The diagnoses at autopsy except for systemic fibrinoid necrosis were cholestatic liver damage, acute renal swelling, acute pancreatitis, slight cardiac hypertrophy (350 g) and latent adenocarcinoma of the prostate
The diagnoses at autopsy except for systemic fibrinoid necrosis were cholestatic liver damage, acute renal swelling, acute pancreatitis, slight cardiac hypertrophy (350 g) and latent adenocarcinoma of the prostate. immunological derangement might be involved in pathogenesis of fibrinoid necrosis. However, the true etiology remains unfamiliar. strong class=”kwd-title” Keywords: Jaundice, Fibrinoid necrosis, Cholangio-graphy, Main sclerosing cholangitis, Liver, Autopsy Intro We herein present an unusual autopsy case showing systemic fibrinoid necroses in various organs. The case was strongly suspected clinically of main sclerosing cholangitis (PSC) based on the cholangiographic findings; however, the autopsy disclosed systemic fibrinoid necroses instead of histological features of PSC. Particularly noteworthy was the presence of fibrinoid necrosis in the portal tracts of the liver caused stenoses of the biliary trees in association with cholestasis. We AMG 837 calcium hydrate could not find any related case reported in the literature. Presently, the etiology remains unknown. CASE Statement About 10 mo before admission to our hospital, an 81-year-old Japanese man was admitted to a local hospital because of jaundice. Laboratory examinations revealed elevated levels of serum total bilirubin and additional serum enzymes such as alkaline phosphatase, -glutamyltranspeptidase, leucine aminopeptidase. Serum autoantibodies such as anti nuclear antibody, anti mitochondrial antibody and anti clean muscle mass antibody were bad. Endoscopical retrograde cholangiogram showed characteristic irregularity and beading of the intrahepatic biliary tree (Number ?(Figure1).1). No extrahepatic involvement was found. The patient was strongly suspected clinically of PSC. The pathologic analysis of the liver biopsy specimen was liver damage with cholestasis. Concentric fibrosis round the bile ducts suggesting PSC was not found. The patient was transferred to our hospital 18 d before death because of aggravation of jaundice, anorexia and easy fatigability. Chronological Itga4 laboratory data is demonstrated in Table ?Table1.1. Serum fibrinogen was within normal lower limit. Plasminogen and D-dimer in serum were not examined. Serologic markers for hepatits B and C viral hepatitis were negative. These findings indicated the liver cell damage rapidly worsened with aggravation of cholestasis, especially in end stage. Despite symptomatic and supportive treatment, the jaundice became gradually worse, and ascites and hepatic encephalopathy developed. The patient eventually died of hepatic failure. Table 1 Laboratory data thead align=”center” d 1d 38d 48d 54 /thead em Peripheral blood /em em Serological checks /em WBC/L(4500-8500)84008800920016900IgGmg/dL(800-1800)1629Hemoglobing/dL(13.5-16.5)12.112.911.511.4IgAmg/dL(130-290)182Platelet 104/L(15.0-35.0)26.826.620.818.6IgMmg/dL(100-180)68C3mg/dL(70-100)99 em Coagulation checks /em C4mg/dL(11-44)46.5Prothrombin time%( 70%)55375226CH50U/mL(28-45)44.6Hepaplastin test%( 70%)90445738Serum-Cug/dL(78-131)247Fibrinogenmg/dL(150-400)158176160 em Viral markers /em em Blood chemistry /em HBsAg(-)ASTIU/L(10-30)18513498422HCVAb(-)ALTIU/L(6-40)1269159174LDHIU/L(160-325)450646439812 em Auto antibodies /em ALPIU/L(120-400)1359834692556Anti nucleic antibody(-)LAPIU/L(105-235)528472425LE test(-)-GTPIU/L(4-70)29615111973Anti DNA antibody(-)T-Bilmg/dL(0.1-0.8)10.715.816.218.5Anti SMA antibody(-)D-Bilmg/dL(0-0.3)59.110.9Anti mitochondrion antibody(-)TPg/dL(6.7-8.3)6.46.35.24.9Albg/dL(3.5-5.2)3.32.82.82.6 em Tumor markers /em TCmg/dL(120-220)260319193158AFPng/mL(1-15)2BUNmg/dL(8-20)3268150CEAng/mL(5.8)6.1Crmg/dL(0.5-1.1)1.21.33.17.7CA19-9U/mL(37)2470 em Serological test /em mg/dLCRP(0-0.5)1.81.31.58.8 Open in a separate window D: hospital day; Underline: AMG 837 calcium hydrate irregular value. Open in a separate window Number 1 Endoscopic retrograde cholangiogram. Intrahepatic biliary branches showed irregularity and considerable beading (arrows). The long arrow shows the dilatation of the bile duct due to stenosis caused by the pressure of cysts. An autopsy was performed about two hours after his death. The diagnoses at autopsy except for systemic fibrinoid necrosis were cholestatic liver damage, acute renal swelling, acute pancreatitis, slight cardiac hypertrophy (350 g) and latent adenocarcinoma of the prostate. Pathologic findings concerning fibrinoid necrosis are described as follows. The liver (1380 g) showed a deeply yellow-brown cut-surface due to cholestasis with spread simple small cysts. Gross exam could not detect remarkable changes of the common bile duct and bilateral hepatic ducts. Microscopically probably the most stunning feature was a massive deposition of homogeneous material stained scarlet with Masson’s trichrome in the portal tracts (Number ?(Figure2).2). Careful examinations disclosed the material was deposited in the walls of arteries and portal veins as well as with the connective cells (Number ?(Figure3).3). In the bile ducts the deposits circumscribed the lumen leaving the epithelial lining undamaged and caused designated stenosis. The area of the involved vessels and ducts prolonged from your distal interlobular portion to the proximal septal portion (Numbers ?(Numbers22 and ?and3).3). The material was stained violet with PTAH and immunohistochemically was positive for fibrinogen and bad for immunoglobulins such as IgG, IgM, and IgA, C3 and C1q (Number ?(Figure4A).4A). Electron microscopic findings of the liver were electron dense materials deposited in the involved tissue (Number ?(Number4B).4B). The results of these stainings and electronmicrogram suggested the lesion with homogeneous material deposition was fibrinoid necrosis. In addition, the portal tracts were enlarged and infiltrated with lymphocytes and plasma cells with an occasional intermingling of polymorphonuclear cells, ductular proliferation and fibrosis. The histologic features were biliary interface activity, resulting from cholestasis. However, it was mentioned the lesion with fibrinoid necrosis was scarcely accompanied by inflammatory reactions. Cholestasis also caused conspicuous paren-chymal damage including feathery degeneration or necrosis. Even though liver was extensively examined histologically, there were no detectable features of PSC. Open in AMG 837 calcium hydrate a separate window Number 2 Considerable fibrinoid necrosis in large portal tract. Septal bile duct is definitely buried in the fibrinoid material (Massons trichrome x 1). PT: portal tract, BD: bile duct. Open in a separate window Number 3 A smaller portal tract. Fibrinoid material is deposited in the.