MCID: FBR028
MIFTS: 25

Fibrosing Mediastinitis

Categories: Rare diseases, Respiratory diseases, Bone diseases

Aliases & Classifications for Fibrosing Mediastinitis

MalaCards integrated aliases 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 Immunoglobulins and Antibodies have been mentioned in the context of this disorder. Affiliated tissues include heart, lung and trachea.

Related Diseases for Fibrosing Mediastinitis

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

(show all 27)
id Related Disease Score Top Affiliating Genes
1 mediastinitis 10.7
2 superior vena cava syndrome 10.1
3 pulmonary hypertension 10.0
4 histoplasmosis 10.0
5 pulmonary vein stenosis 10.0
6 cervicitis 9.9
7 igg4-related disease 9.9
8 lymphoma 9.8
9 thrombosis 9.8
10 pulmonary edema 9.8
11 tracheal stenosis 9.8
12 tuberculosis 9.8
13 connective tissue disease 9.7
14 myocardial infarction 9.7
15 breast cancer 9.7
16 anaplastic large cell lymphoma 9.7
17 hepatitis 9.7
18 peripheral t-cell lymphoma 9.7
19 sarcoma 9.7
20 pyelonephritis 9.7
21 xanthogranulomatous pyelonephritis 9.7
22 esophagitis 9.7
23 blastomycosis 9.7
24 histiocytosis 9.7
25 thyroiditis 9.7
26 bronchogenic cyst 9.7
27 zygomycosis 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 Immunoglobulins
2 Antibodies

Interventional clinical trials:


id Name Status NCT ID Phase Drugs
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
Heart, Lung, Trachea, Lymph Node, Bone, Breast, Thyroid

Publications for Fibrosing Mediastinitis

Articles related to Fibrosing Mediastinitis:

(show top 50) (show all 145)
id Title Authors Year
1
Severe fibrosing mediastinitis with atypical presentation: Effective control with novel therapeutic approach. ( 28808494 )
2017
2
Post-tuberculous fibrosing mediastinitis: a review of the literature. ( 28725444 )
2017
3
Follicular dendritic sarcoma masquerading as fibrosing mediastinitis. ( 28611161 )
2017
4
Bronchoscopic Management of Airway Compression due to Fibrosing Mediastinitis. ( 28719233 )
2017
5
Idiopathic fibrosing mediastinitis mimicking nodal metastasis from breast cancer. ( 28114793 )
2017
6
Mediastinal peripheral T-cell lymphoma diagnosed by repeated biopsies after an initial diagnosis of fibrosing mediastinitis. ( 28932400 )
2017
7
Unexpected Fibrosing Mediastinitis Shown on FDG PET/CT in a Patient With IgG4-Related Disease. ( 28806261 )
2017
8
Pulmonary hypertension associated with combined fibrosing mediastinitis and bronchial anthracofibrosis: A retrospective analysis in a single Chinese hospital. ( 28419740 )
2017
9
Clinical Manifestations of Fibrosing Mediastinitis in Chinese Patients. ( 27824002 )
2016
10
Tubercular fibrosing mediastinitis rare cause for hoarseness of voice. ( 27728713 )
2016
11
Tuberculosis-associated Fibrosing Mediastinitis: Case Report and Literature Review. ( 27625898 )
2016
12
Reply: Further Progress in Understanding Fibrosing Mediastinitis. ( 26371818 )
2015
13
Further Progress in Understanding Fibrosing Mediastinitis. ( 26371817 )
2015
14
Catheter-based intervention for pulmonary vein stenosis due to fibrosing mediastinitis: The Mayo Clinic experience. ( 26925456 )
2015
15
The role of PET scan in monitoring the progression of fibrosing mediastinitis. ( 26414539 )
2015
16
Pulmonary Hypertension Complicating Fibrosing Mediastinitis. ( 26554778 )
2015
17
Successful stenting of left pulmonary veins stenosis resulting from fibrosing mediastinitis. ( 25586119 )
2015
18
Idiopathic fibrosing mediastinitis: spectrum of imaging findings with emphasis on its association with IgG4-related disease. ( 26257115 )
2015
19
Pulmonary Hypertension due to Fibrosing Mediastinitis Treated Successfully With Stenting of Pulmonary VeinA Stenoses. ( 25840104 )
2015
20
Fibrosing mediastinitis: a rare complication of histoplasmosis. ( 26538131 )
2015
21
Fibrosing mediastinitis complicating prior histoplasmosis is associated with human leukocyte antigen DQB1*04:02 - a case control study. ( 25940591 )
2015
22
Fibrosing mediastinitis: a squeeze on arterial and venous segments of the heart. ( 25024126 )
2014
23
Pulmonary arterial hypertension in childhood: an unsual presentation with fibrosing mediastinitis. ( 24897974 )
2014
24
Thoracoscopic pneumonectomy in management of histoplasmosis and fibrosing mediastinitis. ( 25282249 )
2014
25
Fibrosing mediastinitis mimicking postpartum heart failure. ( 25417662 )
2014
26
Fibrosing Mediastinitis Mimicking Sarcoidosis. ( 24405501 )
2014
27
Pericardial Varices Secondary to Fibrosing Mediastinitis. ( 24766731 )
2014
28
Fibrosing mediastinitis due to Aspergillus with dominant cardiac involvement: report of two autopsy cases with review of literature. ( 24998315 )
2014
29
Targeting B lymphocytes in progressive fibrosing mediastinitis. ( 25360730 )
2014
30
Anaplastic large cell lymphoma mimicking fibrosing mediastinitis. ( 24292756 )
2013
31
Fibrosing mediastinitis causing ostial coronary artery compression in a young woman: idiopathic or IgG4? ( 23603700 )
2013
32
Fibrosing mediastinitis mimicking bronchogenic carcinoma. ( 23372962 )
2013
33
Medical image. Fibrosing mediastinitis. A rare "mimicker" of lung malignancy. ( 24217596 )
2013
34
Fibrosing mediastinitis: an unusual cause of pulmonary symptoms. ( 23807725 )
2013
35
Intracardiac mass due to fibrosing mediastinitis: the first reported case. ( 23395223 )
2013
36
The treatment of superior vena cava syndrome secondary to idiopathic fibrosing mediastinitis with balloon angioplasty and stenting. ( 23265133 )
2012
37
Histopathologic Overlap between Fibrosing Mediastinitis and IgG4-Related Disease. ( 22654916 )
2012
38
Imaging of granulomatous fibrosing mediastinitis. ( 22826392 )
2012
39
Fibrosing mediastinitis as a cause of right claudication. ( 21562863 )
2012
40
Fibrosing mediastinitis causing ostial coronary artery compression in a young woman. ( 23257305 )
2012
41
Malignant Wegener's granulomatosis with fibrosing mediastinitis and vena cava superior syndrome. ( 22260549 )
2012
42
Fibrosing mediastinitis with severe bilateral pulmonary artery narrowing: RV-RPA bypass with a homograft conduit. ( 22719157 )
2012
43
Pulmonary artery stenosis secondary to fibrosing mediastinitis: management with cutting balloon angioplasty and endovascular stenting. ( 21278182 )
2011
44
Focal fibrosing mediastinitis. ( 21874806 )
2011
45
Fibrosing mediastinitis: clinical presentation, therapeutic outcomes, and adaptive immune response. ( 22033450 )
2011
46
Percutaneous vascular stent implantation as treatment for central vascular obstruction due to fibrosing mediastinitis. ( 21422386 )
2011
47
Bilateral breast enlargement: an unusual presentation of superior vena cava obstruction in a hemodialysis patient with fibrosing mediastinitis. ( 19949792 )
2011
48
Fibrosing mediastinitis as a rare mechanism of pulmonary oedema in sarcoidosis. ( 20123854 )
2010
49
Embolization of a symptomatic systemic to pulmonary (right-to-left) venous shunt caused by fibrosing mediastinitis and superior vena caval occlusion. ( 20123198 )
2010
50
Fibrosing mediastinitis causing acute ostial left main myocardial infarction. ( 20814057 )
2010

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 HPO
66 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
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