MCID: FBR028
MIFTS: 28

Fibrosing Mediastinitis

Categories: Rare diseases, Respiratory diseases, Bone diseases

Aliases & Classifications for Fibrosing Mediastinitis

MalaCards integrated aliases for Fibrosing Mediastinitis:

Name: Fibrosing Mediastinitis 49 55
Mediastinal Fibrosis 49 55 69
Igg4-Related Mediastinitis 49 55
Sclerosing Mediastinitis 49 55
Idiopathic Mediastinal Fibrosis 49
Idiopathic Hilar Fibrosis 69

Characteristics:

Orphanet epidemiological data:

55
igg4-related mediastinitis
Inheritance: Not applicable;

Classifications:



External Ids:

Orphanet 55 ORPHA63999
MESH via Orphanet 42 C536136
UMLS via Orphanet 70 C0221386 C0264573
ICD10 via Orphanet 33 J98.5

Summaries for Fibrosing Mediastinitis

NIH Rare Diseases : 49 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 fibrosclerosis, multifocal and mediastinitis. 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

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):


# Name Status Phase Clinical Trials Cas Number PubChem Id
1 Immunoglobulins
2 Antibodies

Interventional clinical trials:


# 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:

38
Heart, Lung, Trachea, Lymph Node, Bone, Thyroid, T Cells

Publications for Fibrosing Mediastinitis

Articles related to Fibrosing Mediastinitis:

(show top 50) (show all 149)
# Title Authors Year
1
Fibrosing mediastinitis with pulmonary hypertension as a complication of pulmonary vein stenosis: A case report and review of the literature. ( 29369193 )
2018
2
Endovascular Management of SVC Syndrome due to Fibrosing Mediastinitis-A Feasibility and Safety Analysis. ( 29433409 )
2018
3
Clinical and imaging spectrum of tuberculosis-associated fibrosing mediastinitis. ( 29356415 )
2018
4
Bronchoscopic Management of Airway Compression due to Fibrosing Mediastinitis. ( 28719233 )
2017
5
Pulmonary hypertension associated with combined fibrosing mediastinitis and bronchial anthracofibrosis: A retrospective analysis in a single Chinese hospital. ( 28419740 )
2017
6
Follicular dendritic sarcoma masquerading as fibrosing mediastinitis. ( 28611161 )
2017
7
GWAS of 58 Individuals with Fibrosing Mediastinitis Reveals Possible Underlying Genetic Susceptibility. ( 29043856 )
2017
8
Post-tuberculous fibrosing mediastinitis: a review of the literature. ( 28725444 )
2017
9
Idiopathic fibrosing mediastinitis mimicking nodal metastasis from breast cancer. ( 28114793 )
2017
10
Mediastinal peripheral T-cell lymphoma diagnosed by repeated biopsies after an initial diagnosis of fibrosing mediastinitis. ( 28932400 )
2017
11
Unexpected Fibrosing Mediastinitis Shown on FDG PET/CT in a Patient With IgG4-Related Disease. ( 28806261 )
2017
12
Severe fibrosing mediastinitis with atypical presentation: Effective control with novel therapeutic approach. ( 28808494 )
2017
13
Tuberculosis-associated Fibrosing Mediastinitis: Case Report and Literature Review. ( 27625898 )
2016
14
Tubercular fibrosing mediastinitis rare cause for hoarseness of voice. ( 27728713 )
2016
15
Clinical Manifestations of Fibrosing Mediastinitis in Chinese Patients. ( 27824002 )
2016
16
Pulmonary Hypertension Complicating Fibrosing Mediastinitis. ( 26554778 )
2015
17
Fibrosing mediastinitis: a rare complication of histoplasmosis. ( 26538131 )
2015
18
Catheter-based intervention for pulmonary vein stenosis due to fibrosing mediastinitis: The Mayo Clinic experience. ( 26925456 )
2015
19
Pulmonary Hypertension due to Fibrosing Mediastinitis Treated Successfully With Stenting of Pulmonary VeinA Stenoses. ( 25840104 )
2015
20
Idiopathic fibrosing mediastinitis: spectrum of imaging findings with emphasis on its association with IgG4-related disease. ( 26257115 )
2015
21
Successful stenting of left pulmonary veins stenosis resulting from fibrosing mediastinitis. ( 25586119 )
2015
22
Further Progress in Understanding Fibrosing Mediastinitis. ( 26371817 )
2015
23
Reply: Further Progress in Understanding Fibrosing Mediastinitis. ( 26371818 )
2015
24
The role of PET scan in monitoring the progression of fibrosing mediastinitis. ( 26414539 )
2015
25
Fibrosing mediastinitis complicating prior histoplasmosis is associated with human leukocyte antigen DQB1*04:02 - a case control study. ( 25940591 )
2015
26
Pericardial Varices Secondary to Fibrosing Mediastinitis. ( 24766731 )
2014
27
Fibrosing Mediastinitis Mimicking Sarcoidosis. ( 24405501 )
2014
28
Fibrosing mediastinitis due to Aspergillus with dominant cardiac involvement: report of two autopsy cases with review of literature. ( 24998315 )
2014
29
Thoracoscopic pneumonectomy in management of histoplasmosis and fibrosing mediastinitis. ( 25282249 )
2014
30
Fibrosing mediastinitis: a squeeze on arterial and venous segments of the heart. ( 25024126 )
2014
31
Pulmonary arterial hypertension in childhood: an unsual presentation with fibrosing mediastinitis. ( 24897974 )
2014
32
Targeting B lymphocytes in progressive fibrosing mediastinitis. ( 25360730 )
2014
33
Fibrosing mediastinitis mimicking postpartum heart failure. ( 25417662 )
2014
34
Fibrosing mediastinitis: an unusual cause of pulmonary symptoms. ( 23807725 )
2013
35
Medical image. Fibrosing mediastinitis. A rare "mimicker" of lung malignancy. ( 24217596 )
2013
36
Fibrosing mediastinitis causing ostial coronary artery compression in a young woman: idiopathic or IgG4? ( 23603700 )
2013
37
Anaplastic large cell lymphoma mimicking fibrosing mediastinitis. ( 24292756 )
2013
38
Intracardiac mass due to fibrosing mediastinitis: the first reported case. ( 23395223 )
2013
39
Fibrosing mediastinitis mimicking bronchogenic carcinoma. ( 23372962 )
2013
40
Imaging of granulomatous fibrosing mediastinitis. ( 22826392 )
2012
41
Histopathologic Overlap between Fibrosing Mediastinitis and IgG4-Related Disease. ( 22654916 )
2012
42
Fibrosing mediastinitis causing ostial coronary artery compression in a young woman. ( 23257305 )
2012
43
Fibrosing mediastinitis with severe bilateral pulmonary artery narrowing: RV-RPA bypass with a homograft conduit. ( 22719157 )
2012
44
Malignant Wegener's granulomatosis with fibrosing mediastinitis and vena cava superior syndrome. ( 22260549 )
2012
45
Fibrosing mediastinitis as a cause of right claudication. ( 21562863 )
2012
46
The treatment of superior vena cava syndrome secondary to idiopathic fibrosing mediastinitis with balloon angioplasty and stenting. ( 23265133 )
2012
47
Percutaneous vascular stent implantation as treatment for central vascular obstruction due to fibrosing mediastinitis. ( 21422386 )
2011
48
Bilateral breast enlargement: an unusual presentation of superior vena cava obstruction in a hemodialysis patient with fibrosing mediastinitis. ( 19949792 )
2011
49
Focal fibrosing mediastinitis. ( 21874806 )
2011
50
Fibrosing mediastinitis: clinical presentation, therapeutic outcomes, and adaptive immune response. ( 22033450 )
2011

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
27 GO
28 GTR
29 HGMD
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 MedGen
41 MeSH
42 MESH via Orphanet
43 MGI
45 NCI
46 NCIt
47 NDF-RT
50 NINDS
51 Novoseek
53 OMIM
54 OMIM via Orphanet
58 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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