MCID: FBR028
MIFTS: 24

Fibrosing Mediastinitis malady

Categories: Rare diseases, Respiratory diseases, Bone diseases

Aliases & Classifications for Fibrosing Mediastinitis

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Sources:
31ICD10 via Orphanet, 40MESH via Orphanet, 48NIH Rare Diseases, 54Orphanet, 68UMLS, 69UMLS via Orphanet
See all MalaCards sources

Aliases & Descriptions for Fibrosing Mediastinitis:

Name: Fibrosing Mediastinitis 48 54
Mediastinal Fibrosis 48 54 68
Igg4-Related Mediastinitis 48 54
 
Sclerosing Mediastinitis 48 54
Idiopathic Mediastinal Fibrosis 48
Idiopathic Hilar Fibrosis 68

Characteristics:

Orphanet epidemiological data:

54
mediastinal fibrosis:
Inheritance: Not applicable

Classifications:



External Ids:

Orphanet54 ORPHA63999
MESH via Orphanet40 C536136
UMLS via Orphanet69 C0221386, C0264573
ICD10 via Orphanet31 J98.5

Summaries for Fibrosing Mediastinitis

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NIH Rare Diseases:48 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. Affiliated tissues include bone, lung and heart.

Related Diseases for Fibrosing Mediastinitis

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

(show all 23)
idRelated DiseaseScoreTop Affiliating Genes
1mediastinitis10.7
2superior vena cava syndrome10.1
3histoplasmosis10.1
4pulmonary hypertension10.0
5pulmonary vein stenosis10.0
6cervicitis9.9
7pulmonary edema9.8
8tracheal stenosis9.8
9tuberculosis9.8
10igg4-related disease9.8
11connective tissue disease9.7
12myocardial infarction9.7
13anaplastic large cell lymphoma9.7
14hepatitis9.7
15lymphoma9.7
16pyelonephritis9.7
17xanthogranulomatous pyelonephritis9.7
18esophagitis9.7
19blastomycosis9.7
20histiocytosis9.7
21thyroiditis9.7
22bronchogenic cyst9.7
23zygomycosis9.7

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



Diseases related to fibrosing mediastinitis

Symptoms & Phenotypes for Fibrosing Mediastinitis

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Drugs & Therapeutics for Fibrosing Mediastinitis

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

idNameStatusPhaseClinical TrialsCas NumberPubChem Id
1ImmunoglobulinsEarly Phase 16045
2Immunoglobulin GEarly Phase 1265
3AntibodiesEarly Phase 16045

Interventional clinical trials:

idNameStatusNCT IDPhase
1Cohort Study of IgG4RDUnknown statusNCT01670695Early Phase 1

Search NIH Clinical Center for Fibrosing Mediastinitis

Genetic Tests for Fibrosing Mediastinitis

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Anatomical Context for Fibrosing Mediastinitis

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MalaCards organs/tissues related to Fibrosing Mediastinitis:

36
Bone, Lung, Heart, Trachea, Thyroid, Breast

Publications for Fibrosing Mediastinitis

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Articles related to Fibrosing Mediastinitis:

(show top 50)    (show all 136)
idTitleAuthorsYear
1
Tuberculosis-associated Fibrosing Mediastinitis: Case Report and Literature Review. (27625898)
2016
2
Clinical Manifestations of Fibrosing Mediastinitis in Chinese Patients. (27824002)
2016
3
Tubercular fibrosing mediastinitis rare cause for hoarseness of voice. (27728713)
2016
4
Successful stenting of left pulmonary veins stenosis resulting from fibrosing mediastinitis. (25586119)
2015
5
Pulmonary Hypertension due to Fibrosing Mediastinitis Treated Successfully With Stenting of Pulmonary VeinA Stenoses. (25840104)
2015
6
Catheter-based intervention for pulmonary vein stenosis due to fibrosing mediastinitis: The Mayo Clinic experience. (26925456)
2015
7
The role of PET scan in monitoring the progression of fibrosing mediastinitis. (26414539)
2015
8
Fibrosing mediastinitis complicating prior histoplasmosis is associated with human leukocyte antigen DQB1*04:02 - a case control study. (25940591)
2015
9
Idiopathic fibrosing mediastinitis: spectrum of imaging findings with emphasis on its association with IgG4-related disease. (26257115)
2015
10
Fibrosing mediastinitis: a rare complication of histoplasmosis. (26538131)
2015
11
Pulmonary Hypertension Complicating Fibrosing Mediastinitis. (26554778)
2015
12
Reply: Further Progress in Understanding Fibrosing Mediastinitis. (26371818)
2015
13
Further Progress in Understanding Fibrosing Mediastinitis. (26371817)
2015
14
Fibrosing Mediastinitis Mimicking Sarcoidosis. (24405501)
2014
15
Fibrosing mediastinitis mimicking postpartum heart failure. (25417662)
2014
16
Thoracoscopic pneumonectomy in management of histoplasmosis and fibrosing mediastinitis. (25282249)
2014
17
Pericardial Varices Secondary to Fibrosing Mediastinitis. (24766731)
2014
18
Pulmonary arterial hypertension in childhood: an unsual presentation with fibrosing mediastinitis. (24897974)
2014
19
Fibrosing mediastinitis: a squeeze on arterial and venous segments of the heart. (25024126)
2014
20
Fibrosing mediastinitis due to Aspergillus with dominant cardiac involvement: report of two autopsy cases with review of literature. (24998315)
2014
21
Targeting B lymphocytes in progressive fibrosing mediastinitis. (25360730)
2014
22
Fibrosing mediastinitis: an unusual cause of pulmonary symptoms. (23807725)
2013
23
Anaplastic large cell lymphoma mimicking fibrosing mediastinitis. (24292756)
2013
24
Medical image. Fibrosing mediastinitis. A rare "mimicker" of lung malignancy. (24217596)
2013
25
Fibrosing mediastinitis mimicking bronchogenic carcinoma. (23372962)
2013
26
Fibrosing mediastinitis causing ostial coronary artery compression in a young woman: idiopathic or IgG4? (23603700)
2013
27
Intracardiac mass due to fibrosing mediastinitis: the first reported case. (23395223)
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
Fibrosing mediastinitis causing ostial coronary artery compression in a young woman. (23257305)
2012
32
Fibrosing mediastinitis as a cause of right claudication. (21562863)
2012
33
Histopathologic Overlap between Fibrosing Mediastinitis and IgG4-Related Disease. (22654916)
2012
34
Imaging of granulomatous fibrosing mediastinitis. (22826392)
2012
35
Fibrosing mediastinitis: clinical presentation, therapeutic outcomes, and adaptive immune response. (22033450)
2011
36
Focal fibrosing mediastinitis. (21874806)
2011
37
Percutaneous vascular stent implantation as treatment for central vascular obstruction due to fibrosing mediastinitis. (21422386)
2011
38
Bilateral breast enlargement: an unusual presentation of superior vena cava obstruction in a hemodialysis patient with fibrosing mediastinitis. (19949792)
2011
39
Pulmonary artery stenosis secondary to fibrosing mediastinitis: management with cutting balloon angioplasty and endovascular stenting. (21278182)
2011
40
Sarcoidosis-associated fibrosing mediastinitis with resultant pulmonary hypertension: a case report and review of the literature. (19797887)
2010
41
Fibrosing mediastinitis causing acute ostial left main myocardial infarction. (20814057)
2010
42
Octreotide, a somatostatin analogue, in the treatment of chylothorax associated with idiopathic fibrosing mediastinitis. (20814178)
2010
43
Results of intravascular stent placement for fibrosing mediastinitis. (20412484)
2010
44
Embolization of a symptomatic systemic to pulmonary (right-to-left) venous shunt caused by fibrosing mediastinitis and superior vena caval occlusion. (20123198)
2010
45
Fibrosing mediastinitis as a rare mechanism of pulmonary oedema in sarcoidosis. (20123854)
2010
46
Fibrosing mediastinitis associated with complete occlusion of the left main pulmonary artery. (19101331)
2009
47
An unusual case of Aspergillus fibrosing mediastinitis. (19766844)
2009
48
Unilateral pulmonary vein stenosis secondary to idiopathic fibrosing mediastinitis. (21687296)
2009
49
Fibrosing mediastinitis and occlusion of pulmonary veins after radiofrequency ablation. (19853136)
2009
50
Fibrosing mediastinitis as an untypical complication of tuberculosis: case report. (19920801)
2009

Variations for Fibrosing Mediastinitis

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Expression for genes affiliated with Fibrosing Mediastinitis

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Search GEO for disease gene expression data for Fibrosing Mediastinitis.

Pathways for genes affiliated with Fibrosing Mediastinitis

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GO Terms for genes affiliated with Fibrosing Mediastinitis

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Sources for Fibrosing Mediastinitis

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2CDC
6CNVD
10DGIdb
15ExPASy
16FDA
17FMA
27GTR
28HGMD
29HMDB
30ICD10
31ICD10 via Orphanet
32ICD9CM
33IUPHAR
34KEGG
37MedGen
39MeSH
40MESH via Orphanet
41MGI
44NCI
45NCIt
46NDF-RT
49NINDS
50Novoseek
52OMIM
53OMIM via Orphanet
57PubMed
58QIAGEN
63SNOMED-CT via Orphanet
67Tumor Gene Family of Databases
68UMLS
69UMLS via Orphanet