MCID: PLR025
MIFTS: 22

Pleuroparenchymal Fibroelastosis

Categories: Rare diseases, Respiratory diseases

Aliases & Classifications for Pleuroparenchymal Fibroelastosis

MalaCards integrated aliases for Pleuroparenchymal Fibroelastosis:

Name: Pleuroparenchymal Fibroelastosis 53
Idiopathic Pleuroparenchymal Fibroelastosis 53 59
Idiopathic Pleuropulmonary Fibroelastosis 53 59
Ippfe 53 59

Classifications:

Orphanet: 59  
Rare respiratory diseases


External Ids:

Orphanet 59 ORPHA494428

Summaries for Pleuroparenchymal Fibroelastosis

MalaCards based summary : Pleuroparenchymal Fibroelastosis, also known as idiopathic pleuroparenchymal fibroelastosis, is related to interstitial lung disease and pulmonary fibrosis, idiopathic. An important gene associated with Pleuroparenchymal Fibroelastosis is MUC1 (Mucin 1, Cell Surface Associated), and among its related pathways/superpathways is Bacterial infections in CF airways. Affiliated tissues include lung, thyroid and bone, and related phenotype is Increased virus production.

Related Diseases for Pleuroparenchymal Fibroelastosis

Diseases related to Pleuroparenchymal Fibroelastosis via text searches within MalaCards or GeneCards Suite gene sharing:

(show all 20)
# Related Disease Score Top Affiliating Genes
1 interstitial lung disease 29.2 MUC1 SFTPD
2 pulmonary fibrosis, idiopathic 28.8 MUC1 SFTPD
3 connective tissue disease 28.8 MUC1 SFTPD
4 pulmonary fibrosis 10.4
5 pneumonia 10.3
6 pneumothorax 10.2
7 idiopathic interstitial pneumonia 10.2
8 lung disease 10.2
9 graft-versus-host disease 10.2
10 hypothyroidism 10.0
11 limited scleroderma 10.0
12 thyroiditis 10.0
13 scleroderma, familial progressive 10.0
14 chronic graft versus host disease 10.0
15 hematopoietic stem cell transplantation 10.0
16 silicosis 10.0
17 pleurisy 10.0
18 respiratory failure 10.0
19 acute interstitial pneumonia 9.5 MUC1 SFTPD
20 pneumocystosis 9.4 MUC1 SFTPD

Graphical network of the top 20 diseases related to Pleuroparenchymal Fibroelastosis:



Diseases related to Pleuroparenchymal Fibroelastosis

Symptoms & Phenotypes for Pleuroparenchymal Fibroelastosis

GenomeRNAi Phenotypes related to Pleuroparenchymal Fibroelastosis according to GeneCards Suite gene sharing:

26
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Increased virus production GR00378-A 8.62 MUC1 SFTPD

Drugs & Therapeutics for Pleuroparenchymal Fibroelastosis

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Bardoxolone Methyl Evaluation in Patients With Pulmonary Hypertension (PH) - LARIAT Completed NCT02036970 Phase 2 Bardoxolone methyl;Placebo

Search NIH Clinical Center for Pleuroparenchymal Fibroelastosis

Genetic Tests for Pleuroparenchymal Fibroelastosis

Anatomical Context for Pleuroparenchymal Fibroelastosis

MalaCards organs/tissues related to Pleuroparenchymal Fibroelastosis:

41
Lung, Thyroid, Bone, Bone Marrow

Publications for Pleuroparenchymal Fibroelastosis

Articles related to Pleuroparenchymal Fibroelastosis:

(show all 39)
# Title Authors Year
1
Significant increases in the density and number of lymphatic vessels in pleuroparenchymal fibroelastosis. ( 29675827 )
2018
2
Pleuroparenchymal Fibroelastosis and Silicosis: An Unexpected Association. ( 29580565 )
2018
3
Podoplanin-positive myofibroblasts: a pathological hallmark of pleuroparenchymal fibroelastosis. ( 29468722 )
2018
4
Radiological pleuroparenchymal fibroelastosis associated to limited cutaneous systemic sclerosis: a case report. ( 29776440 )
2018
5
Pleuroparenchymal Fibroelastosis With Long History of Asbestos and Silicon Exposure. ( 29113529 )
2018
6
Functional associations of pleuroparenchymal fibroelastosis and emphysema with hypersensitivity pneumonitis. ( 29724400 )
2018
7
Veno-venous extracorporeal membrane oxygenation bridged living-donor lung transplantation for rapid progressive respiratory failure with pleuroparenchymal fibroelastosis after allogeneic hematopoietic stem cell transplantation. ( 29773298 )
2018
8
An adolescent with idiopathic pleuroparenchymal fibroelastosis. Case report. ( 29333842 )
2018
9
Pleuroparenchymal fibroelastosis presenting with pneumothorax. ( 29552343 )
2018
10
Is hypothyroidism in idiopathic pleuroparenchymal fibroelastosis a novel lung-thyroid syndrome? ( 29325681 )
2018
11
Pleuroparenchymal fibroelastosis after haematopoietic stem cell transplantation without graft-versus-host disease findings. ( 29796278 )
2018
12
Bilateral Tuberculous Pleurisy with Subsequent Upper Lobe Predominant Pulmonary Fibrosis Mimicking Pleuroparenchymal Fibroelastosis. ( 29033441 )
2018
13
Pleuroparenchymal fibroelastosis with positive MPO-ANCA diagnosed with a CT-guided percutaneous needle biopsy. ( 29478005 )
2018
14
Pleuroparenchymal fibroelastosis in heterogeneous clinical conditions: Clinicopathologic analysis of 7 cases. ( 28842969 )
2018
15
Radiologic pleuroparenchymal fibroelastosis-like lesion in connective tissue disease-related interstitial lung disease. ( 28666014 )
2017
16
Proliferation of elastic fibres in idiopathic pulmonary fibrosis: a whole-slide image analysis and comparison with pleuroparenchymal fibroelastosis. ( 28718917 )
2017
17
Pleuroparenchymal Fibroelastosis: A Review of Histopathologic Features and the Relationship Between Histologic Parameters and Survival. ( 28877057 )
2017
18
Evaluation of urinary desmosines as a noninvasive diagnostic biomarker in patients with idiopathic pleuroparenchymal fibroelastosis (PPFE). ( 28137498 )
2017
19
Idiopathic pleuroparenchymal fibroelastosis (PPFE) - A case study of a rare entity. ( 28780989 )
2017
20
Idiopathic Pleuroparenchymal Fibroelastosis. ( 28344924 )
2017
21
Restrictive allograft syndrome and idiopathic pleuroparenchymal fibroelastosis: do they really have the same histology? ( 28120410 )
2017
22
Idiopathic pleuroparenchymal fibroelastosis with suggestive biopsy of pulmonary carcinoma - Case report. ( 28034563 )
2017
23
A CARE-compliant case report: Lung transplantation for a Chinese young man with idiopathic pleuroparenchymal fibroelastosis. ( 28489801 )
2017
24
Clinical diagnosis of idiopathic pleuroparenchymal fibroelastosis: A retrospective multicenter study. ( 29173442 )
2017
25
Unilateral Upper Lung Field Pulmonary Fibrosis Radiologically Consistent with Pleuroparenchymal Fibroelastosis after Thoracotomy: A New Disease Entity Related to Thoracotomy. ( 28858871 )
2017
26
A Case of Pleuroparenchymal Fibroelastosis. ( 29372959 )
2017
27
A Patient with Idiopathic Pleuroparenchymal Fibroelastosis Showing a Sustained Pulmonary Function due to Treatment with Pirfenidone. ( 26935370 )
2016
28
Idiopathic pleuroparenchymal fibroelastosis - A rare idiopathic interstitial pneumonia. ( 27141432 )
2016
29
Idiopathic pleuroparenchymal fibroelastosis, a new idiopathic interstitial pneumonia: A case report. ( 26810762 )
2016
30
Idiopathic pleuroparenchymal fibroelastosis: a rare but increasingly recognized entity. ( 25854135 )
2015
31
Idiopathic pleuroparenchymal fibroelastosis: incidental findings in a patient with suspected pneumonia. ( 26578141 )
2015
32
A Case of Interstitial Lung Disease With Apical Pleural Thickening: Idiopathic Pleuroparenchymal Fibroelastosis. ( 24139332 )
2014
33
Distinct characteristics of pleuroparenchymal fibroelastosis with usual interstitial pneumonia compared with idiopathic pulmonary fibrosis. ( 25367470 )
2014
34
WITHDRAWN: Idiopathic pleuroparenchymal fibroelastosis: A rare but increasingly recognized entity. ( 24975296 )
2014
35
Quantitative analysis of lung elastic fibers in idiopathic pleuroparenchymal fibroelastosis (IPPFE): comparison of clinical, radiological, and pathological findings with those of idiopathic pulmonary fibrosis (IPF). ( 24886550 )
2014
36
Idiopathic pleuroparenchymal fibroelastosis is characterized by an elevated serum level of surfactant protein-D, but Not Krebs von den Lungen-6. ( 24880792 )
2014
37
Pleuroparenchymal Fibroelastosis as a Series of Airway Complications Associated with Chronic Graft-versus-host Disease following Allogeneic Bone Marrow Transplantation. ( 24390527 )
2014
38
Idiopathic pleuroparenchymal fibroelastosis presenting in recurrent pneumothorax: a case report. ( 25368665 )
2014
39
Idiopathic pleuroparenchymal fibroelastosis: consideration of a clinicopathological entity in a series of Japanese patients. ( 23216996 )
2012

Variations for Pleuroparenchymal Fibroelastosis

Expression for Pleuroparenchymal Fibroelastosis

Search GEO for disease gene expression data for Pleuroparenchymal Fibroelastosis.

Pathways for Pleuroparenchymal Fibroelastosis

Pathways related to Pleuroparenchymal Fibroelastosis according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1
Show member pathways
10.84 MUC1 SFTPD

GO Terms for Pleuroparenchymal Fibroelastosis

Sources for Pleuroparenchymal Fibroelastosis

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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