MCID: RTR011
MIFTS: 44

Retroperitoneal Fibrosis

Categories: Nephrological diseases, Rare diseases

Aliases & Classifications for Retroperitoneal Fibrosis

MalaCards integrated aliases for Retroperitoneal Fibrosis:

Name: Retroperitoneal Fibrosis 53 25 73
Idiopathic Retroperitoneal Fibrosis 53 73
Ormond's Disease 53 25
Ormond Disease 53 25
Igg4-Related Retroperitoneal Fibrosis 53

Classifications:



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Summaries for Retroperitoneal Fibrosis

NIH Rare Diseases : 53 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. 

MalaCards based summary : Retroperitoneal Fibrosis, also known as idiopathic retroperitoneal fibrosis, is related to liposarcoma and reticulum cell sarcoma. An important gene associated with Retroperitoneal Fibrosis is CCL11 (C-C Motif Chemokine Ligand 11), and among its related pathways/superpathways are Lung fibrosis and G-protein signaling_Rap2B regulation pathway. The drugs Prednisone and Prednisolone have been mentioned in the context of this disorder. Affiliated tissues include kidney, thyroid and cervix, 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 : 76 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 199)
# Related Disease Score Top Affiliating Genes
1 liposarcoma 29.7 MDM2 VIM
2 reticulum cell sarcoma 29.6 PTPRC VIM
3 fibrosclerosis, multifocal 12.0
4 igg4-related disease 11.6
5 mikulicz disease 11.2
6 desmoid tumor 11.2
7 pancreatitis 10.4
8 thyroiditis 10.4
9 aortic aneurysm 10.3
10 ureteral obstruction 10.3
11 autoimmune pancreatitis 10.3
12 aortic aneurysm, familial abdominal, 1 10.2
13 cholangitis 10.2
14 lymphoma 10.2
15 sclerosing cholangitis 10.2
16 hydronephrosis 10.2
17 spondyloarthropathy 1 10.1
18 spondylitis 10.1
19 systemic lupus erythematosus 10.1
20 thrombosis 10.1
21 lupus erythematosus 10.1
22 smooth muscle tumor 10.1 CCL11 VIM
23 spindle cell lipoma 10.1 MDM2 VIM
24 pleomorphic liposarcoma 10.1 MDM2 VIM
25 glomerulonephritis 10.1
26 sialadenitis 10.1
27 portal hypertension 10.1
28 aortitis 10.1
29 polyarteritis nodosa 10.1
30 anaplastic ependymoma 10.1 MDM2 VIM
31 meningioma, radiation-induced 10.1 CADM1 VIM
32 spindle cell sarcoma 10.1 MDM2 VIM
33 embryonal sarcoma 10.0 MDM2 VIM
34 primitive neuroectodermal tumor of the cervix uteri 10.0 PTPRC VIM
35 multicentric reticulohistiocytosis 10.0 PTPRC VIM
36 hemangiopericytoma, malignant 10.0 MDM2 VIM
37 extraskeletal ewing sarcoma 10.0 PTPRC VIM
38 biliary cirrhosis, primary, 1 10.0
39 renal cell carcinoma, nonpapillary 10.0
40 polyarteritis nodosa, childhood-onset 10.0
41 arthritis 10.0
42 primary biliary cirrhosis 10.0
43 duodenal obstruction 10.0
44 vasculitis 10.0
45 carcinoid syndrome 10.0
46 fibrosing mediastinitis 10.0
47 primary biliary cholangitis 10.0
48 mucinous cystadenocarcinoma 10.0 PTPRC VIM
49 conventional fibrosarcoma 10.0 MDM2 PTPRC
50 dendritic cell tumor 10.0 PTPRC VIM

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:

46
# 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 65)
# 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
Methylprednisolone Approved, Vet_approved Phase 4 83-43-2 6741
4
Methylprednisolone hemisuccinate Approved Phase 4 2921-57-5
5
Prednisolone phosphate Approved, Vet_approved Phase 4 302-25-0
6
Leflunomide Approved, Investigational Phase 4 75706-12-6 3899
7
Prednisolone hemisuccinate Experimental Phase 4 2920-86-7
8 Antimetabolites Phase 4,Phase 3
9 Immunosuppressive Agents Phase 4,Phase 3,Phase 1
10 Immunologic Factors Phase 4,Phase 3,Phase 1,Phase 2
11 Antineoplastic Agents, Hormonal Phase 4,Phase 3,Phase 2
12 Hormone Antagonists Phase 4,Phase 3,Phase 2
13 Antirheumatic Agents Phase 4,Phase 3,Phase 1,Phase 2
14 Hormones Phase 4,Phase 3,Phase 2
15 Anti-Inflammatory Agents Phase 4,Phase 3,Phase 2
16 glucocorticoids Phase 4,Phase 3,Phase 2
17 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 4,Phase 3,Phase 2
18 Calcium, Dietary Phase 4
19 Atorvastatin Calcium Phase 4 134523-03-8
20 Anticholesteremic Agents Phase 4
21 Lipid Regulating Agents Phase 4
22 Hypolipidemic Agents Phase 4
23 Hydroxymethylglutaryl-CoA Reductase Inhibitors Phase 4
24 Immunoglobulins Phase 4,Phase 1,Phase 2
25 Immunoglobulin G Phase 4,Phase 1,Phase 2
26 Antibodies Phase 4,Phase 1,Phase 2
27 Methylprednisolone acetate Phase 4
28 Prednisolone acetate Phase 4
29 Liver Extracts Phase 4
30
Methotrexate Approved Phase 3 59-05-2, 1959-05-2 126941
31
leucovorin Approved Phase 3 58-05-9 6006 143
32
Folic Acid Approved, Nutraceutical, Vet_approved Phase 3 59-30-3 6037
33 Folic Acid Antagonists Phase 3
34 Vitamin B Complex Phase 3
35 Nucleic Acid Synthesis Inhibitors Phase 3
36 Dermatologic Agents Phase 3
37 Antimetabolites, Antineoplastic Phase 3
38 Folate Phase 3
39 Vitamin B9 Phase 3
40
Tamoxifen Approved Phase 2 10540-29-1 2733526
41
rituximab Approved Phase 1, Phase 2 174722-31-7 10201696
42 Bone Density Conservation Agents Phase 2
43 Estrogen Receptor Modulators Phase 2
44 Estrogen Receptor Antagonists Phase 2
45 Selective Estrogen Receptor Modulators Phase 2
46 Estrogen Antagonists Phase 2
47 Estrogens Phase 2
48
Lenalidomide Approved Phase 1 191732-72-6 216326
49 Anti-Infective Agents Phase 1,Not Applicable
50 Angiogenesis Modulating Agents Phase 1

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
9 Ureteral Stent Versus Percutaneous Nephrostomy in Acutely Obstructed Infected Kidney Not yet recruiting NCT03498794 Not Applicable

Search NIH Clinical Center for Retroperitoneal Fibrosis

Genetic Tests for Retroperitoneal Fibrosis

Anatomical Context for Retroperitoneal Fibrosis

MalaCards organs/tissues related to Retroperitoneal Fibrosis:

41
Kidney, Thyroid, Cervix, Lung, Prostate, Heart, B Cells

Publications for Retroperitoneal Fibrosis

Articles related to Retroperitoneal Fibrosis:

(show top 50) (show all 1042)
# Title Authors Year
1
Retroperitoneal Fibrosis and Immunoglobulin G4. ( 29555050 )
2018
2
Renal fungus ball in a patient with retroperitoneal fibrosis: Unique complication in a rare disease. ( 29430718 )
2018
3
IgG4-related retroperitoneal fibrosis overlapping with primary biliary cirrhosis and primary SjAPgren's syndrome: A case report. ( 29953016 )
2018
4
The value of<sup>18</sup>F-FDG PET/CT in the distinction between retroperitoneal fibrosis and its malignant mimics. ( 28958769 )
2018
5
Unilateral renal forniceal rupture - A rare presentation of retroperitoneal fibrosis. ( 29321975 )
2018
6
Sporadic Retroperitoneal Fibrosis: a Gentle Giant. ( 29563740 )
2018
7
Clinical features and prognostic factors of ten patients with renal failure caused by IgG4-related retroperitoneal fibrosis. ( 29416818 )
2018
8
Retroperitoneal fibrosis associated with IgG4-related disease diagnosed by prostate biopsy developed with acute post-renal renal failure: A case report. ( 29034178 )
2018
9
Retroperitoneal fibrosis as extramedullary hematopoiesis of a chronic myelomonocytic leukemia. ( 29368535 )
2018
10
Rituximab for retroperitoneal fibrosis due toA IgG4-related disease: A case report and literature review. ( 29845002 )
2018
11
Low-Back Pain Due to Idiopathic Retroperitoneal Fibrosis. ( 29102100 )
2018
12
No specific imaging pattern can help differentiate IgG4-related disease from idiopathic retroperitoneal fibrosis: 18 histologically proven cases. ( 29465374 )
2018
13
Retroperitoneal Fibrosis ( 29494016 )
2018
14
Autoimmune pancreatitis associated with retroperitoneal fibrosis mimicking cholangiocarcinoma. ( 30159151 )
2018
15
A Case of Retroperitoneal Fibrosis Diagnosed as IgG4-related Disease After 35 Years. ( 30333398 )
2018
16
IgG4-related kidney disease and retroperitoneal fibrosis: An update. ( 30499730 )
2018
17
[18F]-Fluorodeoxyglucose Positron Emission Tomography and Response to Therapy in Idiopathic Retroperitoneal Fibrosis. ( 29033102 )
2018
18
Renal allotransplantation in retroperitoneal fibrosis: technical aspects ( 30064187 )
2018
19
A large-scale genetic analysis reveals an autoimmune origin of idiopathic retroperitoneal fibrosis. ( 30081155 )
2018
20
Mediastinal and retroperitoneal fibrosis as a manifestation of breast cancer metastasis: A case report and literature review. ( 30095661 )
2018
21
Retroperitoneal fibrosis secondary to non-urology carcinomas: a clinical and outcome analysis of 97 cases. ( 30182211 )
2018
22
Idiopathic retroperitoneal fibrosis presenting as spastic paraparesis. ( 30233024 )
2018
23
Long-term outcomes and predictors of a large cohort of idiopathic retroperitoneal fibrosis patients: a retrospective study. ( 30270709 )
2018
24
Rituximab for idiopathic and IgG4-related retroperitoneal fibrosis. ( 30334947 )
2018
25
Comparison of two subsets of Chinese patients with retroperitoneal fibrosis in terms of IgG4 immunohistochemical staining. ( 30476270 )
2018
26
A case of IgG4-related retroperitoneal fibrosis with multiple involvement. ( 28886329 )
2017
27
Retroperitoneal fibrosis and constrictive pericarditis-IgG4 related diseases: A case report. ( 28588687 )
2017
28
Retroperitoneal fibrosis associated with immunoglobulin g4-related disease in the differential diagnosis in retroperitoneal tumors. Case report. ( 28734747 )
2017
29
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
30
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
31
Idiopathic retroperitoneal fibrosis and its overlap with IgG4-related disease. ( 28070877 )
2017
32
Retroperitoneal Fibrosis as a Cause of Chylous Ascites. ( 29090239 )
2017
33
Contemporary role of ureterolysis in retroperitoneal fibrosis: treatment of last resort or first intent? An analysis of 50 cases. ( 28502080 )
2017
34
An Atypical Presentation of Retroperitoneal Fibrosis. ( 28878601 )
2017
35
Idiopathic retroperitoneal fibrosis: clinical features and long-term renal function outcome. ( 28501912 )
2017
36
Retroperitoneal fibrosis following a long-standing etanercept treatment in a spondyloarthritis patient. ( 28600212 )
2017
37
Idiopathic retroperitoneal fibrosis causing unilateral ureteral and sigmoid colon obstruction: A case report. ( 28207528 )
2017
38
Jaccoud's arthropathy, an unusual manifestation of idiopathic retroperitoneal fibrosis: rapid improvement of symptoms after tocilizumab treatment. ( 28776364 )
2017
39
IgG4 Isolated Retroperitoneal Fibrosis and Aneurysmal Periaortitis. ( 28882661 )
2017
40
Pathologically Proven Spontaneous Remission of IgG4-related Retroperitoneal Fibrosis. ( 28717084 )
2017
41
Idiopathic retroperitoneal fibrosis: a cross-sectional study of 142 Chinese patients. ( 29219671 )
2017
42
Retroperitoneal fibrosis - a report of five cases. ( 28769138 )
2017
43
A rare cause of acute post renal failure: Retroperitoneal fibrosis. ( 29473379 )
2017
44
Ultrasonography for Preoperative Diagnosis of Retroperitoneal Fibrosis. ( 28538448 )
2017
45
Correction: Idiopathic Retroperitoneal Fibrosis With Myofascial Pain Syndrome: A Case Report. ( 29201835 )
2017
46
Chronic periaortitis with retroperitoneal fibrosis successfully treated with first line tocilizumab monotherapy: a case report. ( 28466807 )
2017
47
Impact of hybrid molecular imaging in retroperitoneal fibrosis: a systematic review. ( 28840309 )
2017
48
Retroperitoneal fibrosis-clinical presentation and outcome analysis from urological perspective. ( 28868510 )
2017
49
Differentiation of Lymphoma Presenting as Retroperitoneal Mass and Retroperitoneal Fibrosis: Evaluation with Multidetector-row Computed Tomography. ( 28303852 )
2017
50
Elevated serum IgG4 levels in diagnosis and treatment response in patients with idiopathic retroperitoneal fibrosis. ( 28105551 )
2017

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 10.42 CCL11 SPP1
2 10.03 CCL11 SPP1 VIM

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 viral process GO:0016032 9.33 CADM1 MDM2 VIM
2 cellular response to interferon-gamma GO:0071346 9.26 CCL11 VIM
3 negative regulation of neuron projection development GO:0010977 8.96 MDM2 VIM
4 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
17 ExPASy
19 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 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 SNOMED-CT via Orphanet
71 TGDB
72 Tocris
73 UMLS
74 UMLS via Orphanet
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