MCID: RTR011
MIFTS: 43

Retroperitoneal Fibrosis

Categories: Rare diseases

Aliases & Classifications for Retroperitoneal Fibrosis

MalaCards integrated aliases for Retroperitoneal Fibrosis:

Name: Retroperitoneal Fibrosis 49 24 69
Idiopathic Retroperitoneal Fibrosis 49 69
Ormond's Disease 49 24
Ormond Disease 49 24
Igg4-Related Retroperitoneal Fibrosis 49

Classifications:



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Summaries for Retroperitoneal Fibrosis

NIH Rare Diseases : 49 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 liposarcoma 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 Prednisolone 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 : 24 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.

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 185)
# Related Disease Score Top Affiliating Genes
1 liposarcoma 29.0 MDM2 VIM
2 fibrosclerosis, multifocal 11.8
3 igg4-related disease 11.4
4 igg4-related dacryoadenitis and sialadenitis 11.0
5 desmoid tumor 11.0
6 fibrosis of extraocular muscles, congenital, 1 11.0
7 pancreatitis 10.3
8 thyroiditis 10.2
9 extraosseous osteosarcoma 10.2 MDM2 PTPRC
10 conventional fibrosarcoma 10.2 MDM2 PTPRC
11 aneurysm 10.2
12 alpha/beta t-cell lymphopenia with gamma/delta t-cell expansion, severe cytomegalovirus infection, and autoimmunity 10.2
13 ureteral obstruction 10.2
14 aortic aneurysm 10.1
15 autoimmune pancreatitis 10.1
16 histiocytoma 10.1 MDM2 PTPRC
17 episodic pain syndrome, familial, 1 10.1
18 cholangitis 10.1
19 sclerosing cholangitis 10.1
20 lymphoma 10.1
21 mediastinitis 10.1
22 aortic aneurysm, familial abdominal, 1 10.0
23 duodenitis 10.0
24 spindle cell lipoma 10.0 MDM2 VIM
25 anaplastic ependymoma 10.0 MDM2 VIM
26 pleomorphic liposarcoma 10.0 MDM2 VIM
27 spindle cell sarcoma 10.0 MDM2 VIM
28 glomerulonephritis 10.0
29 complement hyperactivation, angiopathic thrombosis, and protein-losing enteropathy 10.0
30 aortitis 10.0
31 hydronephrosis 10.0
32 spondyloarthropathy 1 10.0
33 spondylitis 10.0
34 thrombosis 10.0
35 sialadenitis 10.0
36 inflammatory spondylopathy 10.0
37 embryonal sarcoma 9.9 MDM2 VIM
38 hemangiopericytoma, malignant 9.9 MDM2 VIM
39 duodenal obstruction 9.9
40 back pain 9.9
41 renal cell carcinoma, nonpapillary 9.9
42 primitive neuroectodermal tumor of the cervix uteri 9.9 PTPRC VIM
43 multicentric reticulohistiocytosis 9.9 PTPRC VIM
44 pleomorphic adenoma 9.9 MDM2 VIM
45 smooth muscle tumor 9.9 CCL11 VIM
46 reticulum cell sarcoma 9.9 PTPRC VIM
47 mucinous cystadenocarcinoma 9.8 PTPRC VIM
48 immune-complex glomerulonephritis 9.8
49 lupus erythematosus 9.8
50 gastric cancer 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:

43
# Description MGI Source Accession Score Top Affiliating Genes
1 hematopoietic system MP:0005397 9.63 CADM1 CCL11 MDM2 PTPRC SPP1 VIM
2 immune system MP:0005387 9.43 CADM1 CCL11 MDM2 PTPRC SPP1 VIM
3 respiratory system MP:0005388 8.92 CCL11 PTPRC SPP1 VIM

Drugs & Therapeutics for Retroperitoneal Fibrosis

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

(show top 50) (show all 58)
# 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
Prednisolone Approved, Vet_approved Phase 4 50-24-8 5755
3
Leflunomide Approved, Investigational Phase 4 75706-12-6 3899
4
Methylprednisolone Approved, Vet_approved Phase 4 83-43-2 6741
5 glucocorticoids Phase 4,Phase 3,Phase 2
6 Hormone Antagonists Phase 4,Phase 3,Phase 2
7 Hormones Phase 4,Phase 3,Phase 2
8 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 4,Phase 3,Phase 2
9 Immunosuppressive Agents Phase 4,Phase 3,Phase 1
10 Antimetabolites Phase 4,Phase 3
11 Anti-Inflammatory Agents Phase 4,Phase 3,Phase 2
12 Antineoplastic Agents, Hormonal Phase 4,Phase 3,Phase 2
13 Antirheumatic Agents Phase 4,Phase 3,Phase 1,Phase 2
14 Atorvastatin Calcium Phase 4 134523-03-8
15 Hydroxymethylglutaryl-CoA Reductase Inhibitors Phase 4
16 Anticholesteremic Agents Phase 4
17 Hypolipidemic Agents Phase 4
18 Immunoglobulins Phase 4,Phase 1,Phase 2
19 Lipid Regulating Agents Phase 4
20 Antibodies Phase 4,Phase 1,Phase 2
21 Calcium, Dietary Phase 4
22 Prednisolone acetate Phase 4
23 Methylprednisolone acetate Phase 4
24 Methylprednisolone Hemisuccinate Phase 4
25 Prednisolone hemisuccinate Phase 4
26 Prednisolone phosphate Phase 4
27 Liver Extracts Phase 4
28
Methotrexate Approved Phase 3 1959-05-2, 59-05-2 126941
29
leucovorin Approved, Nutraceutical Phase 3 58-05-9 143 6006
30
Folic Acid Approved, Nutraceutical, Vet_approved Phase 3 59-30-3 6037
31 Folic Acid Antagonists Phase 3
32 Nucleic Acid Synthesis Inhibitors Phase 3
33 Dermatologic Agents Phase 3
34 Vitamin B Complex Phase 3
35 Antimetabolites, Antineoplastic 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 Selective Estrogen Receptor Modulators Phase 2
41 Estrogen Antagonists Phase 2
42 Estrogen Receptor Modulators Phase 2
43 Estrogens Phase 2
44 Bone Density Conservation Agents 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
Dopamine Approved 51-61-6, 62-31-7 681

Interventional clinical trials:


# 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:

38
Kidney, Thyroid, Liver, Bone, Pancreas, Prostate, Cervix

Publications for Retroperitoneal Fibrosis

Articles related to Retroperitoneal Fibrosis:

(show top 50) (show all 608)
# Title Authors Year
1
Clinical features and prognostic factors of ten patients with renal failure caused by IgG4-related retroperitoneal fibrosis. ( 29416818 )
2018
2
Unilateral renal forniceal rupture - A rare presentation of retroperitoneal fibrosis. ( 29321975 )
2018
3
[<sup>18</sup>F]-Fluorodeoxyglucose Positron Emission Tomography and Response to Therapy in Idiopathic Retroperitoneal Fibrosis. ( 29033102 )
2018
4
Renal fungus ball in a patient with retroperitoneal fibrosis: Unique complication in a rare disease. ( 29430718 )
2018
5
Retroperitoneal fibrosis as extramedullary hematopoiesis of a chronic myelomonocytic leukemia. ( 29368535 )
2018
6
Retroperitoneal fibrosis associated with IgG4-related disease diagnosed by prostate biopsy developed with acute post-renal renal failure: A case report. ( 29034178 )
2018
7
The value of<sup>18</sup>F-FDG PET/CT in the distinction between retroperitoneal fibrosis and its malignant mimics. ( 28958769 )
2018
8
Low-Back Pain Due to Idiopathic Retroperitoneal Fibrosis. ( 29102100 )
2018
9
Idiopathic retroperitoneal fibrosis: a cross-sectional study of 142 Chinese patients. ( 29219671 )
2017
10
IgG4 Isolated Retroperitoneal Fibrosis and Aneurysmal Periaortitis. ( 28882661 )
2017
11
Idiopathic retroperitoneal fibrosis: clinical features and long-term renal function outcome. ( 28501912 )
2017
12
Correction: Idiopathic Retroperitoneal Fibrosis With Myofascial Pain Syndrome: A Case Report. ( 29201835 )
2017
13
A case of retroperitoneal fibrosis responding to steroid therapy. ( 28379669 )
2017
14
A case of IgG4-related retroperitoneal fibrosis with multiple involvement. ( 28886329 )
2017
15
Retroperitoneal Fibrosis. ( 28459474 )
2017
16
Ultrasonography for Preoperative Diagnosis of Retroperitoneal Fibrosis. ( 28538448 )
2017
17
Retroperitoneal fibrosis associated with immunoglobulin g4-related disease in the differential diagnosis in retroperitoneal tumors. Case report. ( 28734747 )
2017
18
Retroperitoneal fibrosis and constrictive pericarditis-IgG4 related diseases: A case report. ( 28588687 )
2017
19
Retroperitoneal Fibrosis as a Cause of Chylous Ascites. ( 29090239 )
2017
20
Chronic periaortitis with retroperitoneal fibrosis successfully treated with first line tocilizumab monotherapy: a case report. ( 28466807 )
2017
21
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
22
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
23
Pathologically Proven Spontaneous Remission of IgG4-related Retroperitoneal Fibrosis. ( 28717084 )
2017
24
Jaccoud's arthropathy, an unusual manifestation of idiopathic retroperitoneal fibrosis: rapid improvement of symptoms after tocilizumab treatment. ( 28776364 )
2017
25
Retroperitoneal fibrosis following a long-standing etanercept treatment in a spondyloarthritis patient. ( 28600212 )
2017
26
Contemporary role of ureterolysis in retroperitoneal fibrosis: treatment of last resort or first intent? An analysis of 50 cases. ( 28502080 )
2017
27
An Atypical Presentation of Retroperitoneal Fibrosis. ( 28878601 )
2017
28
Retroperitoneal fibrosis-clinical presentation and outcome analysis from urological perspective. ( 28868510 )
2017
29
Impact of hybrid molecular imaging in retroperitoneal fibrosis: a systematic review. ( 28840309 )
2017
30
Differentiation of Lymphoma Presenting as Retroperitoneal Mass and Retroperitoneal Fibrosis: Evaluation with Multidetector-row Computed Tomography. ( 28303852 )
2017
31
Idiopathic retroperitoneal fibrosis and its overlap with IgG4-related disease. ( 28070877 )
2017
32
Idiopathic retroperitoneal fibrosis causing unilateral ureteral and sigmoid colon obstruction: A case report. ( 28207528 )
2017
33
Retroperitoneal fibrosis - a report of five cases. ( 28769138 )
2017
34
Hypertensive emergency preceding the progression of periaortitis and retroperitoneal fibrosis: case report and review of the literature. ( 27112324 )
2016
35
Retroperitoneal fibrosis: case series of five patients and review of the literature. ( 27267521 )
2016
36
IgG4-related retroperitoneal fibrosis: a newly characterized disease. ( 27125330 )
2016
37
Chronic Tubulo-interstitial Pyelonephritis with Retroperitoneal Fibrosis. ( 27739273 )
2016
38
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
39
Retroperitoneal fibrosis: retrospective descriptive study on clinical features and management. ( 27822461 )
2016
40
IgG4-Related Kidney Disease and IgG4-Related Retroperitoneal Fibrosis. ( 27466797 )
2016
41
[(18)F]-Fluorodeoxyglucose Positron Emission Tomography in the Diagnosis, Treatment Stratification, and Monitoring of Patients with Retroperitoneal Fibrosis: A Prospective Clinical Study. ( 27876167 )
2016
42
IgG4-related retroperitoneal fibrosis: A case report and review of literature. ( 26819174 )
2016
43
Retroperitoneal Fibrosis Due to Opium Abuse: A Case Series and Literature Review. ( 27896238 )
2016
44
Retroperitoneal Metastasis from Lung Adenocarcinoma Mimics Retroperitoneal Fibrosis. ( 26718879 )
2016
45
Retroperitoneal fibrosis with periaortitis: A case report of an unusual form of presentation of granulomatosis with polyangiitis. ( 27672552 )
2016
46
Presentation of idiopathic retroperitoneal fibrosis at a young age: A rare case report. ( 27904827 )
2016
47
Staphylococcus aureus aortitis and retroperitoneal fibrosis: A case report and literature review. ( 27516965 )
2016
48
Idiopathic Retroperitoneal Fibrosis. ( 26860343 )
2016
49
Outcome in patients with idiopathic retroperitoneal fibrosis treated with corticosteroid or tamoxifen monotherapy. ( 26985367 )
2016
50
A Case of Idiopathic Retroperitoneal Fibrosis Associated With SjAPgren's Syndrome. ( 27753592 )
2016

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:

# 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:

# Name GO ID Score Top Affiliating Genes
1 cellular response to interferon-gamma GO:0071346 9.16 CCL11 VIM
2 negative regulation of neuron projection development GO:0010977 8.96 MDM2 VIM
3 response to steroid hormone GO:0048545 8.62 MDM2 SPP1

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

# 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
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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