MCID: FBR028
MIFTS: 26

Fibrosing Mediastinitis

Categories: Bone diseases, Rare diseases, Respiratory diseases

Aliases & Classifications for Fibrosing Mediastinitis

MalaCards integrated aliases for Fibrosing Mediastinitis:

Name: Fibrosing Mediastinitis 52 58
Mediastinal Fibrosis 52 58 71
Igg4-Related Mediastinitis 52 58
Sclerosing Mediastinitis 52 58
Idiopathic Mediastinal Fibrosis 52
Idiopathic Hilar Fibrosis 71

Characteristics:

Orphanet epidemiological data:

58
igg4-related mediastinitis
Inheritance: Not applicable;

Classifications:

Orphanet: 58  
Rare respiratory diseases
Rare systemic and rhumatological diseases


External Ids:

MESH via Orphanet 44 C536136
ICD10 via Orphanet 33 J98.5
UMLS via Orphanet 72 C0221386 C0264573
Orphanet 58 ORPHA63999
UMLS 71 C0221386 C0264573

Summaries for Fibrosing Mediastinitis

NIH Rare Diseases : 52 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.

MalaCards based summary : Fibrosing Mediastinitis, also known as mediastinal fibrosis, is related to mediastinitis and histoplasmosis. 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 top 50) (show all 133)
# Related Disease Score Top Affiliating Genes
1 mediastinitis 11.0
2 histoplasmosis 10.6
3 pulmonary hypertension 10.6
4 fibrosis of extraocular muscles, congenital, 1 10.5
5 pulmonary vein stenosis 10.4
6 sarcoidosis 1 10.3
7 igg4-related disease 10.2
8 47,xyy 10.2
9 pulmonary edema 10.1
10 tracheal stenosis 10.1
11 dysphagia 10.1
12 pulmonary hypertension, primary, 1 10.0
13 varicose veins 10.0
14 alpha/beta t-cell lymphopenia with gamma/delta t-cell expansion, severe cytomegalovirus infection, and autoimmunity 10.0
15 pulmonary hypertension, chronic thromboembolic, without deep vein thrombosis 10.0
16 cardiac arrest 10.0
17 thrombosis 10.0
18 heart disease 10.0
19 cervicitis 10.0
20 congestive heart failure 10.0
21 pulmonary embolism 10.0
22 chronic thromboembolic pulmonary hypertension 10.0
23 retroperitoneal fibrosis 10.0
24 bronchitis 9.9
25 spondylarthropathy 9.9
26 breast cancer 9.9
27 migraine with or without aura 1 9.9
28 pancreatic cancer 9.9
29 pulmonary venoocclusive disease 1, autosomal dominant 9.9
30 langerhans cell histiocytosis 9.9
31 myocardial infarction 9.9
32 leukemia, acute lymphoblastic 3 9.9
33 anaplastic large cell lymphoma 9.9
34 peripheral t-cell lymphoma 9.9
35 lymphoma 9.9
36 hepatic veno-occlusive disease 9.9
37 castleman disease 9.9
38 brucellosis 9.9
39 bartonellosis 9.9
40 sarcoma 9.9
41 respiratory failure 9.9
42 esophageal varix 9.9
43 pyelonephritis 9.9
44 xanthogranulomatous pyelonephritis 9.9
45 hemopericardium 9.9
46 pericardial effusion 9.9
47 allergic hypersensitivity disease 9.9
48 bronchopneumonia 9.9
49 blastomycosis 9.9
50 sclerosing cholangitis 9.9

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

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Creation of a Biospecimen Repository From Patients With Interstitial Lung Diseases (ILD) Recruiting NCT03478826

Search NIH Clinical Center for Fibrosing Mediastinitis

Genetic Tests for Fibrosing Mediastinitis

Anatomical Context for Fibrosing Mediastinitis

MalaCards organs/tissues related to Fibrosing Mediastinitis:

40
Lung, Heart, Trachea, Lymph Node, Thyroid, Bone, Breast

Publications for Fibrosing Mediastinitis

Articles related to Fibrosing Mediastinitis:

(show top 50) (show all 499)
# Title Authors PMID Year
1
Commentary: Fibrosing mediastinitis or how to give your thoracic surgeon a diagnostic migraine. 61
32562749 2020
2
Massive dilatation of the hemiazygos vein secondary to fibrosing mediastinitis. 61
32497639 2020
3
Fibrosing Mediastinitis Requiring Multimodality Treatment to Maintain Pulmonary Vein Patency. 61
32450211 2020
4
A review of endovascular stenting for superior vena cava syndrome in fibrosing mediastinitis. 61
31804157 2020
5
Successful Stenting of Bilateral Pulmonary Veins Stenosis Secondary to Idiopathic Fibrosing Mediastinitis. 61
32007463 2020
6
Diagnosis of an unusual case of idiopathic mediastinal fibrosis by 18F-FDG PET/CT. 61
32099588 2020
7
Mediastinal Fibrosis, Pulmonary Vein Stenosis, and the Role of Transesophageal Echocardiography. 61
31806471 2020
8
Superior vena cava stenting in IgG4-associated mediastinal fibrosis. 61
32241172 2020
9
Fibrosing mediastinitis mimicking as chronic pulmonary thromboembolism. 61
32201604 2020
10
A Novel Technique for the Management of Massive Hemoptysis: The Customized Endobronchial Silicone Blocker. 61
32352902 2020
11
Successful management of fibrosing mediastinitis with severe vascular compromise: Report of two cases and literature review. 61
31890562 2020
12
Aftermath of pulmonary tuberculosis: computed tomography assessment. 61
32322321 2020
13
Mimickers of chronic thromboembolic pulmonary hypertension on imaging tests: a review. 61
32257112 2020
14
Catheter based treatments for fibrosing mediastinitis. 61
30790443 2019
15
[Feasibility and efficacy of percutaneous pulmonary vein stenting for the treatment of patients with severe pulmonary vein stenosis due to fibrosing mediastinitis]. 61
31648464 2019
16
Balloon angioplasty or stent implantation for pulmonary vein stenosis caused by fibrosing mediastinitis: a systematic review. 61
31737523 2019
17
Fibrosing mediastinitis mimicking a lung carcinoma. 61
31368497 2019
18
Tracheal Stenosis Due to IgG4-Related Fibrosing Mediastinitis. 61
30797571 2019
19
Rituximab to Treat Fibrosing Mediastinitis-Associated Recurrent Hemoptysis. 61
31356339 2019
20
Central Nervous System Infection with Histoplasma capsulatum. 61
31344869 2019
21
Pulmonary Arterial Bypass Surgery for Fibrosing Mediastinitis Causing Severe Pulmonary Hypertension. 61
30447188 2019
22
Tuberculosis mediastinal fibrosis misdiagnosed as chronic bronchitis for 10 years: a case report. 61
31367572 2019
23
Fibrosing mediastinitis. 61
31496535 2019
24
Multimodality Imaging of Focal and Diffuse Fibrosing Mediastinitis. 61
30951437 2019
25
Aggressive and progressive fibrosing mediastinitis involving the thoracic spine mimicking malignancy: A case report. 61
30805073 2019
26
A case of fibrosing mediastinitis complicated by pulmonary hypertension and right-sided heart failure. 61
30202958 2019
27
Chronic thromboembolic pulmonary hypertension versus fibrosing mediastinitis. 61
30694812 2019
28
Fungal fibrosing mediastinitis in pregnancy - Case report with review of literature. 61
30693874 2019
29
Sclerosing Mediastinitis Causing Unilateral Pulmonary Edema Due to Left Atrial and Pulmonary Venous Compression. A Case Report and Literature Review. 61
30810679 2019
30
Is Long-Term Anticoagulation Required after Stent Placement for Benign Superior Vena Cava Syndrome? 61
30396845 2018
31
Chronic Periaortitis: an Update. 61
30397845 2018
32
Fibrosing Mediastinitis: a Rare Cause of Unilateral Absent Lung Perfusion on a V/Q Scan. 61
30344792 2018
33
Intraluminal Arterial Filling Defects Misdiagnosed as Pulmonary Emboli: What Else Could They Be? 61
30122175 2018
34
Mediastinal and retroperitoneal fibrosis as a manifestation of breast cancer metastasis: A case report and literature review. 61
30095661 2018
35
A case of sarcoidosis-associated pulmonary hypertension masquerading as chronic thromboembolic pulmonary hypertension. 61
29537341 2018
36
Lethal fibrosing mediastinitis in a child possibly due to Mycobacterium tuberculosis. 61
29635837 2018
37
IgG4-related fibrosing mediastinitis diagnosed with computed tomography-guided percutaneous needle biopsy: Two case reports and a review of the literature. 61
29851832 2018
38
Genome-Wide Association Study of 58 Individuals with Fibrosing Mediastinitis Reveals Possible Underlying Genetic Susceptibility. 61
29043856 2018
39
Fibrosing mediastinitis-related pulmonary artery and vein stenosis-limiting chemotherapy. 61
29754128 2018
40
Comparison of Covered Versus Uncovered Stents for Benign Superior Vena Cava (SVC) Obstruction. 61
29492630 2018
41
Clinical and imaging spectrum of tuberculosis-associated fibrosing mediastinitis. 61
29356415 2018
42
Fibrosing Mediastinitis: Uncommon Life-threatening Complication of Histoplasmosis. 61
29946500 2018
43
Endovascular Management of SVC Syndrome due to Fibrosing Mediastinitis-A Feasibility and Safety Analysis. 61
29433409 2018
44
Pulmonary hypertension associated with combined fibrosing mediastinitis and bronchial anthracofibrosis: A retrospective analysis in a single Chinese hospital. 61
28419740 2018
45
Fibrosing mediastinitis with pulmonary hypertension as a complication of pulmonary vein stenosis: A case report and review of the literature. 61
29369193 2018
46
Primary Stenting Is Not Necessary in Benign Central Venous Stenosis. 61
28807744 2018
47
Pulmonary venous thrombosis secondary to radiofrequency ablation of the pulmonary veins. 61
29255670 2018
48
Recurrent Pneumonia due to Fibrosing Mediastinitis in a Teenage Girl: A Case Report with Long-Term Follow-Up. 61
29744231 2018
49
Kissing Stents for Superior Vena Cava Syndrome Due to Mediastinal Fibrosis. 61
29872339 2018
50
Idiopathic fibrosing mediastinitis mimicking nodal metastasis from breast cancer. 61
28114793 2017

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
17 EFO
18 ExPASy
19 FMA
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
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
53 NINDS
54 Novoseek
56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
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