MCID: RTR011
MIFTS: 38

Retroperitoneal Fibrosis

Categories: Rare diseases

Aliases & Classifications for Retroperitoneal Fibrosis

MalaCards integrated aliases for Retroperitoneal Fibrosis:

Name: Retroperitoneal Fibrosis 50 25 69
Idiopathic Retroperitoneal Fibrosis 50 69
Ormond's Disease 50 25
Ormond Disease 50 25
Igg4-Related Retroperitoneal Fibrosis 50

Classifications:



Summaries for Retroperitoneal Fibrosis

NIH Rare Diseases : 50 retroperitoneal fibrosis is a slowly progressive disorder in which the tubes that carry urine from the kidneys to the bladder (ureters) and other abdominal organs or vessels become blocked by a fibrous mass and inflammation in the back of the abdomen. the disorder may cause pain in the abdomen that worsens with time, pain or swelling of the legs, decreased urine output, and swelling of the scrotum in men.risk factors for retroperitoneal fibrosis include asbestos exposure, smoking, tumor, infection, trauma, radiotherapy, surgery, and use of certain drugs.treatment may include corticosteroids, tamoxifen, stents or surgery.  last updated: 6/20/2016

MalaCards based summary : Retroperitoneal Fibrosis, also known as idiopathic retroperitoneal fibrosis, is related to igg4-related disease and fibrosclerosis, multifocal. An important gene associated with Retroperitoneal Fibrosis is MDM2 (MDM2 Proto-Oncogene), and among its related pathways/superpathways are Mesenchymal Stem Cell Differentiation Pathways and Lineage-specific Markers and Lung fibrosis. The drugs Prednisone and Leflunomide have been mentioned in the context of this disorder. Affiliated tissues include kidney, thyroid and liver, and related phenotypes are hematopoietic system and immune system

Genetics Home Reference : 25 Retroperitoneal fibrosis is a disorder in which inflammation and extensive scar tissue (fibrosis) occur in the back of the abdominal cavity, behind (retro-) the membrane that surrounds the organs of the digestive system (the peritoneum). This area is known as the retroperitoneal space. Retroperitoneal fibrosis can occur at any age but appears most frequently between the ages of 40 and 60.

Wikipedia : 72 Retroperitoneal fibrosis or Ormond\'s disease is a disease featuring the proliferation of fibrous tissue... more...

Related Diseases for Retroperitoneal Fibrosis

Diseases related to Retroperitoneal Fibrosis via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 148)
id Related Disease Score Top Affiliating Genes
1 igg4-related disease 11.3
2 fibrosclerosis, multifocal 11.1
3 mikulicz disease 10.9
4 desmoid tumor 10.9
5 coronary aneurysm 10.3 MDM2 PTPRC
6 brain stem infarction 10.3 MDM2 PTPRC
7 endodermal sinus tumor 10.3 MDM2 PTPRC
8 pancreatitis 10.2
9 spindle cell sarcoma 10.2 MDM2 PTPRC
10 thyroiditis 10.1
11 aneurysm 10.1
12 ureteral obstruction 10.1
13 skin lipoma 10.0 MDM2 VIM
14 anaplastic oligodendroglioma 10.0 MDM2 VIM
15 aortic aneurysm 10.0
16 autoimmune pancreatitis 10.0
17 spindle cell liposarcoma 10.0 MDM2 VIM
18 cholangitis 10.0
19 coronary restenosis 10.0 MDM2 VIM
20 lymphoma 10.0
21 sclerosing cholangitis 10.0
22 mediastinitis 10.0
23 enamel hypoplasia cataract hydrocephaly 10.0 MDM2 VIM
24 abdominal aortic aneurysm 9.9
25 patent ductus arteriosus and bicuspid aortic valve with hand anomalies 9.9 MDM2 VIM
26 duodenitis 9.9
27 intellectual disability-polydactyly-uncombable hair syndrome 9.9 PTPRC VIM
28 cortical blindness 9.9 PTPRC VIM
29 large cell acanthoma 9.9 CCL11 VIM
30 atherosclerosis 9.9
31 glomerulonephritis 9.9
32 thrombosis 9.9
33 sialadenitis 9.9
34 hydronephrosis 9.9
35 aortitis 9.9
36 spondylitis 9.9
37 short-rib thoracic dysplasia 12 9.9 PTPRC VIM
38 superior vena cava angiosarcoma 9.9 MDM2 VIM
39 ovarian mucinous adenocarcinoma 9.9 PTPRC VIM
40 spondyloepimetaphyseal dysplasia, isidor type 9.8 PTPRC VIM
41 renal cell carcinoma 9.8
42 duodenal obstruction 9.8
43 dense deposit disease 9.8 PTPRC VIM
44 mixed extragonadal germ cell cancer 9.8 MDM2 VIM
45 adenosarcoma 9.8 MDM2 VIM
46 dextrocardia with situs inversus 9.8 PTPRC VIM
47 rheumatoid arthritis 9.8
48 systemic lupus erythematosus 9.8
49 membranous nephropathy 9.8
50 arthritis 9.8

Graphical network of the top 20 diseases related to Retroperitoneal Fibrosis:



Diseases related to Retroperitoneal Fibrosis

Symptoms & Phenotypes for Retroperitoneal Fibrosis

MGI Mouse Phenotypes related to Retroperitoneal Fibrosis:

44
id Description MGI Source Accession Score Top Affiliating Genes
1 hematopoietic system MP:0005397 9.63 VIM CADM1 CCL11 MDM2 PTPRC SPP1
2 immune system MP:0005387 9.43 VIM CADM1 CCL11 MDM2 PTPRC SPP1
3 respiratory system MP:0005388 8.92 VIM CCL11 PTPRC SPP1

Drugs & Therapeutics for Retroperitoneal Fibrosis

Drugs for Retroperitoneal Fibrosis (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show top 50) (show all 58)
id Name Status Phase Clinical Trials Cas Number PubChem Id
1
Prednisone Approved, Vet_approved Phase 4,Phase 3,Phase 2 53-03-2 5865
2
Leflunomide Approved, Investigational Phase 4 75706-12-6 3899
3
Methylprednisolone Approved, Vet_approved Phase 4 83-43-2 6741
4
Prednisolone Approved, Vet_approved Phase 4 50-24-8 5755
5 Antimetabolites Phase 4,Phase 3
6 Anti-Inflammatory Agents Phase 4,Phase 3,Phase 2
7 Antineoplastic Agents, Hormonal Phase 4,Phase 3,Phase 2
8 Antirheumatic Agents Phase 4,Phase 3,Phase 1,Phase 2
9 glucocorticoids Phase 4,Phase 3,Phase 2
10 Hormone Antagonists Phase 4,Phase 3,Phase 2
11 Hormones Phase 4,Phase 3,Phase 2
12 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 4,Phase 3,Phase 2
13 Immunosuppressive Agents Phase 4,Phase 3,Phase 1
14 Anticholesteremic Agents Phase 4
15 Atorvastatin Calcium Phase 4 134523-03-8
16 Calcium, Dietary Phase 4
17 Hydroxymethylglutaryl-CoA Reductase Inhibitors Phase 4
18 Hypolipidemic Agents Phase 4
19 Lipid Regulating Agents Phase 4
20 Antibodies Phase 4,Phase 1,Phase 2
21 Immunoglobulins Phase 4,Phase 1,Phase 2
22 Liver Extracts Phase 4
23 Methylprednisolone acetate Phase 4
24 Methylprednisolone Hemisuccinate Phase 4
25 Prednisolone acetate Phase 4
26 Prednisolone hemisuccinate Phase 4
27 Prednisolone phosphate Phase 4
28
Methotrexate Approved Phase 3 1959-05-2, 59-05-2 126941
29
Folic Acid Approved, Nutraceutical, Vet_approved Phase 3 59-30-3 6037
30
leucovorin Approved, Nutraceutical Phase 3 58-05-9 143 6006
31 Antimetabolites, Antineoplastic Phase 3
32 Dermatologic Agents Phase 3
33 Folic Acid Antagonists Phase 3
34 Nucleic Acid Synthesis Inhibitors Phase 3
35 Vitamin B Complex Phase 3
36 Folate Nutraceutical Phase 3
37 Vitamin B9 Nutraceutical Phase 3
38
Tamoxifen Approved Phase 2 10540-29-1 2733526
39
rituximab Approved Phase 1, Phase 2 174722-31-7 10201696
40 Bone Density Conservation Agents Phase 2
41 Estrogen Antagonists Phase 2
42 Estrogen Receptor Modulators Phase 2
43 Estrogens Phase 2
44 Selective Estrogen Receptor Modulators Phase 2
45
Lenalidomide Approved Phase 1 191732-72-6 216326
46 Angiogenesis Inhibitors Phase 1
47 Angiogenesis Modulating Agents Phase 1
48 Anti-Bacterial Agents Phase 1
49 Anti-Infective Agents Phase 1
50
Cabergoline Approved 81409-90-7 54746

Interventional clinical trials:


id Name Status NCT ID Phase Drugs
1 Atorvastatin as an Adjunct to DFDBA in Intrabony Defects Completed NCT03009097 Phase 4 DFDBA with atorvastatin
2 Leflunomide for Maintenance of Remission in IgG4 Related Disease Recruiting NCT02703194 Phase 4 Prednisone;Leflunomide
3 Methotrexate as a Steroid-sparing Agent in Idiopathic Retroperitoneal Fibrosis: a Randomised, Multicenter Trial Unknown status NCT01240850 Phase 3 Methotrexate+Prednisone;Prednisone
4 Prednisone Versus Tamoxifen in Idiopathic Retroperitoneal Fibrosis Unknown status NCT00440349 Phase 2 Tamoxifen;Prednisone
5 Rituximab in IgG4-RD: A Phase 1-2 Trial Completed NCT01584388 Phase 1, Phase 2 Rituximab
6 Treatment of IgG4-Related Disease With Revlimid and Rituximab Recruiting NCT02705638 Phase 1 Rituximab;Lenalidomide
7 The Association Between Dopamine Agonists and Cardiac Valvulopathy, Fibrosis and Other Cardiopulmonary Events Completed NCT01052948
8 Aortitis and Retroperitoneal Fibrosis: Evaluation of Diagnostic Criteria Recruiting NCT03133949

Search NIH Clinical Center for Retroperitoneal Fibrosis

Genetic Tests for Retroperitoneal Fibrosis

Anatomical Context for Retroperitoneal Fibrosis

MalaCards organs/tissues related to Retroperitoneal Fibrosis:

39
Kidney, Thyroid, Liver, Bone, Pancreas, Prostate, Cervix

Publications for Retroperitoneal Fibrosis

Articles related to Retroperitoneal Fibrosis:

(show top 50) (show all 597)
id Title Authors Year
1
Pathologically Proven Spontaneous Remission of IgG4-related Retroperitoneal Fibrosis. ( 28717084 )
2017
2
Ultrasonography for Preoperative Diagnosis of Retroperitoneal Fibrosis. ( 28538448 )
2017
3
Contemporary role of ureterolysis in retroperitoneal fibrosis: treatment of last resort or first intent? An analysis of 50 cases. ( 28502080 )
2017
4
Idiopathic retroperitoneal fibrosis: clinical features and long-term renal function outcome. ( 28501912 )
2017
5
A case of retroperitoneal fibrosis responding to steroid therapy. ( 28379669 )
2017
6
Retroperitoneal fibrosis - a report of five cases. ( 28769138 )
2017
7
Chronic periaortitis with retroperitoneal fibrosis successfully treated with first line tocilizumab monotherapy: a case report. ( 28466807 )
2017
8
Retroperitoneal fibrosis-clinical presentation and outcome analysis from urological perspective. ( 28868510 )
2017
9
Overlap of Post-obstructive Diuresis and Unmasked Diabetes Insipidus in a Case of IgG4-related Retroperitoneal Fibrosis and Tuberoinfundibular Hypophysitis: A Case Report and Review of the Literature. ( 28049999 )
2017
10
Idiopathic retroperitoneal fibrosis causing unilateral ureteral and sigmoid colon obstruction: A case report. ( 28207528 )
2017
11
Idiopathic retroperitoneal fibrosis and its overlap with IgG4-related disease. ( 28070877 )
2017
12
An Atypical Presentation of Retroperitoneal Fibrosis. ( 28878601 )
2017
13
Impact of hybrid molecular imaging in retroperitoneal fibrosis: a systematic review. ( 28840309 )
2017
14
Retroperitoneal Fibrosis. ( 28459474 )
2017
15
Retroperitoneal fibrosis and constrictive pericarditis-IgG4 related diseases: A case report. ( 28588687 )
2017
16
Retroperitoneal fibrosis associated with immunoglobulin g4-related disease in the differential diagnosis in retroperitoneal tumors. Case report. ( 28734747 )
2017
17
Jaccoud's arthropathy, an unusual manifestation of idiopathic retroperitoneal fibrosis: rapid improvement of symptoms after tocilizumab treatment. ( 28776364 )
2017
18
IgG4 Isolated Retroperitoneal Fibrosis and Aneurysmal Periaortitis. ( 28882661 )
2017
19
Differentiation of Lymphoma Presenting as Retroperitoneal Mass and Retroperitoneal Fibrosis: Evaluation with Multidetector-row Computed Tomography. ( 28303852 )
2017
20
A case of IgG4-related retroperitoneal fibrosis with multiple involvement. ( 28886329 )
2017
21
Re: Archie Fernando, James Pattison, Catherine Horsfield, David D'Cruz, Gary Cook, Tim O'Brien. [(18)F]-Fluorodeoxyglucose Positron Emission Tomography in the Diagnosis, Treatment Stratification, and Monitoring of Patients with Retroperitoneal Fibrosis: A Prospective Clinical Study. Eur Urol 2017;71:926-33. ( 28259475 )
2017
22
Retroperitoneal fibrosis following a long-standing etanercept treatment in a spondyloarthritis patient. ( 28600212 )
2017
23
IgG4-related retroperitoneal fibrosis: a newly characterized disease. ( 27125330 )
2016
24
Retroperitoneal fibrosis: case series of five patients and review of the literature. ( 27267521 )
2016
25
Retroperitoneal fibrosis with periaortitis: A case report of an unusual form of presentation of granulomatosis with polyangiitis. ( 27672552 )
2016
26
Idiopathic Retroperitoneal Fibrosis. ( 26860343 )
2016
27
Hypertensive emergency preceding the progression of periaortitis and retroperitoneal fibrosis: case report and review of the literature. ( 27112324 )
2016
28
Disease activity and vascular involvement in retroperitoneal fibrosis: first experience with fully integrated 18F-fluorodeoxyglucose positron emission tomography/magnetic resonance imaging compared to clinical and laboratory parameters. ( 27974103 )
2016
29
(18)F-FDG PET/MRI evaluation of retroperitoneal fibrosis: a simultaneous multiparametric approach for diagnosing active disease. ( 26969347 )
2016
30
Extrinsic compression of the biliary tract due to idiopathic retroperitoneal fibrosis: MR imaging findings. ( 27986422 )
2016
31
Diagnosis and follow-up of idiopathic retroperitoneal fibrosis: role of (18)F-FDG-PET/CT and biochemical parameters in patients with renal involvement. ( 26970988 )
2016
32
IgG4-related retroperitoneal fibrosis: A case report and review of literature. ( 26819174 )
2016
33
Presentation of idiopathic retroperitoneal fibrosis at a young age: A rare case report. ( 27904827 )
2016
34
Outcome in patients with idiopathic retroperitoneal fibrosis treated with corticosteroid or tamoxifen monotherapy. ( 26985367 )
2016
35
A Case of Idiopathic Retroperitoneal Fibrosis Associated With SjAPgren's Syndrome. ( 27753592 )
2016
36
Retroperitoneal Fibrosis Due to Opium Abuse: A Case Series and Literature Review. ( 27896238 )
2016
37
Retroperitoneal fibrosis - the state-of-the-art. ( 27994271 )
2016
38
Staphylococcus aureus aortitis and retroperitoneal fibrosis: A case report and literature review. ( 27516965 )
2016
39
Laparoscopic right hemicolectomy for a patient with idiopathic retroperitoneal fibrosis: A case report. ( 27245370 )
2016
40
[(18)F]-Fluorodeoxyglucose Positron Emission Tomography in the Diagnosis, Treatment Stratification, and Monitoring of Patients with Retroperitoneal Fibrosis: A Prospective Clinical Study. ( 27876167 )
2016
41
Chronic Tubulo-interstitial Pyelonephritis with Retroperitoneal Fibrosis. ( 27739273 )
2016
42
Retroperitoneal Metastasis from Lung Adenocarcinoma Mimics Retroperitoneal Fibrosis. ( 26718879 )
2016
43
IgG4-Related Kidney Disease and IgG4-Related Retroperitoneal Fibrosis. ( 27466797 )
2016
44
Retroperitoneal fibrosis: retrospective descriptive study on clinical features and management. ( 27822461 )
2016
45
Computed tomography parameters can be used as predictive markers for the improvement of renal function in patients with retroperitoneal fibrosis. ( 26516692 )
2015
46
Retroperitoneal fibrosis: A retrospective review of clinical presentation, treatment and outcomes. ( 26178567 )
2015
47
Contrast-enhanced (18)F-fluorodeoxyglucose positron emission tomography/computed tomography in immunoglobulin G4-related retroperitoneal fibrosis. ( 25589813 )
2015
48
Extensive primary retroperitoneal fibrosis (Ormond's disease) with common bile duct and ureteral obstruction: A rare case report. ( 26074483 )
2015
49
Retroperitoneal Fibrosis: A Retrospective Clinical Data Analysis of 30 Patients in a 10-year Period. ( 25758277 )
2015
50
Idiopathic and secondary forms of retroperitoneal fibrosis: a diagnostic approach. ( 25455951 )
2015

Variations for Retroperitoneal Fibrosis

Expression for Retroperitoneal Fibrosis

Search GEO for disease gene expression data for Retroperitoneal Fibrosis.

Pathways for Retroperitoneal Fibrosis

Pathways related to Retroperitoneal Fibrosis according to GeneCards Suite gene sharing:

id Super pathways Score Top Affiliating Genes
1 10.94 PTPRC SPP1
2 10.42 CCL11 SPP1

GO Terms for Retroperitoneal Fibrosis

Biological processes related to Retroperitoneal Fibrosis according to GeneCards Suite gene sharing:

id Name GO ID Score Top Affiliating Genes
1 negative regulation of neuron projection development GO:0010977 8.96 MDM2 VIM
2 response to steroid hormone GO:0048545 8.62 MDM2 SPP1

Molecular functions related to Retroperitoneal Fibrosis according to GeneCards Suite gene sharing:

id Name GO ID Score Top Affiliating Genes
1 scaffold protein binding GO:0097110 8.62 MDM2 VIM

Sources for Retroperitoneal Fibrosis

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
16 ExPASy
18 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 MedGen
42 MeSH
43 MESH via Orphanet
44 MGI
46 NCI
47 NCIt
48 NDF-RT
51 NINDS
52 Novoseek
54 OMIM
55 OMIM via Orphanet
59 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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