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 135)
# 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 10.3
6 sarcoidosis 1 10.3
7 igg4-related disease 10.2
8 47,xyy 10.2
9 hydrops, lactic acidosis, and sideroblastic anemia 10.1
10 pulmonary edema 10.1
11 tracheal stenosis 10.1
12 dysphagia 10.1
13 pulmonary hypertension, primary, 1 10.0
14 varicose veins 10.0
15 alpha/beta t-cell lymphopenia with gamma/delta t-cell expansion, severe cytomegalovirus infection, and autoimmunity 10.0
16 pulmonary hypertension, chronic thromboembolic, without deep vein thrombosis 10.0
17 cardiac arrest 10.0
18 thrombosis 10.0
19 heart disease 10.0
20 cervicitis 10.0
21 congestive heart failure 10.0
22 pulmonary embolism 10.0
23 chronic thromboembolic pulmonary hypertension 10.0
24 retroperitoneal fibrosis 10.0
25 headache 10.0
26 bronchitis 9.9
27 spondylarthropathy 9.9
28 autoimmune disease 9.9
29 fibrosclerosis, multifocal 9.9
30 pulmonary disease, chronic obstructive 9.9
31 antiphospholipid syndrome 9.9
32 connective tissue disease 9.9
33 anca-associated vasculitis 9.9
34 aneurysm 9.9
35 back pain 9.9
36 breast cancer 9.9
37 pancreatic cancer 9.9
38 pulmonary venoocclusive disease 1, autosomal dominant 9.9
39 langerhans cell histiocytosis 9.9
40 myocardial infarction 9.9
41 leukemia, acute lymphoblastic 3 9.9
42 hyperlipoproteinemia, type iii 9.9
43 anaplastic large cell lymphoma 9.9
44 peripheral t-cell lymphoma 9.9
45 lymphoma 9.9
46 hepatic veno-occlusive disease 9.9
47 castleman disease 9.9
48 brucellosis 9.9
49 bartonellosis 9.9
50 sarcoma 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 488)
# Title Authors PMID Year
1
A review of endovascular stenting for superior vena cava syndrome in fibrosing mediastinitis. 61
31804157 2019
2
Catheter based treatments for fibrosing mediastinitis. 61
30790443 2019
3
[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
4
Mediastinal Fibrosis, Pulmonary Vein Stenosis, and the Role of Transesophageal Echocardiography. 61
31806471 2019
5
Balloon angioplasty or stent implantation for pulmonary vein stenosis caused by fibrosing mediastinitis: a systematic review. 61
31737523 2019
6
Tracheal Stenosis Due to IgG4-Related Fibrosing Mediastinitis. 61
30797571 2019
7
Fibrosing mediastinitis mimicking a lung carcinoma. 61
31368497 2019
8
Rituximab to Treat Fibrosing Mediastinitis-Associated Recurrent Hemoptysis. 61
31356339 2019
9
Central Nervous System Infection with Histoplasma capsulatum. 61
31344869 2019
10
Pulmonary Arterial Bypass Surgery for Fibrosing Mediastinitis Causing Severe Pulmonary Hypertension. 61
30447188 2019
11
Tuberculosis mediastinal fibrosis misdiagnosed as chronic bronchitis for 10 years: a case report. 61
31367572 2019
12
Fibrosing mediastinitis. 61
31496535 2019
13
Multimodality Imaging of Focal and Diffuse Fibrosing Mediastinitis. 61
30951437 2019
14
Aggressive and progressive fibrosing mediastinitis involving the thoracic spine mimicking malignancy: A case report. 61
30805073 2019
15
A case of fibrosing mediastinitis complicated by pulmonary hypertension and right-sided heart failure. 61
30202958 2019
16
Chronic thromboembolic pulmonary hypertension versus fibrosing mediastinitis. 61
30694812 2019
17
Fungal fibrosing mediastinitis in pregnancy - Case report with review of literature. 61
30693874 2019
18
Sclerosing Mediastinitis Causing Unilateral Pulmonary Edema Due to Left Atrial and Pulmonary Venous Compression. A Case Report and Literature Review. 61
30810679 2019
19
Is Long-Term Anticoagulation Required after Stent Placement for Benign Superior Vena Cava Syndrome? 61
30396845 2018
20
Chronic Periaortitis: an Update. 61
30397845 2018
21
Fibrosing Mediastinitis: a Rare Cause of Unilateral Absent Lung Perfusion on a V/Q Scan. 61
30344792 2018
22
Intraluminal Arterial Filling Defects Misdiagnosed as Pulmonary Emboli: What Else Could They Be? 61
30122175 2018
23
Mediastinal and retroperitoneal fibrosis as a manifestation of breast cancer metastasis: A case report and literature review. 61
30095661 2018
24
A case of sarcoidosis-associated pulmonary hypertension masquerading as chronic thromboembolic pulmonary hypertension. 61
29537341 2018
25
Lethal fibrosing mediastinitis in a child possibly due to Mycobacterium tuberculosis. 61
29635837 2018
26
IgG4-related fibrosing mediastinitis diagnosed with computed tomography-guided percutaneous needle biopsy: Two case reports and a review of the literature. 61
29851832 2018
27
Genome-Wide Association Study of 58 Individuals with Fibrosing Mediastinitis Reveals Possible Underlying Genetic Susceptibility. 61
29043856 2018
28
Fibrosing mediastinitis-related pulmonary artery and vein stenosis-limiting chemotherapy. 61
29754128 2018
29
Comparison of Covered Versus Uncovered Stents for Benign Superior Vena Cava (SVC) Obstruction. 61
29492630 2018
30
Clinical and imaging spectrum of tuberculosis-associated fibrosing mediastinitis. 61
29356415 2018
31
Fibrosing Mediastinitis: Uncommon Life-threatening Complication of Histoplasmosis. 61
29946500 2018
32
Endovascular Management of SVC Syndrome due to Fibrosing Mediastinitis-A Feasibility and Safety Analysis. 61
29433409 2018
33
Pulmonary hypertension associated with combined fibrosing mediastinitis and bronchial anthracofibrosis: A retrospective analysis in a single Chinese hospital. 61
28419740 2018
34
Fibrosing mediastinitis with pulmonary hypertension as a complication of pulmonary vein stenosis: A case report and review of the literature. 61
29369193 2018
35
Recurrent Pneumonia due to Fibrosing Mediastinitis in a Teenage Girl: A Case Report with Long-Term Follow-Up. 61
29744231 2018
36
Pulmonary venous thrombosis secondary to radiofrequency ablation of the pulmonary veins. 61
29255670 2018
37
Primary Stenting Is Not Necessary in Benign Central Venous Stenosis. 61
28807744 2018
38
Kissing Stents for Superior Vena Cava Syndrome Due to Mediastinal Fibrosis. 61
29872339 2018
39
Idiopathic fibrosing mediastinitis mimicking nodal metastasis from breast cancer. 61
28114793 2017
40
Mediastinal peripheral T-cell lymphoma diagnosed by repeated biopsies after an initial diagnosis of fibrosing mediastinitis. 61
28932400 2017
41
Unexpected Fibrosing Mediastinitis Shown on FDG PET/CT in a Patient With IgG4-Related Disease. 61
28806261 2017
42
Medical Management of Pulmonary Hypertension with Unclear and/or Multifactorial Mechanisms (Group 5): Is There a Role for Pulmonary Arterial Hypertension Medications? 61
29046979 2017
43
Bronchoscopic Management of Airway Compression due to Fibrosing Mediastinitis. 61
28719233 2017
44
Severe fibrosing mediastinitis with atypical presentation: Effective control with novel therapeutic approach. 61
28808494 2017
45
Black and White Esophagus: Rare Presentations of Severe Esophageal Ischemia. 61
28823340 2017
46
Idiopathic Mediastinal Fibrosis: a Systemic Immune-Mediated Disorder. A Case Series and a Review of the Literature. 61
27553003 2017
47
Follicular dendritic sarcoma masquerading as fibrosing mediastinitis. 61
28611161 2017
48
Idiopathic retroperitoneal fibrosis and its overlap with IgG4-related disease. 61
28070877 2017
49
[Clinical characteristics and prognosis of mediastinal fibrosis]. 61
28297815 2017
50
Tumefactive Fibroinflammatory Lesion: A Diagnostic Dilemma. 61
28360448 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
Content
Loading form....