IPPFE
MCID: PLR025
MIFTS: 24

Pleuroparenchymal Fibroelastosis (IPPFE)

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, bone and bone marrow, and related phenotype is Increased virus production.

Related Diseases for Pleuroparenchymal Fibroelastosis

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, Bone, Bone Marrow, Thyroid, T Cells

Publications for Pleuroparenchymal Fibroelastosis

Articles related to Pleuroparenchymal Fibroelastosis:

(show top 50) (show all 89)
# 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
Clinical significance of radiological pleuroparenchymal fibroelastosis pattern in interstitial lung disease patients registered for lung transplantation: a retrospective cohort study. ( 30165854 )
2018
16
Pleuroparenchymal fibroelastosis diagnosed by multidisciplinary discussions in Japan. ( 30053966 )
2018
17
Poorer Prognosis of Idiopathic Pleuroparenchymal Fibroelastosis Compared with Idiopathic Pulmonary Fibrosis in Advanced Stage. ( 30186537 )
2018
18
Airway-Centered Pleuroparenchymal Fibroelastosis Associated with Non-Necrotizing Granulomas: A Rare New Entity. ( 30223268 )
2018
19
Distinct profile and prognostic impact of body composition changes in idiopathic pulmonary fibrosis and idiopathic pleuroparenchymal fibroelastosis. ( 30232390 )
2018
20
Pleuroparenchymal fibroelastosis as a histological background of autoimmune diseases. ( 30327882 )
2018
21
Pleuroparenchymal Fibroelastosis: A New Entity of Interstitial Pneumonia Related to Connective Tissue Diseases. ( 30391159 )
2018
22
Usual Interstitial Pneumonia Pattern in the Lower Lung Lobes as a Prognostic Factor in Idiopathic Pleuroparenchymal Fibroelastosis. ( 30522096 )
2018
23
Radiologic pleuroparenchymal fibroelastosis-like lesion in connective tissue disease-related interstitial lung disease. ( 28666014 )
2017
24
Proliferation of elastic fibres in idiopathic pulmonary fibrosis: a whole-slide image analysis and comparison with pleuroparenchymal fibroelastosis. ( 28718917 )
2017
25
Pleuroparenchymal Fibroelastosis: A Review of Histopathologic Features and the Relationship Between Histologic Parameters and Survival. ( 28877057 )
2017
26
Evaluation of urinary desmosines as a noninvasive diagnostic biomarker in patients with idiopathic pleuroparenchymal fibroelastosis (PPFE). ( 28137498 )
2017
27
Idiopathic pleuroparenchymal fibroelastosis (PPFE) - A case study of a rare entity. ( 28780989 )
2017
28
Idiopathic Pleuroparenchymal Fibroelastosis. ( 28344924 )
2017
29
Restrictive allograft syndrome and idiopathic pleuroparenchymal fibroelastosis: do they really have the same histology? ( 28120410 )
2017
30
Idiopathic pleuroparenchymal fibroelastosis with suggestive biopsy of pulmonary carcinoma - Case report. ( 28034563 )
2017
31
A CARE-compliant case report: Lung transplantation for a Chinese young man with idiopathic pleuroparenchymal fibroelastosis. ( 28489801 )
2017
32
Clinical diagnosis of idiopathic pleuroparenchymal fibroelastosis: A retrospective multicenter study. ( 29173442 )
2017
33
Pleuroparenchymal fibroelastosis with prominent thrombosis. ( 27998011 )
2017
34
Pleuroparenchymal fibroelastosis: report of two cases in Brazil. ( 28125151 )
2017
35
Pleuroparenchymal fibroelastosis associated with telomerase reverse transcriptase mutations. ( 28495683 )
2017
36
Pleuroparenchymal fibroelastosis associated with telomerase reverse transcriptase mutations. ( 28495689 )
2017
37
Unilateral Upper Lung Field Pulmonary Fibrosis Radiologically Consistent with Pleuroparenchymal Fibroelastosis after Thoracotomy: A New Disease Entity Related to Thoracotomy. ( 28858871 )
2017
38
A Case of Pleuroparenchymal Fibroelastosis. ( 29372959 )
2017
39
A Patient with Idiopathic Pleuroparenchymal Fibroelastosis Showing a Sustained Pulmonary Function due to Treatment with Pirfenidone. ( 26935370 )
2016
40
Idiopathic pleuroparenchymal fibroelastosis - A rare idiopathic interstitial pneumonia. ( 27141432 )
2016
41
Idiopathic pleuroparenchymal fibroelastosis, a new idiopathic interstitial pneumonia: A case report. ( 26810762 )
2016
42
Pleuroparenchymal fibroelastosis in association with carcinomas. ( 26464176 )
2016
43
Pleuroparenchymal fibroelastosis: role of high-resolution computed tomography (HRCT) and CT-guided transthoracic core lung biopsy. ( 26577607 )
2016
44
Air-leak Syndrome by Pleuroparenchymal Fibroelastosis after Bone Marrow Transplantation. ( 26781007 )
2016
45
Living Donor Lung Transplantation for Pleuroparenchymal Fibroelastosis. ( 27106430 )
2016
46
Heterogeneous clinical features in patients with pulmonary fibrosis showing histology of pleuroparenchymal fibroelastosis. ( 27108011 )
2016
47
Pleuroparenchymal fibroelastosis after allogenic hematopoietic stem cell transplantation: important histological component of late-onset noninfectious pulmonary complication accompanied with recurrent pneumothorax. ( 27312041 )
2016
48
Pleuroparenchymal fibroelastosis: the prevalence of secondary forms in hematopoietic stem cell and lung transplantation recipients. ( 27460284 )
2016
49
Pleuroparenchymal Fibroelastosis of the Lung: A Review. ( 27472241 )
2016
50
Two cases of pleuroparenchymal fibroelastosis diagnosed with transbronchial lung biopsy. ( 27536547 )
2016

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
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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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