Presentation
Four years later, he presented to the urologist with severe left hydronephrosis because of marked thickness of the left ureter wall. [ncbi.nlm.nih.gov]
Entire Body System
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Soft Tissue Mass
Computed tomography scans showed a soft-tissue mass with an irregular border and mild homogeneous enhancement in the right renal pelvis and calyces. [ncbi.nlm.nih.gov]
Gastrointestinal
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Pelvic Mass
Keywords: IgG4-related kidney disease ; Autoimmune pancreatitis ; Renal pelvic mass [ekjm.org]
Workup
Kashani, Biomarkers for Early Detection of Acute Kidney Injury, The Journal of Applied Laboratory Medicine: An AACC Publication, September 2017, jalm.2017.023325; DOI: 10.1373/jalm.2017.023325 (May 1, 2017) Arora, P Chronic Kidney Disease Workup. [labtestsonline.it]
The diagnosis agreed upon after the analysis of clinical findings and workup was IgG4-RD with extensive renal, lymph node, and pancreatic involvement. The patient was started on prednisone 60 mg/day. [bjn.org.br]
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]
Imaging workup for IgG4-related disease should include a CT scan of the chest, abdomen and pelvis to detect possible multiorgan involvement, specifics of which will be further described. [rarediseasesjournal.com]
Treatment
A large-scale prospective study to formulate more useful treatment strategies is necessary. [ncbi.nlm.nih.gov]
[…] before corticosteroid treatment had been less than 60 ml/min. [acrabstracts.org]
& Medicine Current slide {CURRENT_SLIDE} of {TOTAL_SLIDES}- Best-selling in Health, Treatments & Medicine This item doesn't belong on this page. [ebay.co.uk]
Prognosis
The prognosis of the disease has not been clearly defined. [springerplus.springeropen.com]
Do mesangial immune complex deposits affect the renal prognosis in membranous glomerulonephritis? Clin. Nephrol. 41, 271–276 (1994). 39. Vaglio, A., Salvarani, C. & Buzio, C. Retroperitoneal fibrosis. Lancet 367, 241–251 (2006). 40. van Bommel, E. [nature.com]
What is the prognosis of IgG4-related disease? Prognosis of IgG4-related disease is variable. It may spontaneously resolve or persist, with remitting and relapsing symptoms. [dermnetnz.org]
Early diagnosis and treatment improve the prognosis of affected patients. REFERENCES Stone JH, Zen Y, Deshpande V. IgG4-related disease. N Engl J Med 2012;366:539-51. [bjn.org.br]
There is a report that the prognosis for progressive renal disease is more likely when the IgE is greater than 436 IU/ml in patients with IgG4 renal disease. In summary, I would not do any additional tests. [aaaai.org]
Etiology
Pt was told that he is in stage 4 renal failure; biopsies are inconclusive; no consultants are able to determine the etiology of the renal disease. [aaaai.org]
Granulomas are unusual and if found suggest alternative etiology. Though histologic features are similar for all organs, subtle pathologic variations exist among affected organ systems. [rarediseasesjournal.com]
"Clinical, radiographic, and pathologic description of IgG4-related perivasal fibrosis: a previously undescribed etiology of chronic orchialgia". Urology. 84 (4): 748–750. doi : 10.1016/j.urology.2014.06.035. [en.wikipedia.org]
Hypertrophic Pachymeningitis: IgG4-related disease as a common etiology. Medicine (Baltimore) 92:206-16, 2013. PMID: 23793110 Wallace ZS, Deshpande V, Stone JH. [pancreapedia.org]
Epidemiology
Few data exist on the epidemiological and clinical features of large series of patients. The mean age at diagnosis of the reviewed cases is 65 years, and 73–87 % are men [ 8 ]. [bmcurol.biomedcentral.com]
Epidemiologic understanding of IgG4-related disease is limited due to insufficient awareness of the diagnosis and its only recent appearance in the medical literature. [rarediseasesjournal.com]
Almost all the IgG-positive plasma cells are also positive for IgG4 (brown colour) The epidemiology has been only partly determined, but prevalence is estimated to be 0.28 – 1.08 cases per 100 000 ( 2 ). [tidsskriftet.no]
[ edit ] As recognition of IgG4-RD is relatively recent, there are limited studies on its epidemiology. [en.wikipedia.org]
Epidemiology data regarding IgG4-RD comes mainly from Japan, reporting 0.28–1.08 new diagnoses per 100,000 population and a prevalence of roughly 8,000 patients (compared to the total country population approximating 130 million people) [4]. [elynsgroup.com]
Pathophysiology
The relation between the location and components of fibrosis determined in whole kidney samples provides new clues to the pathophysiology underlying IgG4-RKD. Copyright 2016 The Authors. Published by Elsevier Inc. All rights reserved. [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]
Humoral and cell-mediated immunity seem to have roles in the pathophysiology of IgG4-RD, but the details of these roles remain unclear. [nature.com]
This suggests that despite a common immunoglobulin subclass, the pathophysiology of primary MGN is dissimilar from IgG4-RD. [elynsgroup.com]
The high rate of relapse observed after RTX treatment in our study also raises pathophysiological issues. [journals.plos.org]
Prevention
If IgG4-RSD is diagnosed early, we can usually control the inflammation within organs and prevent further damage. [massgeneral.org]
Centers for Disease Control and Prevention. CDC Features - Protect Your Kidneys: Control Diabetes, Blood Pressure. Available online at . Accessed February 2013. [labtestsonline.it]
Early recognition and therapy are important to prevent serious and irreversible tissue damage. Further epidemiologic data are needed to confirm and validate early observations. [rarediseasesjournal.com]
Targeting activation-induced cytidine deaminase prevents colon cancer development despite persistent colonic inflammation. Oncogene. 2012 Mar 29;31(13):1733-42. 消化器をつなぐ免疫ネットワーク 近年、消化器疾患の病態に、免疫、代謝などによる全身のネットワークが大きく影響を及ぼしていることが明らかになってきました。 [kuhp.kyoto-u.ac.jp]
Antiproliferative agents such as mycophenolate mofetil or azathioprine combined with methotrexate may be used to prevent or reduce the incidence of steroid-related adverse events, 1, 2, 10, 11 but their efficacy has not been tested in clinical trials. [bjn.org.br]