MCID: FBR028
MIFTS: 25

Fibrosing Mediastinitis malady

Categories: Rare diseases, Respiratory diseases, Bone diseases

Aliases & Classifications for Fibrosing Mediastinitis

Aliases & Descriptions for Fibrosing Mediastinitis:

Name: Fibrosing Mediastinitis 50 56
Mediastinal Fibrosis 50 56 69
Igg4-Related Mediastinitis 50 56
Sclerosing Mediastinitis 50 56
Idiopathic Mediastinal Fibrosis 50
Idiopathic Hilar Fibrosis 69

Characteristics:

Orphanet epidemiological data:

56
igg4-related mediastinitis
Inheritance: Not applicable;

Classifications:



External Ids:

Orphanet 56 ORPHA63999
MESH via Orphanet 43 C536136
UMLS via Orphanet 70 C0221386 C0264573
ICD10 via Orphanet 34 J98.5

Summaries for Fibrosing Mediastinitis

NIH Rare Diseases : 50 fibrosing mediastinitis is a condition that affects the area between the lungs (mediastinum) which contains the heart, large blood vessels, windpipe (trachea), esophagus, and lymph nodes. people with fibrosing mediastinitis have varying amounts of scar tissue in the mediastinum which may cause problems for the organs located there. for example, some affected people may develop blocked airways that can interfere with lung function. others may have compressed blood vessels which can slow or prevent blood flow to and from the heart. the exact cause of fibrosing mediastinitis is not fully understood, but it is not inherited. many cases are linked to a specific type of fungal infection called histoplasmosis. treatment depends on which structures of the mediastinum are affected, the severity of the scarring and, in some cases, the cause of the condition. last updated: 11/5/2014

MalaCards based summary : Fibrosing Mediastinitis, also known as mediastinal fibrosis, is related to mediastinitis and superior vena cava syndrome. The drugs Immunoglobulin G and Immunoglobulins have been mentioned in the context of this disorder. Affiliated tissues include lung, heart and trachea.

Related Diseases for Fibrosing Mediastinitis

Diseases related to Fibrosing Mediastinitis via text searches within MalaCards or GeneCards Suite gene sharing:

(show all 24)
id Related Disease Score Top Affiliating Genes
1 mediastinitis 10.7
2 superior vena cava syndrome 10.1
3 histoplasmosis 10.1
4 pulmonary hypertension 10.0
5 pulmonary vein stenosis 10.0
6 cervicitis 9.9
7 tuberculosis 9.8
8 igg4-related disease 9.8
9 thrombosis 9.8
10 pulmonary edema 9.8
11 tracheal stenosis 9.8
12 connective tissue disease 9.7
13 myocardial infarction 9.7
14 thyroiditis 9.7
15 anaplastic large cell lymphoma 9.7
16 hepatitis 9.7
17 bronchogenic cyst 9.7
18 lymphoma 9.7
19 pyelonephritis 9.7
20 zygomycosis 9.7
21 xanthogranulomatous pyelonephritis 9.7
22 esophagitis 9.7
23 blastomycosis 9.7
24 histiocytosis 9.7

Graphical network of the top 20 diseases related to Fibrosing Mediastinitis:



Diseases related to Fibrosing Mediastinitis

Symptoms & Phenotypes for Fibrosing Mediastinitis

Drugs & Therapeutics for Fibrosing Mediastinitis

Drugs for Fibrosing Mediastinitis (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):


id Name Status Phase Clinical Trials Cas Number PubChem Id
1 Immunoglobulin G
2 Immunoglobulins
3 Antibodies

Interventional clinical trials:


id Name Status NCT ID Phase
1 A Prospective Cohort Study of IgG4RD in China Recruiting NCT01670695

Search NIH Clinical Center for Fibrosing Mediastinitis

Genetic Tests for Fibrosing Mediastinitis

Anatomical Context for Fibrosing Mediastinitis

MalaCards organs/tissues related to Fibrosing Mediastinitis:

39
Lung, Heart, Trachea, Lymph Node, Bone, Breast, Thyroid

Publications for Fibrosing Mediastinitis

Articles related to Fibrosing Mediastinitis:

(show top 50) (show all 137)
id Title Authors Year
1
Clinical Manifestations of Fibrosing Mediastinitis in Chinese Patients. ( 27824002 )
2016
2
Tuberculosis-associated Fibrosing Mediastinitis: Case Report and Literature Review. ( 27625898 )
2016
3
Tubercular fibrosing mediastinitis rare cause for hoarseness of voice. ( 27728713 )
2016
4
Pulmonary Hypertension Complicating Fibrosing Mediastinitis. ( 26554778 )
2015
5
Successful stenting of left pulmonary veins stenosis resulting from fibrosing mediastinitis. ( 25586119 )
2015
6
Fibrosing mediastinitis: a rare complication of histoplasmosis. ( 26538131 )
2015
7
Fibrosing mediastinitis complicating prior histoplasmosis is associated with human leukocyte antigen DQB1*04:02 - a case control study. ( 25940591 )
2015
8
Idiopathic fibrosing mediastinitis: spectrum of imaging findings with emphasis on its association with IgG4-related disease. ( 26257115 )
2015
9
Catheter-based intervention for pulmonary vein stenosis due to fibrosing mediastinitis: The Mayo Clinic experience. ( 26925456 )
2015
10
Pulmonary Hypertension due to Fibrosing Mediastinitis Treated Successfully With Stenting of Pulmonary VeinA Stenoses. ( 25840104 )
2015
11
The role of PET scan in monitoring the progression of fibrosing mediastinitis. ( 26414539 )
2015
12
Reply: Further Progress in Understanding Fibrosing Mediastinitis. ( 26371818 )
2015
13
Further Progress in Understanding Fibrosing Mediastinitis. ( 26371817 )
2015
14
Fibrosing mediastinitis mimicking postpartum heart failure. ( 25417662 )
2014
15
Targeting B lymphocytes in progressive fibrosing mediastinitis. ( 25360730 )
2014
16
Fibrosing mediastinitis due to Aspergillus with dominant cardiac involvement: report of two autopsy cases with review of literature. ( 24998315 )
2014
17
Fibrosing mediastinitis: a squeeze on arterial and venous segments of the heart. ( 25024126 )
2014
18
Pericardial Varices Secondary to Fibrosing Mediastinitis. ( 24766731 )
2014
19
Thoracoscopic pneumonectomy in management of histoplasmosis and fibrosing mediastinitis. ( 25282249 )
2014
20
Fibrosing Mediastinitis Mimicking Sarcoidosis. ( 24405501 )
2014
21
Pulmonary arterial hypertension in childhood: an unsual presentation with fibrosing mediastinitis. ( 24897974 )
2014
22
Fibrosing mediastinitis: an unusual cause of pulmonary symptoms. ( 23807725 )
2013
23
Fibrosing mediastinitis mimicking bronchogenic carcinoma. ( 23372962 )
2013
24
Fibrosing mediastinitis causing ostial coronary artery compression in a young woman: idiopathic or IgG4? ( 23603700 )
2013
25
Anaplastic large cell lymphoma mimicking fibrosing mediastinitis. ( 24292756 )
2013
26
Intracardiac mass due to fibrosing mediastinitis: the first reported case. ( 23395223 )
2013
27
Medical image. Fibrosing mediastinitis. A rare "mimicker" of lung malignancy. ( 24217596 )
2013
28
The treatment of superior vena cava syndrome secondary to idiopathic fibrosing mediastinitis with balloon angioplasty and stenting. ( 23265133 )
2012
29
Malignant Wegener's granulomatosis with fibrosing mediastinitis and vena cava superior syndrome. ( 22260549 )
2012
30
Fibrosing mediastinitis with severe bilateral pulmonary artery narrowing: RV-RPA bypass with a homograft conduit. ( 22719157 )
2012
31
Imaging of granulomatous fibrosing mediastinitis. ( 22826392 )
2012
32
Fibrosing mediastinitis causing ostial coronary artery compression in a young woman. ( 23257305 )
2012
33
Histopathologic Overlap between Fibrosing Mediastinitis and IgG4-Related Disease. ( 22654916 )
2012
34
Fibrosing mediastinitis as a cause of right claudication. ( 21562863 )
2012
35
Focal fibrosing mediastinitis. ( 21874806 )
2011
36
Fibrosing mediastinitis: clinical presentation, therapeutic outcomes, and adaptive immune response. ( 22033450 )
2011
37
Bilateral breast enlargement: an unusual presentation of superior vena cava obstruction in a hemodialysis patient with fibrosing mediastinitis. ( 19949792 )
2011
38
Percutaneous vascular stent implantation as treatment for central vascular obstruction due to fibrosing mediastinitis. ( 21422386 )
2011
39
Pulmonary artery stenosis secondary to fibrosing mediastinitis: management with cutting balloon angioplasty and endovascular stenting. ( 21278182 )
2011
40
Fibrosing mediastinitis causing acute ostial left main myocardial infarction. ( 20814057 )
2010
41
Fibrosing mediastinitis as a rare mechanism of pulmonary oedema in sarcoidosis. ( 20123854 )
2010
42
Embolization of a symptomatic systemic to pulmonary (right-to-left) venous shunt caused by fibrosing mediastinitis and superior vena caval occlusion. ( 20123198 )
2010
43
Results of intravascular stent placement for fibrosing mediastinitis. ( 20412484 )
2010
44
Octreotide, a somatostatin analogue, in the treatment of chylothorax associated with idiopathic fibrosing mediastinitis. ( 20814178 )
2010
45
Sarcoidosis-associated fibrosing mediastinitis with resultant pulmonary hypertension: a case report and review of the literature. ( 19797887 )
2010
46
Fibrosing mediastinitis as an untypical complication of tuberculosis: case report. ( 19920801 )
2009
47
Unilateral pulmonary vein stenosis secondary to idiopathic fibrosing mediastinitis. ( 21687296 )
2009
48
An unusual case of Aspergillus fibrosing mediastinitis. ( 19766844 )
2009
49
Fibrosing mediastinitis and occlusion of pulmonary veins after radiofrequency ablation. ( 19853136 )
2009
50
Fibrosing mediastinitis associated with complete occlusion of the left main pulmonary artery. ( 19101331 )
2009

Variations for Fibrosing Mediastinitis

Expression for Fibrosing Mediastinitis

Search GEO for disease gene expression data for Fibrosing Mediastinitis.

Pathways for Fibrosing Mediastinitis

GO Terms for Fibrosing Mediastinitis

Sources for Fibrosing Mediastinitis

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
16 ExPASy
18 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 MedGen
42 MeSH
43 MESH via Orphanet
44 MGI
46 NCI
47 NCIt
48 NDF-RT
51 NINDS
52 Novoseek
54 OMIM
55 OMIM via Orphanet
59 PubMed
60 QIAGEN
65 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
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