MCID: PLR025
MIFTS: 24

Pleuroparenchymal Fibroelastosis

Categories: Rare diseases, Respiratory diseases

Aliases & Classifications for Pleuroparenchymal Fibroelastosis

MalaCards integrated aliases for Pleuroparenchymal Fibroelastosis:

Name: Pleuroparenchymal Fibroelastosis 54
Idiopathic Pleuroparenchymal Fibroelastosis 54 60
Idiopathic Pleuropulmonary Fibroelastosis 54 60
Ippfe 54 60

Classifications:

Orphanet: 60  
Rare respiratory diseases


External Ids:

Orphanet 60 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 SFTPD (Surfactant Protein D), and among its related pathways/superpathways is Bacterial infections in CF airways. Affiliated tissues include bone, t cells 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:

27
# 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:

42
Bone, T Cells, Bone Marrow, Thyroid, Lung

Publications for Pleuroparenchymal Fibroelastosis

Articles related to Pleuroparenchymal Fibroelastosis:

(show top 50) (show all 104)
# Title Authors Year
1
A case of airway aluminosis with likely secondary pleuroparenchymal fibroelastosis. ( 31011423 )
2019
2
Combination of pleuroparenchymal fibroelastosis with non-specific interstitial pneumonia and bronchiolitis obliterans as a complication of hematopoietic stem cell transplantation - Clues to a potential mechanism. ( 30792952 )
2019
3
Pleuroparenchymal fibroelastosis secondary to autologous hematopoietic stem cell transplantation: A case report. ( 30906445 )
2019
4
Cause of pleuroparenchymal fibroelastosis following allogeneic hematopoietic stem cell transplantation. ( 31080050 )
2019
5
Pleuroparenchymal fibroelastosis as a histological background of autoimmune diseases. ( 30327882 )
2019
6
Usual Interstitial Pneumonia Pattern in the Lower Lung Lobes as a Prognostic Factor in Idiopathic Pleuroparenchymal Fibroelastosis. ( 30522096 )
2019
7
Pleuroparenchymal fibroelastosis-like lesions on chest computed tomography in routine clinical practice. ( 30600481 )
2019
8
Idiopathic pleuroparenchymal fibroelastosis presenting as bilateral spontaneous pneumothorax: A case report. ( 30604712 )
2019
9
Idiopathic pleuroparenchymal fibroelastosis: The first case to be managed with a successful lung transplant at King Faisal Specialist Hospital and Research Center, Riyadh. ( 30745942 )
2019
10
The upward shift of hilar structures and tracheal deviation in pleuroparenchymal fibroelastosis. ( 30886711 )
2019
11
Criteria for the diagnosis of idiopathic pleuroparenchymal fibroelastosis: A proposal. ( 30981683 )
2019
12
Clinico-radiologic features of pleuroparenchymal fibroelastosis in children. ( 31004186 )
2019
13
Pleuroparenchymal fibroelastosis (PPFE) treated with lung transplantation and review of the literature. ( 31005872 )
2019
14
Pleuroparenchymal fibroelastosis (PPFE) associated with giant cell arteritis: A coincidence or a novel phenotype? ( 31024793 )
2019
15
Chronic Pulmonary Aspergillosis with Pleuroparenchymal Fibroelastosis-like Features: A Case Report. ( 30568135 )
2018
16
Pleuroparenchymal fibroelastosis after haematopoietic stem cell transplantation without graft-versus-host disease findings. ( 29796278 )
2018
17
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
18
Is hypothyroidism in idiopathic pleuroparenchymal fibroelastosis a novel lung-thyroid syndrome? ( 29325681 )
2018
19
Clinical significance of radiological pleuroparenchymal fibroelastosis pattern in interstitial lung disease patients registered for lung transplantation: a retrospective cohort study. ( 30165854 )
2018
20
Bilateral Tuberculous Pleurisy with Subsequent Upper Lobe Predominant Pulmonary Fibrosis Mimicking Pleuroparenchymal Fibroelastosis. ( 29033441 )
2018
21
Pleuroparenchymal fibroelastosis in heterogeneous clinical conditions: Clinicopathologic analysis of 7 cases. ( 28842969 )
2018
22
Pleuroparenchymal Fibroelastosis With Long History of Asbestos and Silicon Exposure. ( 29113529 )
2018
23
An adolescent with idiopathic pleuroparenchymal fibroelastosis. Case report. ( 29333842 )
2018
24
Podoplanin-positive myofibroblasts: a pathological hallmark of pleuroparenchymal fibroelastosis. ( 29468722 )
2018
25
Pleuroparenchymal fibroelastosis with positive MPO-ANCA diagnosed with a CT-guided percutaneous needle biopsy. ( 29478005 )
2018
26
Pleuroparenchymal fibroelastosis presenting with pneumothorax. ( 29552343 )
2018
27
Pleuroparenchymal Fibroelastosis and Silicosis: An Unexpected Association. ( 29580565 )
2018
28
Significant increases in the density and number of lymphatic vessels in pleuroparenchymal fibroelastosis. ( 29675827 )
2018
29
Functional associations of pleuroparenchymal fibroelastosis and emphysema with hypersensitivity pneumonitis. ( 29724400 )
2018
30
Radiological pleuroparenchymal fibroelastosis associated to limited cutaneous systemic sclerosis: a case report. ( 29776440 )
2018
31
Pleuroparenchymal fibroelastosis diagnosed by multidisciplinary discussions in Japan. ( 30053966 )
2018
32
Poorer Prognosis of Idiopathic Pleuroparenchymal Fibroelastosis Compared with Idiopathic Pulmonary Fibrosis in Advanced Stage. ( 30186537 )
2018
33
Airway-Centered Pleuroparenchymal Fibroelastosis Associated with Non-Necrotizing Granulomas: A Rare New Entity. ( 30223268 )
2018
34
Distinct profile and prognostic impact of body composition changes in idiopathic pulmonary fibrosis and idiopathic pleuroparenchymal fibroelastosis. ( 30232390 )
2018
35
Pleuroparenchymal Fibroelastosis: A New Entity of Interstitial Pneumonia Related to Connective Tissue Diseases. ( 30391159 )
2018
36
Various Bronchiolar Lesions Accompanied by Idiopathic Pleuroparenchymal Fibroelastosis with a Usual Interstitial Pneumonia Pattern Demonstrating Acute Exacerbation: A Case Study. ( 30568138 )
2018
37
Lung Transplantation as Successful Treatment of End-stage Idiopathic Pleuroparenchymal Fibroelastosis: A Case Report. ( 30655147 )
2018
38
Radiologic pleuroparenchymal fibroelastosis-like lesion in connective tissue disease-related interstitial lung disease. ( 28666014 )
2017
39
Evaluation of urinary desmosines as a noninvasive diagnostic biomarker in patients with idiopathic pleuroparenchymal fibroelastosis (PPFE). ( 28137498 )
2017
40
Idiopathic Pleuroparenchymal Fibroelastosis. ( 28344924 )
2017
41
A CARE-compliant case report: Lung transplantation for a Chinese young man with idiopathic pleuroparenchymal fibroelastosis. ( 28489801 )
2017
42
Pleuroparenchymal fibroelastosis associated with telomerase reverse transcriptase mutations. ( 28495683 )
2017
43
Pleuroparenchymal fibroelastosis associated with telomerase reverse transcriptase mutations. ( 28495689 )
2017
44
Proliferation of elastic fibres in idiopathic pulmonary fibrosis: a whole-slide image analysis and comparison with pleuroparenchymal fibroelastosis. ( 28718917 )
2017
45
Idiopathic pleuroparenchymal fibroelastosis (PPFE) - A case study of a rare entity. ( 28780989 )
2017
46
Unilateral Upper Lung Field Pulmonary Fibrosis Radiologically Consistent with Pleuroparenchymal Fibroelastosis after Thoracotomy: A New Disease Entity Related to Thoracotomy. ( 28858871 )
2017
47
Pleuroparenchymal Fibroelastosis: A Review of Histopathologic Features and the Relationship Between Histologic Parameters and Survival. ( 28877057 )
2017
48
Clinical diagnosis of idiopathic pleuroparenchymal fibroelastosis: A retrospective multicenter study. ( 29173442 )
2017
49
A Case of Pleuroparenchymal Fibroelastosis. ( 29372959 )
2017
50
Pleuroparenchymal fibroelastosis with prominent thrombosis. ( 27998011 )
2017

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 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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