MCID: PNB004
MIFTS: 38

Panbronchiolitis, Diffuse

Categories: Rare diseases, Respiratory diseases

Aliases & Classifications for Panbronchiolitis, Diffuse

MalaCards integrated aliases for Panbronchiolitis, Diffuse:

Name: Panbronchiolitis, Diffuse 58 54 13
Diffuse Panbronchiolitis 77 54 60 38 74
Pblt 58 54
Dpb 58 54

Characteristics:

Orphanet epidemiological data:

60
diffuse panbronchiolitis
Inheritance: Multigenic/multifactorial; Age of onset: Adult;

OMIM:

58
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:

33
panbronchiolitis, diffuse:
Onset and clinical course progressive


Classifications:

Orphanet: 60  
Rare respiratory diseases


External Ids:

OMIM 58 604809
KEGG 38 H01713
MESH via Orphanet 46 C536174
ICD10 via Orphanet 35 J44.8
UMLS via Orphanet 75 C0878555
Orphanet 60 ORPHA171700
MedGen 43 C0878555
UMLS 74 C0878555

Summaries for Panbronchiolitis, Diffuse

NIH Rare Diseases : 54 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 cystic fibrosis, and has symptoms including wheezing and coughing. An important gene associated with Panbronchiolitis, Diffuse is MUCL3 (Mucin Like 3). The drugs Clarithromycin and Cytochrome P-450 CYP3A Inhibitors have been mentioned in the context of this disorder. Affiliated tissues include t cells, lung and neutrophil, and related phenotypes are hypoxemia and cough

OMIM : 58 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 : 77 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:

(show top 50) (show all 61)
# Related Disease Score Top Affiliating Genes
1 bronchiolitis 10.3
2 cystic fibrosis 10.2
3 thymoma 10.2
4 bronchitis 10.2
5 lung disease 10.2
6 thymoma, familial 10.1
7 adult t-cell leukemia 10.1
8 bronchiolitis obliterans 10.1
9 leukemia 10.1
10 chlamydia pneumonia 10.1
11 good syndrome 10.1
12 lymphoma 10.1
13 chlamydia 10.1
14 t-cell leukemia 10.1
15 bronchiectasis 10.1
16 bronchiolitis obliterans with obstructive pulmonary disease 10.1
17 biliary cirrhosis, primary, 1 10.0
18 asthma 10.0
19 pulmonary disease, chronic obstructive 10.0
20 cholangitis, primary sclerosing 10.0
21 primary biliary cirrhosis 10.0
22 nephrotic syndrome 10.0
23 sclerosing cholangitis 10.0
24 iga glomerulonephritis 10.0
25 cholangitis 10.0
26 primary biliary cholangitis 10.0
27 yellow nail syndrome 9.9
28 myositis 9.9
29 pulmonary fibrosis, idiopathic 9.9
30 rheumatoid arthritis 9.9
31 thrombocytopenic purpura, autoimmune 9.9
32 bare lymphocyte syndrome, type ii 9.9
33 lung cancer 9.9
34 epidermodysplasia verruciformis 1 9.9
35 myasthenia gravis 9.9
36 bare lymphocyte syndrome, type i 9.9
37 hepatitis c virus 9.9
38 pulmonary hypertension 9.9
39 alopecia 9.9
40 arthritis 9.9
41 common variable immunodeficiency 9.9
42 hepatitis 9.9
43 pancreatic ductal adenocarcinoma 9.9
44 peripheral t-cell lymphoma 9.9
45 primary ciliary dyskinesia 9.9
46 pulmonary fibrosis 9.9
47 sinusitis 9.9
48 ulcerative colitis 9.9
49 cryptogenic organizing pneumonia 9.9
50 lambert-eaton myasthenic syndrome 9.9

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



Diseases related to Panbronchiolitis, Diffuse

Symptoms & Phenotypes for Panbronchiolitis, Diffuse

Human phenotypes related to Panbronchiolitis, Diffuse:

33 (show all 7)
# Description HPO Frequency HPO Source Accession
1 hypoxemia 33 HP:0012418
2 cough 33 HP:0012735
3 bronchiectasis 33 HP:0002110
4 foam cells 33 HP:0003651
5 wheezing 33 HP:0030828
6 rhonchi 33 HP:0030831
7 crackles 33 HP:0030830

Symptoms via clinical synopsis from OMIM:

58
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

UMLS symptoms related to Panbronchiolitis, Diffuse:


wheezing, coughing

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 Phase 2 81103-11-9 84029
2 Cytochrome P-450 CYP3A Inhibitors Phase 2
3 Antibiotics, Antitubercular Phase 2
4 Anti-Infective Agents Phase 2
5 Cytochrome P-450 Enzyme Inhibitors Phase 2
6 Anti-Bacterial Agents Phase 2

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Macrolide Antibiotic Therapy for Patients With Cystic Fibrosis Completed NCT00205634 Phase 2 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:

42
T Cells, Lung, Neutrophil, Brain, Testes, Whole Blood

Publications for Panbronchiolitis, Diffuse

Articles related to Panbronchiolitis, Diffuse:

(show top 50) (show all 220)
# Title Authors Year
1
Prognostic Value of Concomitant Bronchiectasis in Newly Diagnosed Diffuse Panbronchiolitis Patients on a Maintenance Therapy with Macrolides. ( 30984318 )
2019
2
Diffuse panbronchiolitis: A progressive fatal lung disease that is curable with azithromycin, but only if diagnosed! ( 30609307 )
2019
3
The development of diffuse panbronchiolitis during the treatment with long-term, low-dose clarithromycin for chronic sinusitis. ( 30097373 )
2019
4
Yellow Nail Syndrome Patients with Diffuse Panbronchiolitis-like Pulmonary Manifestation. ( 30210127 )
2019
5
High expression of diffuse panbronchiolitis critical region 1 gene promotes cell proliferation, migration and invasion in pancreatic ductal adenocarcinoma. ( 29242154 )
2018
6
Diffuse panbronchiolitis research in MEDLINE (1969-2017): Report of a bibliometric analysis. ( 30008294 )
2018
7
Diffuse panbronchiolitis: A fine road from the discovery of a disease to the establishment of treatment. ( 30049592 )
2018
8
Diffuse panbronchiolitis. ( 30498005 )
2018
9
A case of sinobronchial syndrome progressing to diffuse panbronchiolitis despite low-dose, long-term macrolide therapy. ( 30505511 )
2018
10
Azithromycin treats diffuse panbronchiolitis by targeting T cells via inhibition of mTOR pathway. ( 30530046 )
2018
11
Case of diffuse panbronchiolitis developed in a patient with epidermodysplasia verruciformis. ( 28836693 )
2017
12
Diffuse panbronchiolitis. ( 28062747 )
2017
13
Is ionizing radiation a risk factor to diffuse panbronchiolitis? ( 28069906 )
2017
14
Diffuse panbronchiolitis in a Samoan man. ( 28286653 )
2017
15
Coexistence of diffuse panbronchiolitis and asthma: reciprocity of neutrophilic and eosinophilic inflammation. ( 28392920 )
2017
16
Successful treatment of diffuse panbronchiolitis in a child from Western China: A case report. ( 28565813 )
2017
17
Early-stage diffuse panbronchiolitis in a young patient confirmed by video-assisted lung biopsy: A case report. ( 29264074 )
2017
18
Diffuse panbronchiolitis-The response and recurrence after erythromycin therapy. ( 26705139 )
2016
19
Lung transplantation for diffuse panbronchiolitis: 5 cases from a single centre. ( 26888742 )
2016
20
eComment. Benefits of macrolide usage and bacteriological profile in patients with diffuse panbronchiolitis. ( 27114397 )
2016
21
Broncholithiasis and Lithoptysis Associated with Diffuse Panbronchiolitis. ( 27523017 )
2016
22
Hypereosinophilic obliterative bronchiolitis clinically mimicking diffuse panbronchiolitis: four-year follow-up. ( 25948354 )
2015
23
A case of diffuse panbronchiolitis, associated with severe pulmonary hypertension, managed with bilateral lung transplantation from a brain-dead donor. ( 26344614 )
2015
24
Nontuberculous mycobacteria in diffuse panbronchiolitis. ( 25269823 )
2015
25
Macrolides for diffuse panbronchiolitis. ( 25618845 )
2015
26
Diffuse panbronchiolitis in a 10-year-old boy. ( 25824890 )
2015
27
Single center experience of five diffuse panbronchiolitis patients clinically presenting as severe asthma. ( 26028938 )
2015
28
Diffuse panbronchiolitis in a patient with common variable immunodeficiency: a casual association or a pathogenetic correlation? ( 24443813 )
2014
29
Primary ciliary dyskinesia complicated with diffuse panbronchiolitis: a case report and literature review. ( 24308375 )
2014
30
Diffuse panbronchiolitis and IgA nephropathy. ( 24381993 )
2014
31
Diffuse panbronchiolitis in an Australian aborigine. ( 25473569 )
2014
32
[A case of myasthenia gravis with invasive thymoma associated with diffuse panbronchiolitis, alopecia, dysgeusia, cholangitis and myositis]. ( 25283823 )
2014
33
Diffuse panbronchiolitis after humanized anti-CCR4 monoclonal antibody therapy for relapsed adult T-cell leukemia/lymphoma. ( 23397210 )
2013
34
Asthma diagnosis and treatment - 1014. Single center experience of 4 cases of diffuse panbronchiolitis clinically presented as treatment resistant ashtma. ( 23819501 )
2013
35
The effects of azithromycin on patients with diffuse panbronchiolitis: a retrospective study of 29 cases. ( 24255774 )
2013
36
Clinical profiles of 12 Chinese patients with diffuse panbronchiolitis. ( 24351621 )
2013
37
Macrolides for diffuse panbronchiolitis. ( 23450579 )
2013
38
A case of Good syndrome with pulmonary lesions similar to diffuse panbronchiolitis. ( 22576393 )
2012
39
A case of diffuse panbronchiolitis complicated by peripheral T cell lymphoma not otherwise specified. ( 21613778 )
2012
40
Effect of diffuse panbronchiolitis critical region 1 polymorphisms on the risk of aspirin-exacerbated respiratory disease in Korean asthmatics. ( 22152429 )
2012
41
Differential association between HLA and diffuse panbronchiolitis in Northern and Southern Chinese. ( 22293801 )
2012
42
Diffuse panbronchiolitis: a very rare disease in Western countries. ( 22349095 )
2012
43
Diffuse panbronchiolitis. ( 22392943 )
2012
44
Diffuse panbronchiolitis. ( 22640847 )
2012
45
Evidence of improved small airways function after azithromycin treatment in diffuse panbronchiolitis. ( 22759949 )
2012
46
Historical changes in epidemiology of diffuse panbronchiolitis. ( 23311119 )
2012
47
Diffuse panbronchiolitis in a 67-year-old Chinese man. ( 20972882 )
2011
48
Molecular cloning of two novel mucin-like genes in the disease-susceptibility locus for diffuse panbronchiolitis. ( 20981447 )
2011
49
Genetic predisposition to diffuse panbronchiolitis. ( 21303426 )
2011
50
Effect of azithromycin on patients with diffuse panbronchiolitis: retrospective study of 51 cases. ( 21841323 )
2011

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 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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