MCID: RTR011
MIFTS: 42

Retroperitoneal Fibrosis

Categories: 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 fibrosclerosis, multifocal. An important gene associated with Retroperitoneal Fibrosis is MDM2 (MDM2 Proto-Oncogene), and among its related pathways/superpathways is 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 : 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 179)
# Related Disease Score Top Affiliating Genes
1 liposarcoma 28.8 MDM2 VIM
2 fibrosclerosis, multifocal 11.8
3 igg4-related disease 11.4
4 desmoid tumor 11.0
5 igg4-related dacryoadenitis and sialadenitis 11.0
6 extraosseous osteosarcoma 10.3 MDM2 PTPRC
7 conventional fibrosarcoma 10.3 MDM2 PTPRC
8 pancreatitis 10.3
9 thyroiditis 10.2
10 aneurysm 10.2
11 histiocytoma 10.2 MDM2 PTPRC
12 ureteral obstruction 10.2
13 aortic aneurysm 10.1
14 autoimmune pancreatitis 10.1
15 cholangitis 10.1
16 lymphoma 10.0
17 mediastinitis 10.0
18 sclerosing cholangitis 10.0
19 aortic aneurysm, familial abdominal, 1 10.0
20 duodenitis 10.0
21 spindle cell lipoma 10.0 MDM2 VIM
22 pleomorphic liposarcoma 10.0 MDM2 VIM
23 anaplastic ependymoma 9.9 MDM2 VIM
24 thrombosis 9.9
25 sialadenitis 9.9
26 spondyloarthropathy 1 9.9
27 aortitis 9.9
28 glomerulonephritis 9.9
29 hydronephrosis 9.9
30 spondylitis 9.9
31 spindle cell sarcoma 9.9 MDM2 VIM
32 arthritis 9.9
33 back pain 9.9
34 fibrosing mediastinitis 9.9
35 renal cell carcinoma, nonpapillary 9.9
36 duodenal obstruction 9.9
37 embryonal sarcoma 9.9 MDM2 VIM
38 hemangiopericytoma, malignant 9.9 MDM2 VIM
39 gastric cancer 9.8
40 primary biliary cholangitis 9.8
41 membranous nephropathy 9.8
42 actinomycosis 9.8
43 immune-complex glomerulonephritis 9.8
44 carcinoid syndrome 9.8
45 biliary cirrhosis, primary, 1 9.8
46 lupus erythematosus 9.8
47 systemic lupus erythematosus 9.8
48 prostatitis 9.8
49 rheumatoid arthritis 9.8
50 primary biliary cirrhosis 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:

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 59)
# 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 Anti-Inflammatory Agents Phase 4,Phase 3,Phase 2
11 Antirheumatic Agents Phase 4,Phase 3,Phase 1,Phase 2
12 Antimetabolites Phase 4,Phase 3
13 Antineoplastic Agents, Hormonal Phase 4,Phase 3,Phase 2
14 Atorvastatin Calcium Phase 4 134523-03-8
15 Hydroxymethylglutaryl-CoA Reductase Inhibitors Phase 4
16 Hypolipidemic Agents Phase 4
17 Immunoglobulin G Phase 4,Phase 1,Phase 2
18 Antibodies Phase 4,Phase 1,Phase 2
19 Immunoglobulins Phase 4,Phase 1,Phase 2
20 Anticholesteremic Agents Phase 4
21 Lipid Regulating Agents Phase 4
22 Calcium, Dietary Phase 4
23 Prednisolone acetate Phase 4
24 Methylprednisolone acetate Phase 4
25 Methylprednisolone Hemisuccinate Phase 4
26 Prednisolone hemisuccinate Phase 4
27 Prednisolone phosphate Phase 4
28 Liver Extracts Phase 4
29
Methotrexate Approved Phase 3 1959-05-2, 59-05-2 126941
30
leucovorin Approved, Nutraceutical Phase 3 58-05-9 143 6006
31
Folic Acid Approved, Nutraceutical, Vet_approved Phase 3 59-30-3 6037
32 Folic Acid Antagonists Phase 3
33 Dermatologic Agents Phase 3
34 Nucleic Acid Synthesis Inhibitors Phase 3
35 Vitamin B Complex Phase 3
36 Antimetabolites, Antineoplastic Phase 3
37 Folate Nutraceutical Phase 3
38 Vitamin B9 Nutraceutical Phase 3
39
Tamoxifen Approved Phase 2 10540-29-1 2733526
40
rituximab Approved Phase 1, Phase 2 174722-31-7 10201696
41 Selective Estrogen Receptor Modulators Phase 2
42 Estrogen Antagonists Phase 2
43 Estrogen Receptor Modulators Phase 2
44 Estrogens Phase 2
45 Bone Density Conservation Agents Phase 2
46
Lenalidomide Approved Phase 1 191732-72-6 216326
47 Angiogenesis Inhibitors Phase 1
48 Angiogenesis Modulating Agents Phase 1
49 Anti-Bacterial Agents Phase 1
50 Anti-Infective 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, Liver, Bone, Pancreas, Prostate, Skin

Publications for Retroperitoneal Fibrosis

Articles related to Retroperitoneal Fibrosis:

(show top 50) (show all 617)
# 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
[<sup>18</sup>F]-Fluorodeoxyglucose Positron Emission Tomography and Response to Therapy in Idiopathic Retroperitoneal Fibrosis. ( 29033102 )
2018
15
A case of IgG4-related retroperitoneal fibrosis with multiple involvement. ( 28886329 )
2017
16
Retroperitoneal fibrosis and constrictive pericarditis-IgG4 related diseases: A case report. ( 28588687 )
2017
17
Retroperitoneal fibrosis associated with immunoglobulin g4-related disease in the differential diagnosis in retroperitoneal tumors. Case report. ( 28734747 )
2017
18
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
19
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
20
Idiopathic retroperitoneal fibrosis and its overlap with IgG4-related disease. ( 28070877 )
2017
21
Retroperitoneal Fibrosis as a Cause of Chylous Ascites. ( 29090239 )
2017
22
Contemporary role of ureterolysis in retroperitoneal fibrosis: treatment of last resort or first intent? An analysis of 50 cases. ( 28502080 )
2017
23
An Atypical Presentation of Retroperitoneal Fibrosis. ( 28878601 )
2017
24
Idiopathic retroperitoneal fibrosis: clinical features and long-term renal function outcome. ( 28501912 )
2017
25
Retroperitoneal fibrosis following a long-standing etanercept treatment in a spondyloarthritis patient. ( 28600212 )
2017
26
Idiopathic retroperitoneal fibrosis causing unilateral ureteral and sigmoid colon obstruction: A case report. ( 28207528 )
2017
27
Jaccoud's arthropathy, an unusual manifestation of idiopathic retroperitoneal fibrosis: rapid improvement of symptoms after tocilizumab treatment. ( 28776364 )
2017
28
IgG4 Isolated Retroperitoneal Fibrosis and Aneurysmal Periaortitis. ( 28882661 )
2017
29
Pathologically Proven Spontaneous Remission of IgG4-related Retroperitoneal Fibrosis. ( 28717084 )
2017
30
Idiopathic retroperitoneal fibrosis: a cross-sectional study of 142 Chinese patients. ( 29219671 )
2017
31
Retroperitoneal fibrosis - a report of five cases. ( 28769138 )
2017
32
A rare cause of acute post renal failure: Retroperitoneal fibrosis. ( 29473379 )
2017
33
Ultrasonography for Preoperative Diagnosis of Retroperitoneal Fibrosis. ( 28538448 )
2017
34
Correction: Idiopathic Retroperitoneal Fibrosis With Myofascial Pain Syndrome: A Case Report. ( 29201835 )
2017
35
Chronic periaortitis with retroperitoneal fibrosis successfully treated with first line tocilizumab monotherapy: a case report. ( 28466807 )
2017
36
Impact of hybrid molecular imaging in retroperitoneal fibrosis: a systematic review. ( 28840309 )
2017
37
Retroperitoneal fibrosis-clinical presentation and outcome analysis from urological perspective. ( 28868510 )
2017
38
Differentiation of Lymphoma Presenting as Retroperitoneal Mass and Retroperitoneal Fibrosis: Evaluation with Multidetector-row Computed Tomography. ( 28303852 )
2017
39
A case of retroperitoneal fibrosis responding to steroid therapy. ( 28379669 )
2017
40
Retroperitoneal Fibrosis. ( 28459474 )
2017
41
Idiopathic Retroperitoneal Fibrosis. ( 26860343 )
2016
42
Hypertensive emergency preceding the progression of periaortitis and retroperitoneal fibrosis: case report and review of the literature. ( 27112324 )
2016
43
Presentation of idiopathic retroperitoneal fibrosis at a young age: A rare case report. ( 27904827 )
2016
44
Laparoscopic right hemicolectomy for a patient with idiopathic retroperitoneal fibrosis: A case report. ( 27245370 )
2016
45
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
46
IgG4-related retroperitoneal fibrosis: A case report and review of literature. ( 26819174 )
2016
47
Chronic Tubulo-interstitial Pyelonephritis with Retroperitoneal Fibrosis. ( 27739273 )
2016
48
Retroperitoneal Metastasis from Lung Adenocarcinoma Mimics Retroperitoneal Fibrosis. ( 26718879 )
2016
49
Retroperitoneal fibrosis: case series of five patients and review of the literature. ( 27267521 )
2016
50
Retroperitoneal fibrosis with periaortitis: A case report of an unusual form of presentation of granulomatosis with polyangiitis. ( 27672552 )
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 10.42 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
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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