IPPFE
MCID: PLR025
MIFTS: 21

Pleuroparenchymal Fibroelastosis (IPPFE)

Categories: Rare diseases, Respiratory diseases

Aliases & Classifications for Pleuroparenchymal Fibroelastosis

MalaCards integrated aliases for Pleuroparenchymal Fibroelastosis:

Name: Pleuroparenchymal Fibroelastosis 20
Idiopathic Pleuroparenchymal Fibroelastosis 20 58
Idiopathic Pleuropulmonary Fibroelastosis 20 58
Ippfe 20 58

Classifications:

Orphanet: 58  
Rare respiratory diseases


External Ids:

Orphanet 58 ORPHA494428

Summaries for Pleuroparenchymal Fibroelastosis

MalaCards based summary : Pleuroparenchymal Fibroelastosis, also known as idiopathic pleuroparenchymal fibroelastosis, is related to lung disease and interstitial lung disease. 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 and bone marrow, 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 top 50) (show all 71)
# Related Disease Score Top Affiliating Genes
1 lung disease 31.0 SFTPD MUC1
2 interstitial lung disease 30.9 SFTPD MUC1
3 pulmonary fibrosis, idiopathic 30.7 SFTPD MUC1
4 pneumonia 30.7 SFTPD MUC1
5 nonspecific interstitial pneumonia 30.3 SFTPD MUC1
6 connective tissue disease 30.1 SFTPD MUC1
7 eosinophilic pneumonia 29.9 SFTPD MUC1
8 rheumatoid arthritis interstitial lung disease 29.8 SFTPD MUC1
9 bird fancier's lung 29.8 SFTPD MUC1
10 idiopathic interstitial pneumonia 11.4
11 pulmonary fibrosis 10.8
12 pneumothorax 10.8
13 fibrosis of extraocular muscles, congenital, 1 10.6
14 bronchiectasis 10.6
15 respiratory failure 10.6
16 bronchiolitis obliterans 10.6
17 bronchiolitis 10.6
18 scleroderma, familial progressive 10.5
19 graft-versus-host disease 10.4
20 extrinsic allergic alveolitis 10.4
21 pneumothorax, primary spontaneous 10.4
22 sarcoidosis 1 10.4
23 chronic graft versus host disease 10.4
24 bronchiolitis obliterans with obstructive pulmonary disease 10.3
25 autoimmune disease 10.2
26 esophageal cancer 10.2
27 body mass index quantitative trait locus 1 10.2
28 aspergillosis 10.2
29 allergic disease 10.2
30 vasculitis 10.2
31 pulmonary fibrosis predisposition 10.2
32 complications after hematopoietic stem cell transplantation 10.2
33 amyotrophic lateral sclerosis 1 10.1
34 lung cancer 10.1
35 pulmonary disease, chronic obstructive 10.1
36 granulomatosis with polyangiitis 10.1
37 alpha/beta t-cell lymphopenia with gamma/delta t-cell expansion, severe cytomegalovirus infection, and autoimmunity 10.1
38 leukemia, acute lymphoblastic 10.1
39 aspiration pneumonia 10.1
40 thrombosis 10.1
41 hepatic veno-occlusive disease 10.1
42 rapidly involuting congenital hemangioma 10.1
43 dermatomyositis 10.1
44 pleurisy 10.1
45 silicosis 10.1
46 telangiectasis 10.1
47 hypothyroidism 10.1
48 thrombocytopenia 10.1
49 lateral sclerosis 10.1
50 muscular atrophy 10.1

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

Search Clinical Trials , NIH Clinical Center for Pleuroparenchymal Fibroelastosis

Genetic Tests for Pleuroparenchymal Fibroelastosis

Anatomical Context for Pleuroparenchymal Fibroelastosis

MalaCards organs/tissues related to Pleuroparenchymal Fibroelastosis:

40
Lung, Bone Marrow

Publications for Pleuroparenchymal Fibroelastosis

Articles related to Pleuroparenchymal Fibroelastosis:

(show top 50) (show all 199)
# Title Authors PMID Year
1
Effectiveness of pirfenidone for idiopathic pulmonary fibrosis associated with pleuroparenchymal fibroelastosis-like lesions and nonspecific interstitial pneumonia. 61
33058535 2021
2
Pleuroparenchymal fibroelastosis in Korean patients: clinico-radiologic-pathologic features and 2-year follow-up. 61
32088939 2021
3
Prognostic role of KL-6 in SSc-ILD patients with pleuroparenchymal fibroelastosis. 61
33759179 2021
4
Idiopathic pleuroparenchymal fibroelastosis presenting in recurrent pneumothorax and bilateral pleural effusion: A case report. 61
33277197 2021
5
Lung Transplantation for Pleuroparenchymal Fibroelastosis. 61
33804467 2021
6
Lung Ultrasound to Phenotype Chronic Lung Allograft Dysfunction in Lung Transplant Recipients. A Prospective Observational Study. 61
33807615 2021
7
Evaluation of Respiratory Mechanics With Dynamic Breathing Magnetic Resonance Imaging in Pleuroparenchymal Fibroelastosis: Limited Lower Chest Wall Motion and Preserved Diaphragm Motion. 61
33810896 2021
8
Pleuroparenchymal Fibroelastosis Post Liver Transplantation. 61
33662223 2021
9
Pleuroparenchymal fibroelastosis in interstitial lung disease with antineutrophil cytoplasmic antibody-associated vasculitis. 61
33635211 2021
10
Comparison of Clinical Characteristics and Outcomes between Idiopathic and Secondary Pleuroparenchymal Fibroelastosis. 61
33670776 2021
11
Idiopathic pleuroparenchymal fibroelastosis confirmed by pathology: a case report. 61
33626951 2021
12
Outcomes of lung transplantation for idiopathic pleuroparenchymal fibroelastosis. 61
33576927 2021
13
Alveolar Epithelial Denudation Is a Major Factor in the Pathogenesis of Pleuroparenchymal Fibroelastosis. 61
33668178 2021
14
Treatment with antifibrotic agents in idiopathic pleuroparenchymal fibroelastosis with usual interstitial pneumonia. 61
33681342 2021
15
Acute exacerbation in chronic bird fancier's lung with pleuroparenchymal fibroelastosis. 61
33251015 2021
16
Pneumothorax in Patients with Idiopathic Pleuroparenchymal Fibroelastosis: Incidence, Clinical Features, and Risk Factors. 61
33401281 2021
17
Pleuroparenchymal fibroelastosis in Mycobacterium avium complex pulmonary disease: clinical characteristics and prognostic impact. 61
33569500 2021
18
Prominent eustachian valve: An uncommon cause of a common problem during extracorporeal membrane oxygenation support. 61
33225477 2021
19
The pathogenesis and pathology of idiopathic pleuroparenchymal fibroelastosis. 61
33315234 2020
20
Pleuroparenchymal fibroelastosis in rheumatic autoimmune diseases: a systematic literature review. 61
33313932 2020
21
Pleuroparenchymal fibroelastosis in a patient with lung cancer: Usefulness of PET/CT. 61
33279212 2020
22
Pleuroparenchymal fibroelastosis in a patient with systemic sclerosis. 61
33367850 2020
23
Physiological Criteria Are Useful for the Diagnosis of Idiopathic Pleuroparenchymal Fibroelastosis. 61
33266381 2020
24
Exacerbation of Secondary Pulmonary Hypertension by Flat Chest after Lung Transplantation. 61
33148928 2020
25
Acute Exacerbation of Pleuroparenchymal Fibroelastosis Secondary to Allogenic Hematopoietic Stem Cell Transplantation. 61
32669500 2020
26
Pleuroparenchymal Fibroelastosis: A New Entity of Interstitial Pneumonia Related to Connective Tissue Diseases. 61
30391159 2020
27
A case report of idiopathic pleuroparenchymal fibroelastosis with severe respiratory failure in pregnancy. 61
33054813 2020
28
Pleuroparenchymal fibroelastosis in patients with idiopathic pulmonary fibrosis. 61
32147954 2020
29
Serum latent transforming growth factor-β binding protein 4 as a novel biomarker for idiopathic pleuroparenchymal fibroelastosis. 61
32658840 2020
30
Disease course and prognosis of pleuroparenchymal fibroelastosis compared with idiopathic pulmonary fibrosis. 61
32917352 2020
31
Unilateral pleuroparenchymal fibroelastosis as a rare form of idiopathic interstitial pneumonia: A case report. 61
33024777 2020
32
Extent of pulmonary fibrosis on high-resolution computed tomography is a prognostic factor in patients with pleuroparenchymal fibroelastosis. 61
32762953 2020
33
Unilateral upper lung-field pulmonary fibrosis radiologically consistent with pleuroparenchymal fibroelastosis after thoracic surgery: Clinical and radiological courses with autopsy findings. 61
32660899 2020
34
Isolated Left Lower Lobe Pleuroparenchymal Fibroelastosis Presenting as Hemothorax. 61
31926159 2020
35
Pleuroparenchymal fibroelastosis in systemic sclerosis: prevalence and prognostic impact. 61
32299855 2020
36
An Autopsy Case of Idiopathic Pleuroparenchymal Fibroelastosis with Left Vocal Cord Paralysis and a Rapid Deterioration without an Acute Exacerbation. 61
32188804 2020
37
Pleuroparenchymal fibroelastosis after allogeneic hematopoietic stem cell transplantation. 61
31413312 2020
38
Is unclassifiable idiopathic interstitial pneumonia a result of idiopathic pleuroparenchymal fibroelastosis? 61
32278606 2020
39
Bacterial burden in the lower airways predicts disease progression in idiopathic pulmonary fibrosis and is independent of radiological disease extent. 61
31980496 2020
40
Clinical significance of thyroid hormone and antibodies in patients with idiopathic interstitial pneumonia. 61
32274118 2020
41
Pleuroparenchymal fibroelastosis in an 8-year old treated for neuroblastoma. 61
31944579 2020
42
Identification and characterization of chronic lung allograft dysfunction patients with mixed phenotype: A single-center study. 61
31958356 2020
43
Transbronchial cryobiopsy for diagnosis of pleuroparenchymal fibroelastosis. 61
32714826 2020
44
Pleuroparenchymal fibroelastosis (PPFE)-like finding on CT in daily practice -prevalence and serial changes. 61
33304942 2020
45
Remodeling of the pulmonary artery in idiopathic pleuroparenchymal fibroelastosis. 61
31941942 2020
46
Pleuroparenchymal fibroelastosis-like lesions in patients with interstitial pneumonia diagnosed by multidisciplinary discussion with surgical lung biopsy. 61
33354595 2020
47
Role of alveolar collapse in idiopathic pleuroparenchymal fibroelastosis. 61
33093785 2020
48
Pleuroparenchymal Fibroelastosis Associated with Crohn's Disease: A New Aetiology? 61
33457362 2020
49
Pleuroparenchymal Fibroelastosis as Another Potential Lung Toxicity Pattern Induced by Amiodarone. 61
31377110 2020
50
Suspected pleuroparenchymal fibroelastosis relapse after lung transplantation: a case report and literature review. 61
31938568 2019

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 SFTPD MUC1

GO Terms for Pleuroparenchymal Fibroelastosis

Sources for Pleuroparenchymal Fibroelastosis

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 05-Apr-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 Tocris
70 UMLS
71 UMLS via Orphanet
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