MCID: FBR028
MIFTS: 24

Fibrosing Mediastinitis

Categories: Bone diseases, Rare diseases, Respiratory diseases

Aliases & Classifications for Fibrosing Mediastinitis

MalaCards integrated aliases for Fibrosing Mediastinitis:

Name: Fibrosing Mediastinitis 20 58
Mediastinal Fibrosis 20 58 70
Igg4-Related Mediastinitis 20 58
Sclerosing Mediastinitis 20 58
Idiopathic Mediastinal Fibrosis 20
Idiopathic Hilar Fibrosis 70

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 45 C536136
ICD10 via Orphanet 33 J98.5
UMLS via Orphanet 71 C0221386 C0264573
Orphanet 58 ORPHA63999
UMLS 70 C0221386 C0264573

Summaries for Fibrosing Mediastinitis

GARD : 20 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 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 127)
# Related Disease Score Top Affiliating Genes
1 mediastinitis 11.1
2 histoplasmosis 10.7
3 pulmonary hypertension 10.7
4 pulmonary vein stenosis 10.4
5 sarcoidosis 1 10.4
6 igg4-related disease 10.4
7 mycobacterium tuberculosis 1 10.3
8 fibrosis of extraocular muscles, congenital, 1 10.2
9 pulmonary edema 10.2
10 tracheal stenosis 10.2
11 pulmonary hypertension, primary, 1 10.1
12 varicose veins 10.1
13 alpha/beta t-cell lymphopenia with gamma/delta t-cell expansion, severe cytomegalovirus infection, and autoimmunity 10.1
14 pulmonary hypertension, chronic thromboembolic, without deep vein thrombosis 10.1
15 thrombosis 10.1
16 cervicitis 10.1
17 vasculitis 10.1
18 chronic thromboembolic pulmonary hypertension 10.1
19 retroperitoneal fibrosis 10.1
20 dysphagia 10.1
21 migraine with or without aura 1 10.0
22 scleroderma, familial progressive 10.0
23 pulmonary venoocclusive disease 1, autosomal dominant 10.0
24 langerhans cell histiocytosis 10.0
25 proteinuria, chronic benign 10.0
26 anaplastic large cell lymphoma 10.0
27 peripheral t-cell lymphoma 10.0
28 infective endocarditis 10.0
29 lymphoma 10.0
30 ptosis 10.0
31 cardiac arrest 10.0
32 hepatic veno-occlusive disease 10.0
33 b-lymphoblastic leukemia/lymphoma 10.0
34 castleman disease 10.0
35 endocarditis 10.0
36 brucellosis 10.0
37 bartonellosis 10.0
38 sarcoma 10.0
39 anhidrosis 10.0
40 respiratory failure 10.0
41 esophageal varix 10.0
42 heart disease 10.0
43 pyelonephritis 10.0
44 xanthogranulomatous pyelonephritis 10.0
45 hemopericardium 10.0
46 horner's syndrome 10.0
47 pericardial effusion 10.0
48 allergic disease 10.0
49 bronchopneumonia 10.0
50 blastomycosis 10.0

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
Heart, Trachea

Publications for Fibrosing Mediastinitis

Articles related to Fibrosing Mediastinitis:

(show top 50) (show all 514)
# Title Authors PMID Year
1
Complete lung collapse as a rare complication of sarcoidosis-associated mediastinal lymphadenopathy. 61
33732471 2021
2
Fibrosing Mediastinitis Associated With Tuberculosis in Children. 61
33710984 2021
3
A rare case of primary angiosarcoma of the anterior mediastinum. 61
33222090 2021
4
Evaluation of Outcomes Following Pulmonary Artery Stenting in Fibrosing Mediastinitis. 61
33205295 2021
5
Tuberculosis lymphadenopathy: A rare etiology of the superior vena cava syndrome. 61
33546823 2021
6
CD4+CTLs in Fibrosing Mediastinitis Linked to Histoplasma capsulatum. 61
33328214 2021
7
An unusual pediatric case of tuberculosis-associated mediastinal fibrosis with concomitant pulmonary arterial and venous occlusion. 61
33259092 2021
8
Ptosis and Miosis Associated with Fibrosing Mediastinitis. 61
33431787 2021
9
Thoracic Cardiovascular Complications of Tuberculosis. 61
33475319 2021
10
Rituximab to Treat Fibrosing Mediastinitis-Associated Recurrent Hemoptysis. 61
31356339 2020
11
IgG4-related fibrosing mediastinitis: clinical presentation, treatment efficacy and comparison with IgG4-RD without fibrosing mediastinitis. 61
32896252 2020
12
[Advances in diagnosis and therapy of pulmonary vascular stenosis induced by fibrosing mediastinitis]. 61
33076618 2020
13
Imaging features and differential diagnoses of non-neoplastic diffuse mediastinal diseases. 61
33057984 2020
14
Imaging of IgG4-related fibrosing mediastinitis. 61
32972745 2020
15
Massive Dilatation of the Hemiazygos Vein Secondary to Fibrosing Mediastinitis. 61
32497639 2020
16
A Rare Case of Bronchomediastinal Pulmonary Vein Fistula due to Fibrosing Mediastinitis. 61
33072449 2020
17
A case of IgG4-related anterior mediastinal sclerosing disease coexisting with autoimmune pancreatitis. 61
32705358 2020
18
Diagnosis of an unusual case of idiopathic mediastinal fibrosis by 18F-FDG PET/CT. 61
32099588 2020
19
A review of endovascular stenting for superior vena cava syndrome in fibrosing mediastinitis. 61
31804157 2020
20
Successful Stenting of Bilateral Pulmonary Veins Stenosis Secondary to Idiopathic Fibrosing Mediastinitis. 61
32007463 2020
21
Mediastinal Fibrosis, Pulmonary Vein Stenosis, and the Role of Transesophageal Echocardiography. 61
31806471 2020
22
Superior vena cava stenting in IgG4-associated mediastinal fibrosis. 61
32241172 2020
23
A Novel Technique for the Management of Massive Hemoptysis: The Customized Endobronchial Silicone Blocker. 61
32352902 2020
24
Fibrosing mediastinitis mimicking as chronic pulmonary thromboembolism. 61
32201604 2020
25
Successful management of fibrosing mediastinitis with severe vascular compromise: Report of two cases and literature review. 61
31890562 2020
26
Aftermath of pulmonary tuberculosis: computed tomography assessment. 61
32322321 2020
27
Commentary: Fibrosing Mediastinitis or How to Give Your Thoracic Surgeon a Diagnostic Migraine. 61
32562749 2020
28
Mimickers of chronic thromboembolic pulmonary hypertension on imaging tests: a review. 61
32257112 2020
29
Fibrosing Mediastinitis Requiring Multimodality Treatment to Maintain Pulmonary Vein Patency. 61
32450211 2020
30
Catheter based treatments for fibrosing mediastinitis. 61
30790443 2019
31
Tracheal Stenosis Due to IgG4-Related Fibrosing Mediastinitis. 61
30797571 2019
32
Balloon angioplasty or stent implantation for pulmonary vein stenosis caused by fibrosing mediastinitis: a systematic review. 61
31737523 2019
33
Fibrosing mediastinitis mimicking a lung carcinoma. 61
31368497 2019
34
[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
35
Central Nervous System Infection with Histoplasma capsulatum. 61
31344869 2019
36
Fibrosing mediastinitis. 61
31496535 2019
37
Pulmonary Arterial Bypass Surgery for Fibrosing Mediastinitis Causing Severe Pulmonary Hypertension. 61
30447188 2019
38
Tuberculosis mediastinal fibrosis misdiagnosed as chronic bronchitis for 10 years: a case report. 61
31367572 2019
39
Multimodality Imaging of Focal and Diffuse Fibrosing Mediastinitis. 61
30951437 2019
40
Aggressive and progressive fibrosing mediastinitis involving the thoracic spine mimicking malignancy: A case report. 61
30805073 2019
41
A case of fibrosing mediastinitis complicated by pulmonary hypertension and right-sided heart failure. 61
30202958 2019
42
Chronic thromboembolic pulmonary hypertension versus fibrosing mediastinitis. 61
30694812 2019
43
Sclerosing Mediastinitis Causing Unilateral Pulmonary Edema Due to Left Atrial and Pulmonary Venous Compression. A Case Report and Literature Review. 61
30810679 2019
44
Fungal fibrosing mediastinitis in pregnancy - Case report with review of literature. 61
30693874 2019
45
Is Long-Term Anticoagulation Required after Stent Placement for Benign Superior Vena Cava Syndrome? 61
30396845 2018
46
Chronic Periaortitis: an Update. 61
30397845 2018
47
Fibrosing Mediastinitis: a Rare Cause of Unilateral Absent Lung Perfusion on a V/Q Scan. 61
30344792 2018
48
Intraluminal Arterial Filling Defects Misdiagnosed as Pulmonary Emboli: What Else Could They Be? 61
30122175 2018
49
Mediastinal and retroperitoneal fibrosis as a manifestation of breast cancer metastasis: A case report and literature review. 61
30095661 2018
50
A case of sarcoidosis-associated pulmonary hypertension masquerading as chronic thromboembolic pulmonary hypertension. 61
29537341 2018

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
20 GARD
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
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
53 NINDS
54 Novoseek
56 OMIM via Orphanet
57 OMIM® (Updated 05-Apr-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 Tocris
70 UMLS
71 UMLS via Orphanet
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