MCID: RTR011
MIFTS: 47

Retroperitoneal Fibrosis

Categories: Blood diseases, Gastrointestinal diseases, Nephrological diseases, Rare diseases

Aliases & Classifications for Retroperitoneal Fibrosis

MalaCards integrated aliases for Retroperitoneal Fibrosis:

Name: Retroperitoneal Fibrosis 20 43 70
Idiopathic Retroperitoneal Fibrosis 20 70
Ormond's Disease 20 43
Ormond Disease 20 43
Igg4-Related Retroperitoneal Fibrosis 20

Classifications:



Summaries for Retroperitoneal Fibrosis

MedlinePlus Genetics : 43 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.The inflamed tissue characteristic of retroperitoneal fibrosis typically causes gradually increasing pain in the lower abdomen, back, or side. Other symptoms arise from blockage of blood flow to and from various parts of the lower body, due to the development of scar tissue around blood vessels. The fibrosis usually develops first around the aorta, which is the large blood vessel that distributes blood from the heart to the rest of the body. Additional blood vessels including the inferior vena cava, which returns blood from the lower part of the body to the heart, may also be involved. Obstruction of blood flow to and from the legs can result in pain, changes in color, and swelling in these limbs. Impairment of blood flow in the intestines may lead to death (necrosis) of intestinal tissue, severe pain, and excessive bleeding (hemorrhage). In men, reduced blood flow back toward the heart (venous flow) may cause swelling of the scrotum.Because the kidneys are located in the retroperitoneal space, retroperitoneal fibrosis may result in blockage of the ureters, which are tubes that carry urine from each kidney to the bladder. Such blockages can lead to decreased or absent urine flow and kidney failure. When the kidneys fail, toxic substances build up in the blood and tissues, leading to nausea, vomiting, weight loss, itching, a low number of red blood cells (anemia), and changes in brain function.

MalaCards based summary : Retroperitoneal Fibrosis, also known as idiopathic retroperitoneal fibrosis, is related to sarcoidosis 1 and reticulum cell sarcoma. An important gene associated with Retroperitoneal Fibrosis is MDM2 (MDM2 Proto-Oncogene), and among its related pathways/superpathways are Cell adhesion molecules and Lung fibrosis. The drugs Atorvastatin and Methylprednisolone have been mentioned in the context of this disorder. Affiliated tissues include heart, kidney and pancreas, and related phenotypes are fatigue and abdominal pain

GARD : 20 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.

Wikipedia : 73 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 406)
# Related Disease Score Top Affiliating Genes
1 sarcoidosis 1 30.0 PTPRC HLA-DRB1 CCL11
2 reticulum cell sarcoma 30.0 VIM PTPRC
3 retroperitoneum carcinoma 29.8 PTPRC MDM2
4 churg-strauss syndrome 29.7 HLA-DRB1 CCL11
5 dilated cardiomyopathy 28.4 SPP1 PTPRC MDM2 HLA-DRB1
6 fibrosclerosis, multifocal 11.7
7 igg4-related disease 11.3
8 desmoid tumor 11.1
9 mikulicz disease 11.1
10 fibrosis of extraocular muscles, congenital, 1 11.0
11 hydronephrosis 10.9
12 ureteral obstruction 10.8
13 aortic aneurysm 10.6
14 urinary tract obstruction 10.6
15 acute kidney failure 10.6
16 thyroiditis 10.6
17 autoimmune pancreatitis 10.5
18 pancreatitis 10.5
19 aortic aneurysm, familial abdominal, 1 10.5
20 cholangitis 10.5
21 sclerosing cholangitis 10.5
22 vasculitis 10.5
23 fibrosing mediastinitis 10.5
24 igg4-related thyroid disease 10.5
25 kidney disease 10.4
26 back pain 10.4
27 autoimmune disease 10.4
28 aortitis 10.4
29 aneurysm 10.4
30 portal hypertension 10.3
31 obstructive jaundice 10.3
32 glomerulonephritis 10.3
33 anuria 10.3
34 connective tissue disease 10.3
35 diabetes insipidus 10.3
36 inflammatory spondylopathy 10.3
37 spondylitis 10.3
38 spondyloarthropathy 1 10.3
39 sialadenitis 10.3
40 obstructive nephropathy 10.3
41 duodenal obstruction 10.3
42 systemic lupus erythematosus 10.2
43 uremia 10.2
44 actinomycosis 10.2
45 hypereosinophilic syndrome 10.2
46 carcinoid syndrome 10.2
47 atherosclerosis susceptibility 10.2
48 lymphoma, hodgkin, classic 10.2
49 lymphoma, non-hodgkin, familial 10.2
50 membranous nephropathy 10.2

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



Diseases related to Retroperitoneal Fibrosis

Symptoms & Phenotypes for Retroperitoneal Fibrosis

Human phenotypes related to Retroperitoneal Fibrosis:

31 (show all 40)
# Description HPO Frequency HPO Source Accession
1 fatigue 31 frequent (33%) HP:0012378
2 abdominal pain 31 frequent (33%) HP:0002027
3 anorexia 31 frequent (33%) HP:0002039
4 hydronephrosis 31 frequent (33%) HP:0000126
5 weight loss 31 frequent (33%) HP:0001824
6 elevated c-reactive protein level 31 frequent (33%) HP:0011227
7 elevated erythrocyte sedimentation rate 31 frequent (33%) HP:0003565
8 large vessel vasculitis 31 frequent (33%) HP:0005310
9 normocytic anemia 31 frequent (33%) HP:0001897
10 unilateral renal hypoplasia 31 frequent (33%) HP:0012583
11 elevated serum creatinine 31 frequent (33%) HP:0003259
12 increased blood urea nitrogen 31 frequent (33%) HP:0003138
13 anti-thyroid peroxidase antibody positivity 31 frequent (33%) HP:0025379
14 low back pain 31 frequent (33%) HP:0003419
15 flank pain 31 frequent (33%) HP:0030157
16 deep dermal perivascular inflammatory infiltrate 31 frequent (33%) HP:0031191
17 nausea and vomiting 31 occasional (7.5%) HP:0002017
18 constipation 31 occasional (7.5%) HP:0002019
19 fever 31 occasional (7.5%) HP:0001945
20 hashimoto thyroiditis 31 occasional (7.5%) HP:0000872
21 ureteropelvic junction obstruction 31 occasional (7.5%) HP:0000074
22 pedal edema 31 occasional (7.5%) HP:0010741
23 psoriasiform dermatitis 31 occasional (7.5%) HP:0003765
24 acute kidney injury 31 occasional (7.5%) HP:0001919
25 rheumatoid arthritis 31 occasional (7.5%) HP:0001370
26 systemic lupus erythematosus 31 occasional (7.5%) HP:0002725
27 rheumatoid factor positive 31 occasional (7.5%) HP:0002923
28 smooth muscle antibody positivity 31 occasional (7.5%) HP:0003262
29 antineutrophil antibody positivity 31 occasional (7.5%) HP:0003453
30 membranous nephropathy 31 occasional (7.5%) HP:0012578
31 nephrotic syndrome 31 very rare (1%) HP:0000100
32 hematuria 31 very rare (1%) HP:0000790
33 renovascular hypertension 31 very rare (1%) HP:0100817
34 impotence 31 very rare (1%) HP:0000802
35 dysuria 31 very rare (1%) HP:0100518
36 budd-chiari syndrome 31 very rare (1%) HP:0002639
37 hydrocele testis 31 very rare (1%) HP:0000034
38 varicocele 31 very rare (1%) HP:0012871
39 retrograde ejaculation 31 very rare (1%) HP:0012877
40 renal tubular epithelial necrosis 31 very rare (1%) HP:0008682

MGI Mouse Phenotypes related to Retroperitoneal Fibrosis:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 normal MP:0002873 9.02 CADM1 CCL11 MDM2 PTPRC VIM

Drugs & Therapeutics for Retroperitoneal Fibrosis

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

(show top 50) (show all 53)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Atorvastatin Approved Phase 4 134523-00-5 60823
2
Methylprednisolone Approved, Vet_approved Phase 4 83-43-2 6741
3
Prednisolone Approved, Vet_approved Phase 4 50-24-8 5755
4
Prednisolone phosphate Approved, Vet_approved Phase 4 302-25-0
5
Methylprednisolone hemisuccinate Approved Phase 4 2921-57-5
6
Prednisolone acetate Approved, Vet_approved Phase 4 52-21-1
7
Prednisone Approved, Vet_approved Phase 4 53-03-2 5865
8
Cyclophosphamide Approved, Investigational Phase 4 50-18-0, 6055-19-2 2907
9
Prednisolone hemisuccinate Experimental Phase 4 2920-86-7
10 Antimetabolites Phase 4
11 Hydroxymethylglutaryl-CoA Reductase Inhibitors Phase 4
12 Lipid Regulating Agents Phase 4
13 Anticholesteremic Agents Phase 4
14 Hypolipidemic Agents Phase 4
15 Hormone Antagonists Phase 4
16 Antibodies Phase 4
17 glucocorticoids Phase 4
18 Hormones Phase 4
19 Methylprednisolone Acetate Phase 4
20 Immunoglobulins Phase 4
21 Antibodies, Monoclonal Phase 4
22 Anti-Inflammatory Agents Phase 4
23 Antirheumatic Agents Phase 4
24 Immunologic Factors Phase 4
25 Immunosuppressive Agents Phase 4
26 Alkylating Agents Phase 4
27
Methotrexate Approved Phase 3 1959-05-2, 59-05-2 126941
28
Levoleucovorin Approved, Investigational Phase 3 68538-85-2 149436
29
Clotrimazole Approved, Vet_approved Phase 2, Phase 3 23593-75-1 2812
30
tannic acid Approved Phase 2, Phase 3 1401-55-4
31
Miconazole Approved, Investigational, Vet_approved Phase 2, Phase 3 22916-47-8 4189
32
Sirolimus Approved, Investigational Phase 2, Phase 3 53123-88-9 6436030 5284616
33
Benzocaine Approved, Investigational Phase 2, Phase 3 1994-09-7, 94-09-7 2337
34
Folic acid Approved, Nutraceutical, Vet_approved Phase 3 59-30-3 6037
35 Folic Acid Antagonists Phase 3
36 Vitamin B9 Phase 3
37 Vitamin B Complex Phase 3
38 Dermatologic Agents Phase 3
39 Folate Phase 3
40 Antineoplastic Agents, Hormonal Phase 2, Phase 3
41 Antibiotics, Antitubercular Phase 2, Phase 3
42 Anti-Bacterial Agents Phase 2, Phase 3
43 Anti-Infective Agents Phase 2, Phase 3
44 Antifungal Agents Phase 2, Phase 3
45
Tamoxifen Approved Phase 2 10540-29-1 2733526
46
rituximab Approved Phase 1, Phase 2 174722-31-7 10201696
47 Estrogens Phase 2
48 Estrogen Receptor Antagonists Phase 2
49 Estrogen Antagonists Phase 2
50 Estrogen Receptor Modulators Phase 2

Interventional clinical trials:

(show all 11)
# Name Status NCT ID Phase Drugs
1 "Comparative Evaluation Of The Efficacy Of Atorvastatin Gel As An Adjunct To Allografts In The Treatment Of Intrabony Defects" - A Clinico Radiological Study Completed NCT03009097 Phase 4 DFDBA with atorvastatin
2 A Prospective, Controlled, Interventional Study to Evaluate the Efficacy and Safety of Tocilizumab in the Treatment of Idiopathic Retroperitoneal Fibrosis Recruiting NCT04762784 Phase 4 Tocilizumab;Glucocorticoids
3 A Prospective Study to Evaluate the Efficacy and Safety of Cyclophosphamide in the Treatment of Idiopathic Retroperitoneal Fibrosis Recruiting NCT04762810 Phase 4 Cyclophosphamide and glucocorticoids;Glucocorticoids
4 Medical Therapy in Idiopathic Retroperitoneal Fibrosis: a Multicenter, Randomized, Controlled Trial of Prednisone vs Low-dose Prednisone Plus Methotrexate Unknown status NCT01240850 Phase 3 Methotrexate+Prednisone;Prednisone
5 Prospective Study of Prednisone Versus Sirolimus in the Treatment of Idiopathic Retroperitoneal Fibrosis Recruiting NCT04047576 Phase 2, Phase 3 Sirolimus;Corticosteroid
6 A Randomised Trial of Prednisone and Tamoxifen in Patients With Idiopathic Retroperitoneal Fibrosis Unknown status NCT00440349 Phase 2 Tamoxifen;Prednisone
7 Rituximab (RTX) for IgG4-related Disease (IgG4-RD): a Prospective,Open-label Trial Completed NCT01584388 Phase 1, Phase 2 Rituximab
8 Treatment of IgG4-Related Disease With Revlimid and Rituximab: The TIGR2 Trial Completed NCT02705638 Phase 1 Rituximab;Lenalidomide
9 Aortitis and Retroperitoneal Fibrosis: Evaluation of Diagnostic Criteria Unknown status NCT03133949
10 A Prospective Cohort Study of Idiopathic Retroperitoneal Fibrosis in China Recruiting NCT04312854
11 National Registry of Idiopathic Retroperitoneal Fibrosis in China Recruiting NCT04314323

Search NIH Clinical Center for Retroperitoneal Fibrosis

Genetic Tests for Retroperitoneal Fibrosis

Anatomical Context for Retroperitoneal Fibrosis

MalaCards organs/tissues related to Retroperitoneal Fibrosis:

40
Heart, Kidney, Pancreas, Colon, Testis, Smooth Muscle, Thyroid

Publications for Retroperitoneal Fibrosis

Articles related to Retroperitoneal Fibrosis:

(show top 50) (show all 2440)
# Title Authors PMID Year
1
Retroperitoneal fibrosis. 20 61
25161213 2014
2
Retroperitoneal fibrosis. 61 20
16427494 2006
3
[The 487th case: prominent eyes, headache, blurred vision]. 61
33765713 2021
4
The Association of Smoking with IgG4-Related Disease: A Case-Control Study. 61
33751033 2021
5
Clinical features and relapse risks of IgG4-related ophthalmic disease: a single-center experience in China. 61
33789746 2021
6
Oncology and complications. 61
33754613 2021
7
IgG4-related retroperitoneal fibrosis - clinical condition commonly overlooked in daily clinical practice. 61
33769001 2021
8
Characteristics of Adult Patients with Idiopathic Retroperitoneal Fibrosis and Assessment of Risk of Relapse at Diagnosis. 61
33808093 2021
9
Anti-PD-1 associated retroperitoneal fibrosis. 61
33729466 2021
10
A case of idiopathic retroperitoneal fibrosis presenting as gallbladder carcinoma. 61
33765983 2021
11
Including Medical Management in the Urologic Approach to Idiopathic Retroperitoneal Fibrosis. 61
33716037 2021
12
Validation of the diagnostic criteria for IgG4-related kidney disease (IgG4-RKD) 2011, and proposal of a new 2020 version. 61
33398598 2021
13
Clinical features of IgG4-related retroperitoneal fibrosis among 407 patients with IgG4-related disease: a retrospective study. 61
32793960 2021
14
Into Clinical Practice: Diagnosis and Therapy of Retroperitoneal Fibrosis. 61
33569638 2021
15
The First Case of Eosinophilic Granulomatosis with Polyangiitis Simultaneously Demonstrating Various Clinical Manifestations with Retroperitoneal Fibrosis and Membranous Nephropathy. 61
33518573 2021
16
Idiopathic Retroperitoneal Fibrosis With Intense Uptake of 68Ga-Fibroblast Activation Protein Inhibitor and 18F-FDG. 61
33208623 2021
17
The Case | A 64-year-old woman with retroperitoneal fibrosis, acute kidney injury, and active urine sediment. 61
33509364 2021
18
The risks of medical treatment of prolactinoma. 61
33373604 2021
19
The 2020 revised comprehensive diagnostic (RCD) criteria for IgG4-RD. 61
33274670 2021
20
Phenotyping of IgG4-related diseases based on affected organ pattern: A multicenter cohort study using cluster analysis. 61
31902317 2021
21
Long-term Outcome of Yang-Monti Ileal Replacement of the Ureter: A Technique Suitable for Mild, Moderate Loss of Kidney Function and Solitary Kidney. 61
33359492 2021
22
[A Case of IgG4-Associated Tubulointerstitial Nephritis Developing into End-Stage Renal Dysfunction Even after Release of Urinary Obstruction by Ureteral Stenting]. 61
33535292 2021
23
Primary and secondary diseases of the perinephric space: an approach to imaging diagnosis with emphasis on MRI. 61
32709392 2021
24
Living-donor liver transplantation in Budd-Chiari syndrome with inferior vena cava complete thrombosis: A case report and review of the literature. 61
33584994 2021
25
Retroperitoneal Fibrosis With a Damaged Kidney in IgG4-Related Disease. 61
31651647 2021
26
Idiopathic retroperitoneal fibrosis diagnosed by endoscopic ultrasonography-guided fine-needle biopsy. 61
33490626 2021
27
Outcomes of ureterolysis for primary retroperitoneal fibrosis: A single-center experience. 61
33477202 2021
28
Idiopathic Retroperitoneal Fibrosis. 61
33638333 2021
29
Clinical characteristics of IgG4-related retroperitoneal fibrosis versus idiopathic retroperitoneal fibrosis. 61
33600452 2021
30
IgG4 related disease as a cause of isolated retroperitoneal fibrosis with no other organ involvement; Case report. 61
33408856 2021
31
Implications of Erdheim-Chester disease and percutaneous renal access. 61
33325355 2020
32
La fibrose rétropéritonéale idiopathique. 61
32727694 2020
33
Overview of IgG4-related aortitis and periaortitis. A decade since their first description. 61
33121641 2020
34
Long-term effects of intensive B cell depletion therapy in severe cases of IgG4-related disease with renal involvement. 61
33174125 2020
35
IgG4 plasma cell myeloma without clinical evidence of IgG4-related disease: a report of two cases. 61
32893754 2020
36
Bilateral Hydronephrosis From Retroperitoneal Fibrosis. 61
33489559 2020
37
An International Patient Centred Study Of Retroperitoneal Fibrosis. 61
33377941 2020
38
Retroperitoneal fibrosis and β-blocking agents: Is there an association? 61
33326117 2020
39
Retroperitoneal fibrosis: A rare mimicker of a perirenal hematoma. 61
33072232 2020
40
[Clinical features of hydronephrosis induced by retroperitoneal fibrosis: 17 cases reports]. 61
33331315 2020
41
A Rare Cause of AA Amyloidosis: Idiopathic Retroperitoneal Fibrosis. 61
33298821 2020
42
Remission of "mistletoe sign" after treatment. 61
31409552 2020
43
Idiopathic Retroperitoneal Fibrosis Associated With a Rare Case of Multifocal Tuberculosis. 61
33252395 2020
44
Complete Remission of Advanced Hepatocellular Carcinoma Treated with Sorafenib and Concomitant Appearance of IgG4- related Diseases. A Case Report. 61
33118539 2020
45
Management of an inflammatory abdominal aortic aneurysm causing ureteric obstruction: a case report. 61
33294158 2020
46
Erdheim-Chester disease: a rapidly evolving disease model. 61
32591646 2020
47
Idiopathic membranous nephropathy in a patient diagnosed with IgG4-related disease: A case report. 61
33080759 2020
48
IgG4-related disease mimicking renal pelvis tumor with peritoneal carcinomatosis. 61
33088547 2020
49
Idiopathic retroperitoneal fibrosis: an update for nephrologists. 61
33005943 2020
50
Retroperitoneal Fibrosis and its Differential Diagnoses: The Role of Radiological Imaging. 61
32698236 2020

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 10.97 PTPRC HLA-DRB1 CADM1
2 10.76 SPP1 CCL11

GO Terms for Retroperitoneal Fibrosis

Cellular components related to Retroperitoneal Fibrosis according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 endocytic vesicle membrane GO:0030666 8.62 MDM2 HLA-DRB1

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

# Name GO ID Score Top Affiliating Genes
1 positive regulation of ERK1 and ERK2 cascade GO:0070374 9.33 PTPRC HLA-DRB1 CCL11
2 negative regulation of neuron projection development GO:0010977 9.32 VIM MDM2
3 response to steroid hormone GO:0048545 9.26 SPP1 MDM2
4 positive regulation of T cell mediated cytotoxicity GO:0001916 8.96 PTPRC HLA-DRB1
5 viral process GO:0016032 8.92 VIM PTPRC MDM2 CADM1

Sources for Retroperitoneal Fibrosis

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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