffuse panbronchiolitis in an Australian aborigine. ( 25473569 )
2014
9
Nontuberculous mycobacteria in diffuse panbronchiolitis. ( 25269823 )
2014
10
Diffuse panbronchiolitis in a patient with common variable immunodeficiency: a casual association or a pathogenetic correlation? ( 24443813 )
2014
11
Diffuse panbronchiolitis and IgA nephropathy. ( 24381993 )
2014
12
A case of myasthenia gravis with invasive thymoma associated with diffuse panbronchiolitis, alopecia, dysgeusia, cholangitis and myositis. ( 25283823 )
2014
13
Asthma diagnosis and treatment - 1014. Single center experience of 4 cases of diffuse panbronchiolitis clinically presented as treatment resistant ashtma. ( 23819501 )
2013
14
Clinical profiles of 12 Chinese patients with diffuse panbronchiolitis. ( 24351621 )
2013
15
The effects of azithromycin on patients with diffuse panbronchiolitis: a retrospective study of 29 cases. ( 24255774 )
2013
16
Diffuse panbronchiolitis after humanized anti-CCR4 monoclonal antibody therapy for relapsed adult T-cell leukemia/lymphoma. ( 23397210 )
2013
17
Primary ciliary dyskinesia complicated with diffuse panbronchiolitis: a case report and literature review. ( 24308375 )
2013
18
Macrolides for diffuse panbronchiolitis. ( 23450579 )
2013
19
Evidence of improved small airways function after azithromycin treatment in diffuse panbronchiolitis. ( 22759949 )
2012
20
Diffuse panbronchiolitis: a very rare disease in Western countries. ( 22349095 )
2012
21
Diffuse panbronchiolitis. ( 22392943 )
2012
22
A case of Good syndrome with pulmonary lesions similar to diffuse panbronchiolitis. ( 22576393 )
2012
23
Effect of diffuse panbronchiolitis critical region 1 polymorphisms on the risk of aspirin-exacerbated respiratory disease in Korean asthmatics. ( 22152429 )
2012
24
Differential association between HLA and diffuse panbronchiolitis in Northern and Southern Chinese. ( 22293801 )
2012
25
Historical changes in epidemiology of diffuse panbronchiolitis. ( 23311119 )
2012
26
A case of diffuse panbronchiolitis complicated by peripheral T cell lymphoma not otherwise specified. ( 21613778 )
2012
27
Diffuse panbronchiolitis. ( 22640847 )
2012
28
Carbamazepine-induced agammagloblinaemia clinically mimicking diffuse panbronchiolitis. ( 22707469 )
2011
29
Diffuse panbronchiolitis in a 67-year-old Chinese man. ( 20972882 )
2011
30
Effect of azithromycin on patients with diffuse panbronchiolitis: retrospective study of 51 cases. ( 21841323 )
2011
31
Molecular cloning of two novel mucin-like genes in the disease- susceptibility locus for diffuse panbronchiolitis. ( 20981447 )
2011
32
Genetic predisposition to diffuse panbronchiolitis. ( 21303426 )
2011
33
Diffuse panbronchiolitis: report of a rare disease from India. ( 20364614 )
2010
34
Macrolides for diffuse panbronchiolitis. ( 21154381 )
2010
35
Deterioration of asthma in a patient with diffuse panbronchiolitis (DPB) after macrolide therapy. ( 20528606 )
2010
36
Diffuse panbronchiolitis associated with malignant thymoma. ( 20364613 )
2010
37
Idiopathic bronchiolitis with features of diffuse panbronchiolitis in an African-American patient with hepatitis C virus infection. ( 21074694 )
2010
38
Diffuse panbronchiolitis: not just an Asian disease: Australian case series and review of the literature. ( 21610988 )
2009
39
Comparison of clinical and radiographic characteristics between nodular bronchiectatic form of nontuberculous mycobacterial lung disease and diffuse panbronchiolitis. ( 19543504 )
2009
40
[Human leukocyte antigen genes to genetic predisposition in diffuse panbronchiolitis]. ( 18846964 )
2008
41
Diffuse panbronchiolitis. ( 18591343 )
2008
42
Diffuse panbronchiolitis in China: analysis of 45 cases. ( 18067795 )
2007
43
Diffuse panbronchiolitis: East meets West. ( 17400883 )
2007
44
Association of HLA genes with diffuse panbronchiolitis in Chinese patients. ( 17350353 )
2007
45
Test and teach. Number Fifty-three. A rare lung disease in an Indian man. Diagnosis: Diffuse panbronchiolitis. ( 18027265 )
2007
46
The treatment of refractory pneumothorax in diffuse panbronchiolitis by intravenous administration of coagulation factor XIII concentrate. ( 16641533 )
2006
47
The expression of pro- and anti-apoptotic Bcl-2 family proteins in peribronchiolar lymphocytes from patients with diffuse panbronchiolitis. ( 16574390 )
2006
48
Comparative clinicopathology of obliterative bronchiolitis and diffuse panbronchiolitis. ( 16195663 )
2006
49
Diffuse panbronchiolitis. ( 17012632 )
2006
50
Mechanism of mucin secretion in diffuse panbronchiolitis. ( 16424438 )
2006

Variations for Panbronchiolitis, Diffuse

Expression for Panbronchiolitis, Diffuse

Search GEO for disease gene expression data for Panbronchiolitis, Diffuse.

Pathways for Panbronchiolitis, Diffuse

GO Terms for Panbronchiolitis, Diffuse

Sources for Panbronchiolitis, Diffuse

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
Content
Loading form....