Hepatology: A clinical textbook - Phần 2
➤ Gửi thông báo lỗi ⚠️ Báo cáo tài liệu vi phạmNội dung chi tiết: Hepatology: A clinical textbook - Phần 2
Hepatology: A clinical textbook - Phần 2
Extrahepatic Manifestations of Chronic HCV 32315. Extrahepatic Manifestations of ChronicHCVAlbrecht Bôhlig Karl-Philipp Puchner and Thomas BergIntrodu Hepatology: A clinical textbook - Phần 2uctionPatients with chronic hepatitis c virus (HCV) infection are at risk of a variety of extrahepatic manifestations (EHMs) (Table 1) - up to 70% of patients develop HCV EHMs according to large cohort studies (Cacoub 2000. Cacoub 1999). EHMs may often be the first and only clinical sign of chronic Hepatology: A clinical textbook - Phần 2hepatitis c infection. Evidence of HCV infection should always be ruled out in cases of non-specific chronic fatigue and or rheumatic, hematological,Hepatology: A clinical textbook - Phần 2
endocrine or dermatological disorders. The pathogenesis of EHM is still not fully understood although most studies suggest that the presence of mixed Extrahepatic Manifestations of Chronic HCV 32315. Extrahepatic Manifestations of ChronicHCVAlbrecht Bôhlig Karl-Philipp Puchner and Thomas BergIntrodu Hepatology: A clinical textbook - Phần 2ors (Ferri 2007). Nevertheless, the pathogenesis and epidemiology of many EHMs require further investigation (Figure 1). Our aim is to give a brief insight into the epidemiology, pathogenesis, clinical relevance and therapeutic management of HCV-associated EHM (Zignego 2007a).Mixed cryoglobulinemiaC Hepatology: A clinical textbook - Phần 2ryoglobulinemia refers to the presence of abnormal immunoglobulins in the serum, which have the unusual property of precipitating at temperatures beloHepatology: A clinical textbook - Phần 2
w 37°c and redissolving at higher temperatures. The phenomenon of cryoprecipitation was first described in 1933 (Wintrobe 1933). Cryoglobulins (CGs) aExtrahepatic Manifestations of Chronic HCV 32315. Extrahepatic Manifestations of ChronicHCVAlbrecht Bôhlig Karl-Philipp Puchner and Thomas BergIntrodu Hepatology: A clinical textbook - Phần 2unoglobulins, are prevalent in patients with chronic HCV infection, while type I CGs, consisting exclusively of monoclonal components, are mostly found in patients with lymphoproliferative disorders (multiple myeloma. B cell lymphoma, Waldenstrom macroglobulinemia). Tv-na n fa mA ĩĩĩ mivadnamin irin Hepatology: A clinical textbook - Phần 2 10^4t i An tr Iirifli324 Hepatology 2015immunocomplexes in only 30% of them (Lunel 1994. Wong 1996). Asymptomatic mixed cryoglobulinemia during the cHepatology: A clinical textbook - Phần 2
ourse of chronic HCV infection may evolve into symptomatic disease. Patients with symptomatic mixed cryoglobulinemia exhibit higher cryoglobulin conceExtrahepatic Manifestations of Chronic HCV 32315. Extrahepatic Manifestations of ChronicHCVAlbrecht Bôhlig Karl-Philipp Puchner and Thomas BergIntrodu Hepatology: A clinical textbook - Phần 2e a key incidence in cryoglobulinemia-derived pathogenesis. Factors that seem to favour the development of .VIC are female sex. age. alcohol intake (>50gd), advanced liver fibrosis and steatosis (Lunel 1994, Wong 1996. Saadoun 2006).Table 1. Extrahepatic manifestations of chronic hepatitis c infecti Hepatology: A clinical textbook - Phần 2onOrgan/System involvedManifestationEndocrine•Autoimmune thyroidopathies (in particular. Hashimoto thyroiditis) •Insulin resistance/diabetes mellitus*Hepatology: A clinical textbook - Phần 2
•Growth hormone (GH) insufficiency •Vitamin D deficiencyRheumatic disorders•Mixed cryoglobulinemia’ •Cryoglobulinemic vasculitis’ •Peripheral neuropaExtrahepatic Manifestations of Chronic HCV 32315. Extrahepatic Manifestations of ChronicHCVAlbrecht Bôhlig Karl-Philipp Puchner and Thomas BergIntrodu Hepatology: A clinical textbook - Phần 2 syndromeHematologic disorders•Lymphoproliferative disorders/Non-Hodgkin Lymphomas* •Immune thrombocytopenic purpura (ITP) •Monoclonal gammopathies* •Autoimmune hemolytic anemiaDermatologic disorders•Palpable purpura •Porphyria cutanea tarda (PCT) •Lichen planus •PruritusCentral nervous system disor Hepatology: A clinical textbook - Phần 2ders•Chronic fatigue*, subclinical cognitive impairment, psychomotoric deceleration, symptoms of depression* •Neurocognitive disordersMiscellaneous•MyHepatology: A clinical textbook - Phần 2
opathy •Cardiomyopathy/Myocarditis •Idiopathic pulmonal fibrosis'Associations based on strong epidemiological prevalence and/or clear pathogenetic mecExtrahepatic Manifestations of Chronic HCV 32315. Extrahepatic Manifestations of ChronicHCVAlbrecht Bôhlig Karl-Philipp Puchner and Thomas BergIntrodu Hepatology: A clinical textbook - Phần 2gM) Polyclonal immunoglobulins (mainly IgG) and monoclonal IgM with rheumatoid factor activity (RF)Type IIIPolyclonal IgG and IgMAutoimmune haemolytic anaemiaGH'D NoncryogloblinaemiclnsuffiencvneuropathiesSB RheumatoidMyocarditisIdiopatic pumlmonary fibrosisarthritiscFigure 1. Schematic representati Hepatology: A clinical textbook - Phần 2on of EHM categories (modified after Zignego 2007a). A) Associations with strong epidemiological evidence and clear pathogenetic mechanisms; B) AssociHepatology: A clinical textbook - Phần 2
ations with high prevalence, but unclear pathogenetic mechanisms; C) Associations for which the high prevalence in HCV collectives could be due to HCVExtrahepatic Manifestations of Chronic HCV 32315. Extrahepatic Manifestations of ChronicHCVAlbrecht Bôhlig Karl-Philipp Puchner and Thomas BergIntrodu Hepatology: A clinical textbook - Phần 2ys. When cryoprecipitate is visible. CG can be purified and characterized using immunofixation electrophoresis. In case of evidence of mixed cryoglobulinemia in HCV-positive patients, cryoglobulinemic syndrome needs to be looked for. Vigilant monitoring is required, as asymptomatic mixed cryoglobuli Hepatology: A clinical textbook - Phần 2nemia patients may develop MC-related disorders in the course of the disease. The diagnosis of the MC syndrome is based on serologic, pathologic and cHepatology: A clinical textbook - Phần 2
linical criteria (Table 3).326 Hepatology 2015Table 3. Diagnostic criteria of cryoglobulinemic syndromeExtrahepatic Manifestations of Chronic HCV 32315. Extrahepatic Manifestations of ChronicHCVAlbrecht Bôhlig Karl-Philipp Puchner and Thomas BergIntroduExtrahepatic Manifestations of Chronic HCV 32315. Extrahepatic Manifestations of ChronicHCVAlbrecht Bôhlig Karl-Philipp Puchner and Thomas BergIntroduGọi ngay
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