We discuss the IgG4 related retroperitoneal fibrosis in detail along with its varied presentations. [ncbi.nlm.nih.gov]

• Soft Tissue Mass

  With a diagnosis of IgG4-related retroperitoneal fibrosis, he was treated with steroid, and the leg edema decreased with improvement of patency of the IVC and reduction of the soft tissue mass. [ncbi.nlm.nih.gov]

• Leg Swelling

  Herein we report a case of a 46 year old male presenting with 4 months history of backache, fever, flank pain and leg swelling for 2 weeks. Investigations revealed acute kidney injury diagnosed as a result of bilateral ureteric obstruction. [ncbi.nlm.nih.gov]

  Pain, leg swelling, and a reduction in kidney function can occur. This disorder results in decreased blood flow from the aorta to the lower part of your body. Initially, your body reacts to the reduced blood flow. [healthline.com]

• Inguinal Hernia

  Unusual causes that can cause ureteric obstruction include barium granuloma after colon perforation, use of sclerosing agents for treatment of inguinal hernia and hemorrhoids, and methyl methacrylate cement used for joint replacement. [emedicine.com]

• Leg Edema

  With a diagnosis of IgG4-related retroperitoneal fibrosis, he was treated with steroid, and the leg edema decreased with improvement of patency of the IVC and reduction of the soft tissue mass. [ncbi.nlm.nih.gov]

• Edema of Lower Extremity

  She had edema in lower extremities. [omicsonline.org]

• Left Flank Pain

  A 71-year-old man visited our hospital with the complaint of left flank pain and gross hematuria. [ncbi.nlm.nih.gov]

• Lower Abdominal Pain

  We report a case of 69-year-old male with recurrent lower abdominal pain for 10 months. [ncbi.nlm.nih.gov]

  Case Report and Discussion A 40 year old male with essential hypertension on regular therapy since last 2 years presented with complain of vague lower abdominal pain of 5 months duration. [japi.org]

• Thigh Pain

  A 54-year-old man presented with right lower abdominal and upper thigh pain. Cross-sectional imaging of the abdomen revealed right hydronephrosis caused by a mass adjacent to the bladder. [em-consulte.com]

Computed tomography (CT), magnetic resonance imaging (MRI), and endoscopic retrograde cholangiopancreatography (ERCP) are usually included in the diagnostic workup. [hematologyandoncology.net]

Workups for infectious causes for RPF including histoplasmosis or tuberculosis were negative. She has no history of radiation to the pelvis. [elynsgroup.com]

Workup IgG4-related disease is diagnosed on the basis of clinical, biochemical and histopathological findings. It is essential to exclude differential diagnoses, some of which are shown in box 2. [tidsskriftet.no]

See Workup for more detail. [emedicine.medscape.com]

• Abnormal Renal Function

  Liver dysfunction was absent; however, abnormal renal function was shown. Serum levels of IgG and IgG4 were elevated to 2,281 and 652 mg/dL, respectively. [pancreas.imedpub.com]

• Fibrinoid Necrosis

  Necrosis, especially fibrinoid necrosis, is not characteristic of IgG4-RD. Figure 1. Obliterative phlebitis in a case of retroperitoneal fibrosis. A 54-year-old man presented with right lower abdominal and upper thigh pain. [em-consulte.com]

After 2 months treatment, the patient showed rapid improvement. This is a case of IgG4-RRPF with multiple organs involvement. Glucocorticoid is the first-line treatment. [ncbi.nlm.nih.gov]

References: [3] [1] [4] [5] Pathophysiology Clinical features References: [3] [1] [4] [6] [7] [8] [9] Diagnostics Treatment Prognosis [amboss.com]

References: [3] [1] [4] [5] Pathophysiology Clinical features References: [3] [1] [4] [6] [7] [8] [9] Diagnostics Treatment Prognosis [amboss.com]

We review the definition, epidemiology, clinical features, diagnostic criteria, treatment and prognosis of IgG4-related RPF in this article to raise awareness of this newly characterized disease. 2016 Asia Pacific League of Associations for Rheumatology [ncbi.nlm.nih.gov]

The prognosis for patients with the disorder varies according to the type. It is relatively good for idiopathic retroperitoneal fibrosis, with a small long-term effect on quality and quantity of life. [news-medical.net]

[…] has a poor prognosis. [patient.info]

Understanding of its various clinical manifestations, response to treatment and prognosis has grown substantially in the past decade. It is now recognized to involve potentially any organ system either one at a time or several at once. [medicine.emory.edu]

Etiology Secondary retroperitoneal fibrosis Malignancies and exposure to methysergide are the most common causes of secondary RPF ! [amboss.com]

A 79-year-old man had a 3.5-year history of edema of the lower extremities of unknown etiology. [ncbi.nlm.nih.gov]

Etiology Aetiology is unknown in most patients, hence the name idiopathic retroperitoneal fibrosis''. Diagnostic methods RPF can be diagnosed by computed tomography, magnetic resonance imaging or biopsy. [orpha.net]

Epidemiology Epidemiological data refers to the US, unless otherwise specified. Etiology Secondary retroperitoneal fibrosis Malignancies and exposure to methysergide are the most common causes of secondary RPF ! [amboss.com]

We review the definition, epidemiology, clinical features, diagnostic criteria, treatment and prognosis of IgG4-related RPF in this article to raise awareness of this newly characterized disease. 2016 Asia Pacific League of Associations for Rheumatology [ncbi.nlm.nih.gov]

Summary Epidemiology Its incidence varies between 0.4 and 1/200 000. Clinical description Symptoms may vary depending upon the location and extent of fibrotic growth. The commonest manifestations are pain, weight loss, nausea and vomiting. [orpha.net]

Epidemiology Retroperitoneal fibrosis is an uncommon disease that affects 1 person per 200,000-500,000 population per year, depending on the specific location. [news-medical.net]

References: [3] [1] [4] [5] Pathophysiology Clinical features References: [3] [1] [4] [6] [7] [8] [9] Diagnostics Treatment Prognosis [amboss.com]

Abstract Purpose of review Remarkable insights have been gleaned recently with regard to the pathophysiology of IgG4-related disease (IgG4-RD). [ncbi.nlm.nih.gov]

Pathophysiology: the role of IgG4 IgG4 itself is unlikely to drive the pathogenesis of IgG4-RD  The properties of the IgG4 molecule suggest that it would suppress inflammation. [slideshare.net]

This further supports the similar underlying pathophysiology of the two conditions. Similar results were seen in our patient who responded in the first week after starting the steroid therapy. [japi.org]

They concluded, "As knowledge of the pathophysiology of this fibroinflammatory disease advances, treatment algorithms are also likely to evolve to include therapies that target the problematic fibrotic lesions as well as the highly cellular lymphoplasmacytic [medpagetoday.com]

Since immunesuppressants or B-cell targeted therapies may halt or reverse progression, early diagnosis and treatment is important to prevent terminal fibrosis. [ard.bmj.com]

Since the majority of cases cannot be linked to any specific cause, prevention may not be possible. [healthline.com]

Cancer care at Mayo Clinic's campus in Arizona Mayo Clinic experts in Arizona discuss the new facility, which allows them to create a seamless experience for patients by integrating all cancer services from prevention to survivorship. [medprofvideos.mayoclinic.org]

Surgical ureterolysis can obviously relieve the obstruction of ureter, and can prevent the further deterioration of renal function, but it cannot prevent the progression of the primary disease [ 17 ]. [omicsonline.org]

Can retinitis pigmentosa be prevented? Because it is a genetic condition that is inherited from one or both of your parents, retinitis pigmentosa cannot be prevented. [specsavers.co.uk]