DPB
MCID: PNB004
MIFTS: 34

Panbronchiolitis, Diffuse (DPB)

Categories: Rare diseases, Respiratory diseases

Aliases & Classifications for Panbronchiolitis, Diffuse

MalaCards integrated aliases for Panbronchiolitis, Diffuse:

Name: Panbronchiolitis, Diffuse 57 20 13
Diffuse Panbronchiolitis 73 20 58 36 70
Pblt 57 20
Dpb 57 20

Characteristics:

Orphanet epidemiological data:

58
diffuse panbronchiolitis
Inheritance: Multigenic/multifactorial; Age of onset: Adult;

OMIM®:

57 (Updated 20-May-2021)
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:

31
panbronchiolitis, diffuse:
Onset and clinical course progressive


Classifications:

Orphanet: 58  
Rare respiratory diseases


External Ids:

OMIM® 57 604809
KEGG 36 H01713
MESH via Orphanet 45 C536174
ICD10 via Orphanet 33 J44.8
UMLS via Orphanet 71 C0878555
Orphanet 58 ORPHA171700
MedGen 41 C0878555
UMLS 70 C0878555

Summaries for Panbronchiolitis, Diffuse

KEGG : 36 Diffuse panbronchiolitis (DPB) is a chronic inflammatory lung disease, which predominantly affects East Asians. Clinically, DPB is characterized by chronic inflammation of the respiratory bronchioles and sinobronchial infection. While DPB is frequently compared with cystic fibrosis, a common genetic disease encountered in Caucasians, neither pancreatic insufficiency nor any obvious abnormalities of the sweat electrolytes are seen in DPB, and the two are considered to be entirely different diseases. Immunogenetic studies revealed a strong association with human HLA-B54 in Japanese, whereas an association with HLA-A11 was reported in Koreans. These findings imply that a major susceptibility gene may be located between the HLA-A and HLA-B loci on the short arm of human chromosome 6. Recently, novel mucin-like genes has been cloned in this candidate region. And it was found that their polymorphisms were associated with DPB. Over the past two decades, DPB has shifted from being a near-fatal to a treatable disease. A significant improvement in the prognosis of this disease has been attributed to the long-term use of macrolides. The five-year survival rate increased to about 90% after treatment with erythromycin became widely used.

MalaCards based summary : Panbronchiolitis, Diffuse, also known as diffuse panbronchiolitis, is related to severe pre-eclampsia and bronchitis, and has symptoms including wheezing and coughing. An important gene associated with Panbronchiolitis, Diffuse is MUCL3 (Mucin Like 3). Affiliated tissues include neutrophil, lung and t cells, and related phenotypes are cough and hypoxemia

GARD : 20 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.

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) (Updated 20-May-2021)

Wikipedia : 73 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 121)
# Related Disease Score Top Affiliating Genes
1 severe pre-eclampsia 10.9
2 bronchitis 10.6
3 lung disease 10.5
4 thymoma, familial 10.3
5 thymoma 10.3
6 pulmonary fibrosis, idiopathic 10.3
7 bronchiolitis obliterans 10.3
8 pulmonary fibrosis 10.3
9 vasculitis 10.3
10 haemophilus influenzae 10.3
11 cystic fibrosis 10.2
12 asthma 10.2
13 spastic paraplegia, intellectual disability, nystagmus, and obesity 10.2
14 good syndrome 10.2
15 bronchial disease 10.2
16 agammaglobulinemia 10.2
17 primary ciliary dyskinesia 10.2
18 myasthenia gravis 10.1
19 chlamydia pneumonia 10.1
20 exanthem 10.1
21 bacterial infectious disease 10.1
22 chlamydia 10.1
23 candidiasis 10.1
24 pneumothorax 10.1
25 thymic carcinoma 10.1
26 bronchiolitis obliterans with obstructive pulmonary disease 10.1
27 retinoblastoma 10.0
28 respiratory failure 10.0
29 systemic lupus erythematosus 9.9
30 yellow nail syndrome 9.9
31 myositis 9.9
32 pneumothorax, primary spontaneous 9.9
33 rheumatoid arthritis 9.9
34 thrombocytopenic purpura, autoimmune 9.9
35 epidermodysplasia verruciformis 1 9.9
36 lymphoma, hodgkin, classic 9.9
37 hutterite cerebroosteonephrodysplasia syndrome 9.9
38 immune deficiency disease 9.9
39 bare lymphocyte syndrome, type i 9.9
40 lymphoma, non-hodgkin, familial 9.9
41 hepatitis c virus 9.9
42 aspergillosis 9.9
43 graft-versus-host disease 9.9
44 pulmonary hypertension 9.9
45 aspiration pneumonia 9.9
46 cryptogenic organizing pneumonia 9.9
47 lambert-eaton myasthenic syndrome 9.9
48 inflammatory bowel disease 9.9
49 peripheral t-cell lymphoma 9.9
50 ptosis 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:

31 (show all 7)
# Description HPO Frequency HPO Source Accession
1 cough 31 HP:0012735
2 hypoxemia 31 HP:0012418
3 bronchiectasis 31 HP:0002110
4 foam cells 31 HP:0003651
5 crackles 31 HP:0030830
6 wheezing 31 HP:0030828
7 rhonchi 31 HP:0030831

Symptoms via clinical synopsis from OMIM®:

57 (Updated 20-May-2021)
Respiratory:
cough
wheezing
obstructive respiratory impairment
sputum production

Respiratory Lung:
bronchiectasis
crackles
rhonchi
chronic inflammation in the bronchioles
infiltration of plasma cells, lymphocytes, and histiocytes
more
Laboratory Abnormalities:
hypoxemia

Respiratory Nasopharynx:
paranasal sinusitis, chronic

Clinical features from OMIM®:

604809 (Updated 20-May-2021)

UMLS symptoms related to Panbronchiolitis, Diffuse:


wheezing; coughing

Drugs & Therapeutics for Panbronchiolitis, Diffuse

Search Clinical Trials , NIH Clinical Center for Panbronchiolitis, Diffuse

Genetic Tests for Panbronchiolitis, Diffuse

Anatomical Context for Panbronchiolitis, Diffuse

MalaCards organs/tissues related to Panbronchiolitis, Diffuse:

40
Neutrophil, Lung, T Cells, Monocytes, Bone Marrow, Eye, Pituitary

Publications for Panbronchiolitis, Diffuse

Articles related to Panbronchiolitis, Diffuse:

(show top 50) (show all 695)
# Title Authors PMID Year
1
Diffuse panbronchiolitis. 57 61
17012632 2006
2
Fine localization of a major disease-susceptibility locus for diffuse panbronchiolitis. 61 57
10677310 2000
3
Association of HLA class I antigens with diffuse panbronchiolitis in Korean patients. 57 61
9927368 1999
4
Association of diffuse panbronchiolitis with microsatellite polymorphism of the human interleukin 8 (IL-8) gene. 61 57
10319580 1999
5
Contribution of HLA genes to genetic predisposition in diffuse panbronchiolitis. 57 61
9731015 1998
6
Clinical profiles of Chinese patients with diffuse panbronchiolitis. 57 61
9741370 1998
7
Diffuse panbronchiolitis--a new Japanese export? 57 61
8942772 1996
8
Diffuse panbronchiolitis and cystic fibrosis: East meets West. 57 61
8016786 1994
9
Diffuse panbronchiolitis: report of a case. 57 61
1363394 1992
10
The first report of diffuse panbronchiolitis in Korea: five case reports. 57 61
1504438 1992
11
Analysis of HLA antigens in patients with diffuse panbronchiolitis. 61 57
2350086 1990
12
Diffuse panbronchiolitis. A disease of the transitional zone of the lung. 61 57
6848335 1983
13
[Problems in chronic obstructive bronchial diseases, with special reference to diffuse panbronchiolitis]. 61 57
5783341 1969
14
Isolation of Mycobacterium talmoniae from a patient with diffuse panbronchiolitis: a case report. 61
33691626 2021
15
Airway clearance ameliorated the control of diffuse panbronchiolitis accompanied by asthma and leukocytoclastic vasculitis. 61
32736960 2021
16
The Usefulness of a Transbronchial Lung Cryobiopsy for Diffuse Bronchiolitis. 61
33281160 2020
17
Serum defensin levels in patients with systemic sclerosis. 61
33353556 2020
18
Comorbidities associated with nontuberculous mycobacterial disease in Japanese adults: a claims-data analysis. 61
33036598 2020
19
Three cases of diffuse panbronchiolitis in children with a past history of difficult-to-treat bronchial asthma: A case report from a single medical facility. 61
32217024 2020
20
Azithromycin is the answer in paediatric respiratory medicine, but what was the question? 61
31629643 2020
21
Overview and Challenges of Bronchiolar Disorders. 61
31860801 2020
22
Small Airway Disease: A Step Closer to Etiology-Based Classification of Bronchiolitis. 61
32005432 2020
23
Coexistence of diffuse panbronchiolitis and sarcoidosis revealed during splenectomy: a case report. 61
32228530 2020
24
Hypocomplementemic urticarial vasculitis syndrome with gastrointestinal vasculitis and crescentic membranoproliferative glomerulonephritis without immune complex deposits. 61
31586313 2020
25
Immune-Mediated Peripheral Neuropathy in Patients with Diffuse Panbronchiolitis. 61
31942777 2020
26
Diffuse panbronchiolitis: A case report from a Chinese consanguineous marriage family and literature review. 61
33063535 2020
27
Primary ciliary dyskinesia caused by a large homozygous deletion including exons 1-4 of DRC1 in Japanese patients with recurrent sinopulmonary infection. 61
31701675 2020
28
Development and Clinical Application of a Novel Non-contact Early Airflow Limitation Screening System Using an Infrared Time-of-Flight Depth Image Sensor. 61
33013479 2020
29
Recurring large deletion in DRC1 (CCDC164) identified as causing primary ciliary dyskinesia in two Asian patients. 61
31270959 2019
30
Long-term antibiotics for Chronic Rhinosinusitis: changing views. 61
31374113 2019
31
Diffuse panbronchiolitis: A progressive fatal lung disease that is curable with azithromycin, but only if diagnosed! 61
30609307 2019
32
Thymic neoplasms patients complicated with bronchiectasis: Case series in a Chinese hospital and literature review. 61
30758142 2019
33
Diffuse panbronchiolitis. 61
30498005 2019
34
The development of diffuse panbronchiolitis during the treatment with long-term, low-dose clarithromycin for chronic sinusitis. 61
30097373 2019
35
Azithromycin treats diffuse panbronchiolitis by targeting T cells via inhibition of mTOR pathway. 61
30530046 2019
36
Yellow Nail Syndrome Patients with Diffuse Panbronchiolitis-like Pulmonary Manifestation. 61
30210127 2019
37
Prognostic Value of Concomitant Bronchiectasis in Newly Diagnosed Diffuse Panbronchiolitis Patients on a Maintenance Therapy with Macrolides. 61
30984318 2019
38
[A CASE OF BRONCHIAL ASTHMA ASSOCIATED WITH BRONCHIOLAR EOSINOPHILIA AND TREATED WITH MEPOLIZUMAB]. 61
30867360 2019
39
A case of sinobronchial syndrome progressing to diffuse panbronchiolitis despite low-dose, long-term macrolide therapy. 61
30505511 2018
40
Diffuse panbronchiolitis: A fine road from the discovery of a disease to the establishment of treatment. 61
30049592 2018
41
Diffuse panbronchiolitis research in MEDLINE (1969-2017): Report of a bibliometric analysis. 61
30008294 2018
42
Characteristics of macrolide responders in persistent post-surgical rhinosinusitis. 61
29476191 2018
43
[A progress of macrolides therapy for chronic rhinosinusitis]. 61
29771095 2018
44
High expression of diffuse panbronchiolitis critical region 1 gene promotes cell proliferation, migration and invasion in pancreatic ductal adenocarcinoma. 61
29242154 2018
45
Early-stage diffuse panbronchiolitis in a young patient confirmed by video-assisted lung biopsy: A case report. 61
29264074 2017
46
Case of diffuse panbronchiolitis developed in a patient with epidermodysplasia verruciformis. 61
28836693 2017
47
The role of macrophages in interstitial lung diseases: Number 3 in the Series "Pathology for the clinician" Edited by Peter Dorfmüller and Alberto Cavazza. 61
28724562 2017
48
[Chinese expert consensus for non-antiinfective effects and clinical use of macrolides]. 61
28693067 2017
49
Immunomodulatory indications of azithromycin in respiratory disease: a concise review for the clinician. 61
28116959 2017
50
Successful treatment of diffuse panbronchiolitis in a child from Western China: A case report. 61
28565813 2017

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
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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