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IgG4-Related Hepatopathy

Inflammatory Pseudotumor of the Liver


Presentation

  • We report a rare case of IgG4-related hepatopathy which presented as an ill-defined heterogeneously enhancing hepatic mass with diffusion restriction and a hepatobiliary phase defect in MR and CT images.[ncbi.nlm.nih.gov]
  • Clinical description There are two clinical presentations. The active form most commonly manifests with abdominal pain, fever and loss of body weight.[orpha.net]
  • 1 Imaging findings of IgG4-related hepatopathy: A rare case presenting as a hepatic mass. ( 29886410 ) 2018 2 Inflammatory Pseudotumor of the Liver Presented in a Patient with Cholelithiasis. ( 30410837 ) Pantiora EV...Fragulidis GP 2018 3 A Diagnosis[malacards.org]
  • Yes No Clinical Presentation Diagnostic Evaluation No IgG4-RD characteristics on lymph node histopathology? Perform targeted labs and imaging studies. Consider tissue biopsy. Patient education‡. Annual re-evaluation.[slideblast.com]
  • […] general discussion:10 min 11:15-12:15 Poster Round Presentation and Discussion 12:20-13:25 Topic Seminar (with lunch) 4 speakers: 15min x 4 presentation 13:30-15:15 Symposium 2 6 topics: 15min x 6 presentation general discussion:10 min 15:15-15:30 Break[shinshu-u.ac.jp]
Inflammation
  • Immunoglobulin G4-hepatopathy, which includes various histopathological lesions encountered in the liver of patients with type I autoimmune pancreatitis, is classified into five histological categories: portal inflammation, large bile duct damage, portal[ncbi.nlm.nih.gov]
  • […] article Summary Wikipedia PMID: 25062559 DOI: 10.1016/j.ijcard.2014.07.046 Author(s): Atallah, Pierre Charbel; Kassier, Adnan; Powers, Stephanie Publisher(s): Elsevier BV Tags: Medicine Pericarditis Nov. 15, 2018 English Pericarditis is inflammation of[plu.mx]
  • Umemura T. et al. classified the liver histology of AIP in 5 patterns: evident portal inflammation with or without interface hepatitis, large bile-duct obstructive features, portal sclerosis, lobular hepatitis and canalicular cholestasis.[deepdyve.com]
Pseudotumor
  • pseudotumor of the liver. ( 27476013 ) Hamzaoui L...Msadak Azouz M 2016 7 Inflammatory Pseudotumor of the Liver with Escherichia coli in the Sputum. ( 26347780 ) Narayan S...King CL 2015 8 Inflammatory pseudotumor of the liver: A case report and literature[malacards.org]
  • Immunoglobulin G4-related disease (IgG4-RD) is a systemic disease involving many organs; it includes IgG4-related sclerosing cholangitis and inflammatory pseudotumor in the hepatobiliary system.[ncbi.nlm.nih.gov]
  • In fact, they include only inflammatory pseudotumors. The liver-related manifestations of IgG4-RD are heterogeneous and less well described within the literature [2].[deepdyve.com]
  • Pathological classification of hepatic inflammatory pseudotumor with respect to IgG4-related disease. Mod Pathol 2007; 20 (8) 884-894 8 Nakanuma Y, Zen Y.[thieme-connect.com]
  • 主要主题词: Hepatitis, Autoimmune; Immunoglobulin G 摘要 Immunoglobulin G4-related disease (IgG4-RD) is a systemic disease involving many organs; it includes IgG4-related sclerosing cholangitis and inflammatory pseudotumor in the hepatobiliary system.[paper.medlive.cn]
Sputum
  • […] virus-related hepatocellular carcinoma treatment: A case report. ( 26826935 ) Honmyo N...Ohdan H 2016 6 Inflammatory pseudotumor of the liver. ( 27476013 ) Hamzaoui L...Msadak Azouz M 2016 7 Inflammatory Pseudotumor of the Liver with Escherichia coli in the Sputum[malacards.org]
Chest Pain
  • CLINICAL FEATURES Respiratory symptoms including cough, exertional dyspnoea and chest pain have been described in approximately one-half of patients with pulmonary ISD, while the remaining patients presented with abnormal intrathoracic findings on imaging[erj.ersjournals.com]
  • Patients may present with cough, dyspnea, chest pain or frequently remain asymptomatic [[ 88 ],[ 89 ]]. Intrapulmonary manifestations include nodules and mass lesions (“inflammatory pseudotumors”) but may also present as interstitial lung disease.[ojrd.biomedcentral.com]
Hepatic Mass
  • We report a rare case of IgG4-related hepatopathy which presented as an ill-defined heterogeneously enhancing hepatic mass with diffusion restriction and a hepatobiliary phase defect in MR and CT images.[ncbi.nlm.nih.gov]
  • 1 Imaging findings of IgG4-related hepatopathy: A rare case presenting as a hepatic mass. ( 29886410 ) 2018 2 Inflammatory Pseudotumor of the Liver Presented in a Patient with Cholelithiasis. ( 30410837 ) Pantiora EV...Fragulidis GP 2018 3 A Diagnosis[malacards.org]
Liver Dysfunction
  • Liver dysfunction is frequently seen in autoimmune pancreatitis (AIP) patients. Virtually, all AIP liver biopsies show evidence of various pathological changes and infiltration of IgG4-bearing plasma cells.[deepdyve.com]

Treatment

  • The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.[orpha.net]
  • IgG4-Related Autoimmune Hepatitis and IgG4-Related Hepatopathy in IgG4-Related Disease. ( 27466793 ) Nakanuma Y....Umemura T. 2016 5 Inflammatory pseudotumor of the liver occurring during the course of hepatitis C virus-related hepatocellular carcinoma treatment[malacards.org]
  • All patients were further followed up for 2 years and showed good response to treatment. There was no significant difference in treatment response between IgG4 positive and negative cases.[tropicalgastro.com]
  • In contrast to PSC”, IAC shows features of an autoimmune cholangitis with extra- and intrahepatic biliary strictures which promptly respond to steroid treatment.[thieme-connect.com]
  • Readers will come away with a full overview of the diagnosis and treatment of cholestatic liver diseases.[books.google.it]

Prognosis

  • . 2014 10 Inflammatory pseudotumor of the liver diagnosed as metastatic liver tumor in a patient with a gastrointestinal stromal tumor of the rectum: report of a case. ( 24886578 ) Matsuo Y...Takeyama H 2014 11 Clinical features, image findings, and prognosis[malacards.org]
  • Finally, PSC patients with elevated IgG4 serum levels [IgG4( )PSC] have also been described and may constitute a special steroid-sensitive subpopulation of PSC patients with a worse prognosis than “classical” PSC patients with normal IgG4 serum levels[thieme-connect.com]
  • Prognosis IAC prognosis seems to be more favorable than classic PSC; however, since it is a relatively new entity limited data exists on the its clinical course and prognosis , and further long-term follow-up studies are needed [ 6 ].[autoimmunediseases.imedpub.com]
  • PROGNOSIS Although the response to corticosteroid therapy is favourable in most patients with intrathoracic ISD, long-term follow-up data are currently not available.[erj.ersjournals.com]
  • Treatment and prognosis Although some patients responded to biliary drainage or surgical resection, IgG4-SC displays a good response to steroid therapy, as is the case for pancreatic lesions.[wjgnet.com]

Etiology

  • A significantly elevated cutoff ratio was seen in only one IgG4-related disease and one case with unknown etiology.[scholarlypages.org]
  • Table 1 Etiology of secondary sclerosing cholangitis.[wjgnet.com]
  • Recent evidence has confirmed its neoplastic etiology.[hanspopperhepatopathologysociety.org]
  • Minato H, Shimizu J, Arano Y, Saito K, Masunaga T, Sakashita T et al (2012) IgG4-related sclerosing mesenteritis: a rare mesenteric disease of unknown etiology. Pathol Int 62:281–286 CrossRef PubMed 40.[springermedizin.de]
  • Sclerosing cholangitis of unknown etiology is called PSC. PSC is typically diagnosed between the ages of 30 and 40 years and is frequently associated with inflammatory bowel disease [ 7 ].[autoimmunediseases.imedpub.com]

Epidemiology

  • Summary Epidemiology Approximately 140 cases have been reported worldwide, with a higher prevalence for male adults of Asian origin and subjects affected by systemic diseases such as rheumatoid arthritis.[orpha.net]
  • […] and pathogenesis of autoimmune pancreatitis” Chaired by Kazuichi Okazaki (Kansai Medical University) and Myung-Hwan Kim (Asan Medical Center) Part 1 1) A nationwide epidemiological survey of autoimmune pancreatitis in Japan (15 min) Atsushi Kanno (Tohoku[shinshu-u.ac.jp]
  • Epidemiology As recognition of IgG4-RD is relatively recent, there are limited studies on its epidemiology. It is therefore difficult to make an accurate estimation of prevalence.[infogalactic.com]
  • The impact of race/ethnicity on the clinical epidemiology of autoimmune hepatitis. J Clin Gastroenterol. 2012;46:155–61. Boberg KM. Prevalence and epidemiology of autoimmune hepatitis. Clin Liver Dis. 2002;6:635–47.[tropicalgastro.com]
  • IgG4-RD is relatively recent, there are limited studies on its epidemiology.[ipfs.io]
Sex distribution
Age distribution

Pathophysiology

  • IgG4-related disease: pathophysiologic insights drive emerging treatment approaches. Clin Exp Rheumatol 2016; 34 (Suppl. 98): S00-S00. 9. Della Torre E, Mattoo H, Mahajan VS, Carruthers M, Pillai S, Stone JH.[journals.ke-i.org]
  • But development of cholangiocarcinoma has not been reported to occur in patients with IAC, suggesting a different pathophysiologic mechanism [ 38 ]. Conclusion IAC is a recently described disease with a largely unknown pathogenesis.[autoimmunediseases.imedpub.com]
  • This review summarizes current knowledge on clinical manifestations, pathophysiology and treatment of IgG4-RD.[ojrd.biomedcentral.com]
  • J Hepatobiliary Pancreat Surg 14 35-45 2007 [Refereed] Definitions, pathophysiology, and epidemiology of acute cholangitis and cholecystitis: Tokyo Guidelines.[researchmap.jp]

Prevention

  • France - Agence Nationale du Médicament Vétérinaire 18-7-2018 "Anti-pollution" masks: not enough data to demonstrate a health benefit and justify recommending their use In a context where prevention of ambient air pollution is a real public health issue[myhealthbox.eu]
  • PubMed Text format Abstract available SCHEPIS F, Turco L, Bianchini M, Villa E, et al Prevention and Management of Bleeding Risk Related to Invasive Procedures in Cirrhosis. Semin Liver Dis. 2018;38:215-229.[amedeo.com]
  • Fc-Fc binding could potentially prevent immune responses by blocking interactions with complement proteins and Fc receptors.[kjpbt.org]
  • "Force majeure" term will be regarded as outside of the concerned party's reasonable control and any situation that "Turkiye Klinikleri" cannot prevent even though it shows due diligence.[turkiyeklinikleri.com]
  • Treatment The goal of treatment is the induction and maintenance of remission so as to prevent progression of fibrosis and organ destruction in affected organ(s).[infogalactic.com]

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