IgG4-related retroperitoneal fibrosis

Retroperitoneal fibrosis (RPF) is characterized by the development of a fibrotic mass surrounding retroperitoneal structures, such as aorta, vena cava, ureters and psoas muscle.



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Narrow down the case reports



Total: 222 (papers)

   


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Matched Phenotype  Gene  Mutation  MeSH
Rank
(Similarity)		 PMID
(PMCID)
1
(47.8%)		 18172449
 A case of Erdheim-Chester disease initially mistaken for Ormond's disease.
Loddenkemper K, Hoyer B, Loddenkemper C, Hermann KG, Rogalla P, Forster G, Buttgereit F, Hiepe F, Burmester GR.
Nat Clin Pract Rheumatol. 2008;4(1):50-5.
Anemia Fever Retroperitoneal fibrosis
B2M CRP
Anemia Antineoplastic Agents Differential Diagnosis Erdheim-Chester Disease Homo sapiens Hydronephrosis Interferon Type I Male Middle Aged Recombinant Proteins
1
(47.8%)		 11341126
 [Idiopathic retroperitoneal fibrosis].
Madrigal Rubiales B, Vara A, Ablanedo P, Herreros M, Vazquez L, Fresno M.
Arch Esp Urol. 2001;54(2):176-80.
Anemia Elevated erythrocyte sedimentation rate Retroperitoneal fibrosis
Adult Homo sapiens Male
3
(43.7%)		 8129011
 A case of uveitis associated with idiopathic retroperitoneal fibrosis.
Doi M, Uji Y.
Am J Ophthalmol. 1994;117(3):358-62.
Edema Anemia Abdominal pain
Blood Sedimentation Females Fluorescein Angiography Glucocorticoids Homo sapiens Magnetic Resonance Imaging Middle Aged Panuveitis Urography Vision Disorders X-Ray Computed Tomography
4
(40.8%)		 6119254
 Retroperitoneal fibrosis: a two decade experience and gynecologic manifestations.
Kurzel RB, Yoonesi M, Munabi A.
Int J Gynaecol Obstet. 1981;19(4):271-80.
Thrombocytopenia Azotemia Retroperitoneal fibrosis Pelvic mass
Adult Differential Diagnosis Female Genital Diseases Females Genital Neoplasms, Female Homo sapiens Middle Aged
5
(40.0%)		 15293141
 Hypoplasia of the right hepatic lobe associated with portal hypertension and idiopathic retroperitoneal fibrosis.
Hisatomi K, Ohya T, Asayama M, Shibata M, Ito M, Sakurai Y, Sato Y.
J Gastroenterol. 2004;39(7):685-91.
Cirrhosis Splenomegaly Thrombocytopenia
Abdomen Adult Collateral Circulation Homo sapiens Liver Magnetic Resonance Angiography Magnetic Resonance Imaging Male Portal Hypertension Radiography, Abdominal Thrombocytopenia X-Ray Computed Tomography
6
(34.0%)		 18365137
 An unexpected cause of a febrile patient with huge splenomegaly.
Nonami A, Yamamoto H, Nakamura M, Nagafuji K, Teshima T.
Clin Rheumatol. 2008;27(7):941-3.
Splenomegaly Fever
Adult Anti-Inflammatory Agents Fever of Unknown Origin Homo sapiens Male Splenectomy Splenomegaly
7
(33.5%)		 14749985
 Improvement of Erdheim-Chester disease in two patients by sequential treatment with vinblastine and mycophenolate mofetil.
Jendro MC, Zeidler H, Rosenthal H, Haller H, Schwarz A.
Clin Rheumatol. 2004;23(1):52-6.
Plethora Histiocytosis
Antineoplastic Agents, Phytogenic Combination Drug Therapy Drug Administration Schedule Erdheim-Chester Disease Follow-Up Studies Homo sapiens Immunosuppressive Agents Male Middle Aged
8
(32.8%)		 23857085
 Clinical features of 10 patients with IgG4-related retroperitoneal fibrosis.
Chiba K, Kamisawa T, Tabata T, Hara S, Kuruma S, Fujiwara T, Kuwata G, Egashira H, Koizumi K, Koizumi S, Fujiwara J, Arakawa T, Momma K, Setoguchi K, Shinohara M.
Intern Med. 2013;52(14):1545-51.
Hydronephrosis Edema Eosinophilia Lymphadenopathy
Adult Females Follow-Up Studies Homo sapiens Inflammation Mediators Male Middle Aged Retrospective Studies
9
(30.7%)		 22675100
 Idiopathic retroperitoneal fibrosis presenting with recurrent bilateral uveitis.
Alpert DR, Patel J, Chiang PK.
BMJ Case Rep. 2011;2011:.
Hydronephrosis Deep venous thrombosis Retroperitoneal fibrosis
Anti-Inflammatory Agents Edema Females Homo sapiens Lower Extremity Middle Aged Uveitis
9
(30.7%)		 12402479
 [Idiopathic retroperitoneal fibrosis with large vessel thrombosis].
Tanuma Y, Yokoo A.
Hinyokika Kiyo. 2002;48(9):539-43.
Hydronephrosis Deep venous thrombosis Retroperitoneal fibrosis
Anticoagulants Females Fibrinolytic Agents Homo sapiens Iliac Vein Magnetic Resonance Angiography Middle Aged Venous Thrombosis X-Ray Computed Tomography
        

Phenotype(s) retrieved from Orphanet

    Total: 43

HPO ID Term Frequency
HP:0012531 Pain Very frequent (99-80%)
HP:0000083 Renal insufficiency Frequent (79-30%)
HP:0000126 Hydronephrosis Frequent (79-30%)
HP:0000822 Hypertension Frequent (79-30%)
HP:0001824 Weight loss Frequent (79-30%)
HP:0001897 Normocytic anemia Frequent (79-30%)
HP:0002027 Abdominal pain Frequent (79-30%)
HP:0002039 Anorexia Frequent (79-30%)
HP:0003138 Increased blood urea nitrogen Frequent (79-30%)
HP:0003259 Elevated serum creatinine Frequent (79-30%)
HP:0003419 Low back pain Frequent (79-30%)
HP:0003565 Elevated erythrocyte sedimentation rate Frequent (79-30%)
HP:0005310 Large vessel vasculitis Frequent (79-30%)
HP:0011227 Elevated C-reactive protein level Frequent (79-30%)
HP:0012378 Fatigue Frequent (79-30%)
HP:0012583 Unilateral renal hypoplasia Frequent (79-30%)
HP:0025379 Anti-thyroid peroxidase antibody positivity Frequent (79-30%)
HP:0030157 Flank pain Frequent (79-30%)
HP:0031191 Deep dermal perivascular inflammatory infiltrate Frequent (79-30%)
HP:0000074 Ureteropelvic junction obstruction Occasional (29-5%)
HP:0000872 Hashimoto thyroiditis Occasional (29-5%)
HP:0001370 Rheumatoid arthritis Occasional (29-5%)
HP:0001919 Acute kidney injury Occasional (29-5%)
HP:0001945 Fever Occasional (29-5%)
HP:0002017 Nausea and vomiting Occasional (29-5%)
HP:0002019 Constipation Occasional (29-5%)
HP:0002725 Systemic lupus erythematosus Occasional (29-5%)
HP:0002923 Rheumatoid factor positive Occasional (29-5%)
HP:0003262 Smooth muscle antibody positivity Occasional (29-5%)
HP:0003453 Antineutrophil antibody positivity Occasional (29-5%)
HP:0003765 Psoriasiform dermatitis Occasional (29-5%)
HP:0010741 Pedal edema Occasional (29-5%)
HP:0012578 Membranous nephropathy Occasional (29-5%)
HP:0000034 Hydrocele testis Very rare (4-1%)
HP:0000100 Nephrotic syndrome Very rare (4-1%)
HP:0000790 Hematuria Very rare (4-1%)
HP:0000802 Impotence Very rare (4-1%)
HP:0002639 Budd-Chiari syndrome Very rare (4-1%)
HP:0008682 Acute tubular necrosis Very rare (4-1%)
HP:0012871 Varicocele Very rare (4-1%)
HP:0012877 Retrograde ejaculation Very rare (4-1%)
HP:0100518 Dysuria Very rare (4-1%)
HP:0100817 Renovascular hypertension Very rare (4-1%)


Phenotype(s) retrieved from case reports

    Total: 47

HPO ID Term # of case reports
HP:0005200 Retroperitoneal fibrosis 12
HP:0000126 Hydronephrosis 7
HP:0006000 Ureteral obstruction 7
HP:0005112 Abdominal aortic aneurysm 5
HP:0100646 Thyroiditis 5
HP:0000969 Edema 3
HP:0001733 Pancreatitis 3
HP:0001945 Fever 3
HP:0000819 Diabetes mellitus 2
HP:0000822 Hypertension 2
HP:0000873 Diabetes insipidus 2
HP:0001409 Portal hypertension 2
HP:0002617 Dilatation 2
HP:0003419 Low back pain 2
HP:0030731 Carcinoma 2
HP:0000093 Proteinuria 1
HP:0000099 Glomerulonephritis 1
HP:0000100 Nephrotic syndrome 1
HP:0000622 Blurred vision 1
HP:0000790 Hematuria 1
HP:0001396 Cholestasis 1
HP:0001744 Splenomegaly 1
HP:0001824 Weight loss 1
HP:0001903 Anemia 1
HP:0001920 Renal artery stenosis 1
HP:0001970 Tubulointerstitial nephritis 1
HP:0002027 Abdominal pain 1
HP:0002653 Bone pain 1
HP:0002664 Neoplasm 1
HP:0002835 Aspiration 1
HP:0002955 Granulomatosis 1
HP:0003040 Arthropathy 1
HP:0003418 Back pain 1
HP:0003565 Elevated erythrocyte sedimentation rate 1
HP:0004417 Intermittent claudication 1
HP:0004936 Venous thrombosis 1
HP:0006280 Chronic pancreatitis 1
HP:0012089 Arteritis 1
HP:0012126 Stomach cancer 1
HP:0012490 Panniculitis 1
HP:0025142 Constitutional symptom 1
HP:0030155 Scrotal pain 1
HP:0031281 Sialadenitis 1
HP:0031500 Abdominal mass 1
HP:0100520 Oliguria 1
HP:0100651 Type I diabetes mellitus 1
HP:0100727 Histiocytosis 1


Causative gene(s) retrieved from Orphanet

    Total: 0

Gene Symbol Gene Name Entrez Gene ID