We describe a sibship of two patients with type I polyglandular autoimmune syndrome who presented with autoimmune chronic active hepatitis. [ncbi.nlm.nih.gov]

• Inflammation

  Intralobular inflammation and piecemeal necrosis were mild. Three patients were treated with prednisolone and showed rapid clinical and biochemical remission. [ncbi.nlm.nih.gov]

  Interface hepatitis Parenchymal inflammation and necrosis Cirrhosis (in many cases) Portal inflammation: Hallmark of chronic hepatitis, ranges from mild and patchy to prominent and diffuse Lymphocytes are the predominant component, often with variable [pathologyoutlines.com]

  In chronic lobular hepatitis (CLH) there is inflammation of portal tracts, with spotty parenchymal inflammation but no piece-meal necrosis. This pattern of chronic hepatitis is usually associated with a viral aetiology. [somalidoc.com]

  In autoimmune hepatitis, the chronic inflammation resembles inflammation caused by the body attacking its own tissues (an autoimmune reaction ). Autoimmune hepatitis is more common among women than men. [merckmanuals.com]

• Pain

  Always discuss pain with your medical provider, particularly when pain is constant, severe or interferes with your quality of life. Call your medical provider if you have severe or chronic stomach pain. [hepmag.com]

  A biliary colic will present with a colicky RUQ pain yet without fever, leucocytosis, or jaundice. Cholecystitis will present with RUQ pain and fever yet jaundice will be absent. [teachmesurgery.com]

  These include sudden symptoms like: high fever for more than 24 hours chills nausea vomiting back pain pain below the shoulder blades dull pain or cramps in the upper right side sharp or dull pain in the middle of the stomach low blood pressure confusion [healthline.com]

• Fever

  Abstract A 28-year-old man was admitted to our department with intermittent fever, hepatosplenomegaly and pancytopenia. Liver parameters and serum ferritin were markedly elevated. [ncbi.nlm.nih.gov]

  A biliary colic will present with a colicky RUQ pain yet without fever, leucocytosis, or jaundice. Cholecystitis will present with RUQ pain and fever yet jaundice will be absent. [teachmesurgery.com]

  RUQ pain and jaundice (neither sensitive nor specific) Symptoms Fever/chills Nausea/vomiting Abdominal pain Physical Exam RUQ tenderness to palpation Peritoneal signs are variable Jaundice Frank sepsis (fever, tachycardia, hypotension, tachypnea) is [coreem.net]

• Weight Loss

  A 27-year-old woman, para 0-1-2-1, presented at 36 weeks' gestation with a 2-week history of malaise, nausea, emesis, diarrhea, and an 18-lb weight loss. [ncbi.nlm.nih.gov]

  The dog is usually sick by that point in time, and demonstrating the following clinical signs of CAH: vomiting, diarrhea, jaundice (yellow tinge to skin and whites of eyes), weight loss, and fluid accumulation in the abdomen (ascites).Genetic For more [dpca.org]

  Appetite loss: Be sure to discuss this with your health care provider. If you are experiencing significant weight loss, you may be referred to a nutritionist or dietitian. [hepmag.com]

  In early stages, unspecific manifestations such as fever, fatigue, pruritus, malaise, abdominal pain, and weight loss are reported, however about 40-50% of patients may be asymptomatic at time of diagnosis. [orpha.net]

• Asymptomatic

  A rare case of asymptomatic congenital choledochal cyst (Todani Type V) associated with chronic active hepatitis C is reported. [ncbi.nlm.nih.gov]

  […] to symptomatic decompensated cirrhosis Many patients are asymptomatic or have mild nonspecific complaints such as fatigue Physical findings are typically few; may include hepatomegaly or other stigmata of chronic liver disease, such as palmar erythema [pathologyoutlines.com]

  After an incubation period of 1–6 months, most patients present with an asymptomatic or mild inflammation of the liver, which usually resolves spontaneously within a few weeks or months. [amboss.com]

  Okada K, Kamiyama I, Inomata M, Imai M, Miyakawa Y, Mayumi M: e antigen and anti-e in the serum of asymptomatic carrier mothers as indicators of positive and negative transmission of hepatitis B virus to their infants. [link.springer.com]

• Cough

  These cases shared common clinical features: fever, dry cough, dyspnoea, hypoxaemia, diffuse infiltrates both on chest radiography and chest computed tomography, restrictive pulmonary functional impairment, and alveolitis on examination of transbronchial [ncbi.nlm.nih.gov]

  You can't get hepatitis C by touching, kissing, coughing, sneezing, sharing utensils, or breastfeeding. [webmd.com]

  It does not spread through sneezing or coughing. [mayoclinic.org]

  As a result, the following situations will not expose people to the virus: touching, hugging, holding hands being coughed or sneezed on sharing food or drinks breastfeeding The goal of treatment is to rid the body of the hepatitis C virus. [medicalnewstoday.com]

• Pneumonia

  No significant adverse reactions or aggravation of RA or interstitial pneumonia occurred. [ncbi.nlm.nih.gov]

  CXR (pneumonia, GI perforation) Abdominal CT (if unclear diagnosis) MRCP (if unclear diagnosis) DIFFERENTIAL DIAGNOSIS biliary leak cholecystitis hepatic abscess Mirizzi syndrome pancreatitis right lower lobe pneumonia infected choledochal cyst recurrent [litfl.com]

  Pathophysiology <ul><li>The most common organisms cultured from bile in patients with cholangitis include Escherichia coli, Klebsiella pneumoniae, Streptococcus faecalis, and Bacteroides fragilis. </li></ul> 6. [slideshare.net]

  Infections most commonly associated with IRIS include cytomegalovirus, herpes zoster, Mycobacterium avium complex (MAC), Pneumocystis pneumonia, and Mycobacterium tuberculosis (TB). [dermnetnz.org]

• Dyspnea

  We report the case of a 53-year-old female patient, who developed bilateral blepharoptosis, limb weakness, dysphagia, and dyspnea several days after human natural interferon-beta (IFN-beta) treatment for chronic active hepatitis C. [ncbi.nlm.nih.gov]

• Abdominal Pain

  […] stools Darkened urine Fever Jaundice (yellowing of the skin and eyes) Nausea Pain below the shoulder blade Right upper quadrant or middle abdominal pain that is sharp, crampy or dull Vomiting Diagnosis Making a diagnosis of cholangitis as quickly as [verywellhealth.com]

  Presentation 50-70% of patients present with classic Charcot's triad of jaundice, fever and right upper quadrant pain. In elderly patients, the abdominal pain may be poorly localised. [patient.info]

  You can also take an over-the-counter pain reliever, such as ibuprofen (Advil), to help with any abdominal pain you have. See a doctor if your symptoms are severe or seem to be getting worse. [healthline.com]

  […] and jaundice (neither sensitive nor specific) Symptoms Fever/chills Nausea/vomiting Abdominal pain Physical Exam RUQ tenderness to palpation Peritoneal signs are variable Jaundice Frank sepsis (fever, tachycardia, hypotension, tachypnea) is a common [coreem.net]

  These medications can also lead to nausea, diarrhea, constipation, abdominal pain, high blood pressure, elevated cholesterol, joint pain, diabetes, and gingivitis. [rarediseases.org]

• Loss of Appetite

  They include consumption of large amounts of alcohol, loss of appetite, nausea, and vomiting. [merckmanuals.com]

  Symptoms can include: fatigue muscle aches loss of appetite Most symptoms of chronic hepatitis C don’t appear until cirrhosis develops and the liver begins to fail. [healthline.com]

  Several randomized controlled studies established that corticosteroid not only prolong life but decrease the symptoms and signs of fatigue, loss of appetite and fever. [hepatitiscentral.com]

• Diarrhea

  A 27-year-old woman, para 0-1-2-1, presented at 36 weeks' gestation with a 2-week history of malaise, nausea, emesis, diarrhea, and an 18-lb weight loss. [ncbi.nlm.nih.gov]

  The dog is usually sick by that point in time, and demonstrating the following clinical signs of CAH: vomiting, diarrhea, jaundice (yellow tinge to skin and whites of eyes), weight loss, and fluid accumulation in the abdomen (ascites).Genetic For more [dpca.org]

  The most common symptom of inflammatory bowel disease is diarrhea. There is no medical treatment that can slow the progression of PSC. Instead, treatment focuses on decreasing symptoms using a combination of medical therapies and procedures. [newsnetwork.mayoclinic.org]

  These medications can also lead to nausea, diarrhea, constipation, abdominal pain, high blood pressure, elevated cholesterol, joint pain, diabetes, and gingivitis. [rarediseases.org]

  […] and ankles darkening of the skin (hyperpigmentation) muscle pain bone or joint pain bloating (fluid in the stomach area) fat deposits (xanthomas) in the skin around the eyes and eyelids fat deposits in the elbows, knees, palms, and soles of the feet diarrhea [healthline.com]

• Hypertension

  The clinical evaluation of patients before the treatment revealed that three had hypertension, one had a past medical history of myocardial infarction, one had a prosthetic mitral valve replacement and another had left hemiblock in her ECG record. [ncbi.nlm.nih.gov]

  Splenomegaly (portal hypertension). Hirsutism. Investigation of chronic hepatitis Urinalysis : bilirubin and urobilinogen. [patient.info]

  Prevention Working together, you and your doctor can help prevent these specific complications: Increased pressure in the portal vein (portal hypertension). [mayoclinic.org]

  Some patients present with cirrhosis or its complications (eg, portal hypertension). Biopsy is necessary to confirm the diagnosis and to grade and stage the disease. [merckmanuals.com]

• Hypotension

  Factors which increase the mortality rate include delayed diagnosis, liver failure, cirrhosis, CKD, hypotension, female gender, and >50yrs. [teachmesurgery.com]

  Presentation Charcot’s Triad: Fever, RUQ pain and jaundice (neither sensitive nor specific) Symptoms Fever/chills Nausea/vomiting Abdominal pain Physical Exam RUQ tenderness to palpation Peritoneal signs are variable Jaundice Frank sepsis (fever, tachycardia, hypotension [coreem.net]

  Physical signs may include fever, right upper quadrant tenderness, jaundice, mental status changes, hypotension and tachycardia. Peritonism is an unusual sign and should stimulate the search for an alternative diagnosis. [patient.info]

  The following patient characteristics are associated with higher morbidity and mortality rates: Hypotension High malignant strictures Radiologic cholangitis: Post percutaneous transhepatic cholangiography Female gender Age older than 50 years Failure [emedicine.medscape.com]

  […] cholangiogram previous cholangitis immunocompromise malignancy CLINICAL FEATURES History fever (>80%) abdominal pain (especially RUQ) (>80%) nausea and vomiting, malaise predisposing factors and history of an underlying cause Examination tachycardia, hypotension [litfl.com]

• Liver Tenderness

  It starts with acute symptoms in 30% of the cases with jaundice, fever, malaise, liver tenderness, like an acute viral hepatitis. [meddean.luc.edu]

  Clinical signs include liver tenderness, hepatomegaly, and splenomegaly. Acute HBV infection typically lasts two to four months. [aafp.org]

• Arthralgia

  Symptoms Nonspecific symptoms - eg, fatigue, anorexia, muscle pains, arthralgia, weight loss. Right hypochondrial pain (liver distension). Abdominal distension (ascites). Ankle swelling (fluid retention). [patient.info]

  In addition, these patients may have arthralgia, oligomenorrhea, fluctuating jaundice and cushingoid appearance with striae, hirsutism and acne. [hepatitiscentral.com]

  Symptoms of cryoglobulinemia include fatigue, myalgias, arthralgias, neuropathy, glomerulonephritis, and rashes (urticaria, purpura, leukocytoclastic vasculitis); asymptomatic cryoglobulinemia is more common. [merckmanuals.com]

• Pruritus

  In early stages, unspecific manifestations such as fever, fatigue, pruritus, malaise, abdominal pain, and weight loss are reported, however about 40-50% of patients may be asymptomatic at time of diagnosis. [orpha.net]

  Pruritus (cholestasis). Breast swelling (gynaecomastia), testicular atrophy, loss of libido and amenorrhoea due to endocrine dysfunction. Confusion and drowsiness (encephalopathy). [patient.info]

  The patient may also complain of pruritus (itching), as a result of bile accumulation, and pale stool with dark urine, from the obstructive jaundice. [teachmesurgery.com]

• Hirsutism

  Hirsutism. Investigation of chronic hepatitis Urinalysis : bilirubin and urobilinogen. Blood tests: FBC (associated anaemia, thrombocytopenia, raised MCV with alcohol abuse), clotting studies (clotting impairment with hepatic dysfunction). [patient.info]

  In addition, these patients may have arthralgia, oligomenorrhea, fluctuating jaundice and cushingoid appearance with striae, hirsutism and acne. [hepatitiscentral.com]

  Common symptoms include the following: Fatigue Upper abdominal discomfort Mild pruritus Anorexia Myalgia Diarrhea Cushingoid features Arthralgias Skin rashes (including acne) Edema Hirsutism Amenorrhea Chest pain from pleuritis Weight loss and intense [emedicine.medscape.com]

• Night Sweats

  Some early symptoms of chronic cholangitis may include: tiredness and fatigue itchy skin dry eyes dry mouth If you have chronic cholangitis for a long time, you may have: pain in the upper right side night sweats swollen feet and ankles darkening of the [healthline.com]

• Yellow Discoloration of the Skin

  Jaundice (a yellow discoloration of the skin and whites of the eyes caused by deposits of excess bilirubin) is rare unless liver failure develops. [msdmanuals.com]

  discoloration of the skin and the whites of the eyes (jaundice). [labtestsonline.org]

  discoloration of the skin and whites of the eyes (jaundice), poor appetite, vomiting, tiredness, abdominal pain, and diarrhea.[1][2] Hepatitis is acute if it resolves within six months, and chronic if it lasts longer than six months.[1][5] Acute hepatitis [en.wikipedia.org]

• Urticaria

  Extrahepatic presenting findings of chronic active hepatitis may include arthritis, urticaria, pleurisy and pericarditis, while abdominal lymphadenopathy has been only rarely described. [ncbi.nlm.nih.gov]

  Skin signs of acute viral hepatitis Acute urticaria is commonly observed in patients with viral infections, including HAV, HBV and HCV. [dermnetnz.org]

  Symptoms of cryoglobulinemia include fatigue, myalgias, arthralgias, neuropathy, glomerulonephritis, and rashes (urticaria, purpura, leukocytoclastic vasculitis); asymptomatic cryoglobulinemia is more common. [merckmanuals.com]

• Hunger

  Eat small, frequent meals, as stomach pain may be caused or worsened by hunger. If you feel nauseous, try to eat a small portion of food at regular intervals. [hepmag.com]

• Amenorrhea

  In autoimmune hepatitis, especially in young women, manifestations may involve virtually any body system and can include acne, amenorrhea, arthralgia, ulcerative colitis, pulmonary fibrosis, thyroiditis, nephritis, and hemolytic anemia. [merckmanuals.com]

  Common symptoms include the following: Fatigue Upper abdominal discomfort Mild pruritus Anorexia Myalgia Diarrhea Cushingoid features Arthralgias Skin rashes (including acne) Edema Hirsutism Amenorrhea Chest pain from pleuritis Weight loss and intense [emedicine.medscape.com]

  [18] Jaundice can occur as well, but much later in the disease process and is typically a sign of advanced disease.[18] Chronic hepatitis interferes with hormonal functions of the liver which can result in acne, hirsutism (abnormal hair growth), and amenorrhea [en.wikipedia.org]

HBV DNA >2000 copies/mL along with an elevated alanine aminotransferase (ALT) suggests further workup and possible therapy is needed. Viral copies/mL above 10,000 suggest ongoing hepatitis with greater risk for carcinoma. [library.med.utah.edu]

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

• Hepatocellular Carcinoma

  The five of six patients that progressed to hepatocellular carcinoma were associated with cirrhosis. No patients who returned to normal ALT values developed hepatocellular carcinoma during the period of observation. [ncbi.nlm.nih.gov]

Abstract Few alternative treatments are available for those patients with autoimmune chronic active hepatitis who fail to respond to the conventional treatment of corticosteroids. [ncbi.nlm.nih.gov]

Such patients have a poor prognosis and frequently require liver transplantation. We report a patient with autoimmune hepatitis who failed treatment with corticosteroids and azathioprine. [ncbi.nlm.nih.gov]

(c) Corticosterioids may be given. (4) Prognosis. The prognosis varies. When the disease is caused by drugs, signs and symptoms of this type of hepatitis may become less severe. With adequate therapy, the patient usually lives several years. 7-11. [armymedical.tpub.com]

Treatment and prognosis Treatment involves appropriate antibiotic therapy and biliary tree decompression (usually either via ERCP or PTC). Mortality rates are between 50-90% for severe acute cholangitis 5,6. [radiopaedia.org]

Extensive investigations did not establish an etiological diagnosis. Liver histology confirmed the clinically apparent aggressive hepatitis with fibrosis but gave no clue to its etiology. [ncbi.nlm.nih.gov]

However, it is unequivocally heterogenous with regard to etiology. [hepatitiscentral.com]

To present three patients in whom long-term exposure to the drug resulted in chronic active hepatitis; and to review the epidemiology, clinical immunology, histopathology, pathogenetic features and treatment of previously reported cases. [ncbi.nlm.nih.gov]

Reference [ CR ] [ PM ] [ EČ ][ 2 ][ GS ] Alter, M.J. (1997) Epidemiology of hepatitis C. [scindeks.ceon.rs]

Epidemiology References:[1][2][3][4] Epidemiological data refers to the US, unless otherwise specified. Etiology Virus Transmission The frequency and patterns of transmission vary worldwide. [amboss.com]

Summary Epidemiology The prevalence of PSC in Europe ranges from 1/446,000-1/6,170. A male preponderance is observed with a male to female ratio of approximately 2:1. [orpha.net]

El-Serag HB ; Epidemiology of viral hepatitis and hepatocellular carcinoma. Gastroenterology. 2012 May142(6):1264-1273.e1. doi: 10.1053/j.gastro.2011.12.061. [patient.info]

Pathophysiology <ul><li>A scending bacterial infection association with partial or complete obstruction of bile ducts. [slideshare.net]

Mortality rate 5-10% Hepatobiliary Tract Anatomy Pathophysiology Bile duct develops an obstruction Obstruction may be incomplete (more common) or complete Causes: Gallstones (most common), malignancy, benign stricture, iatrogenic (i.e. [coreem.net]

For related pathophysiology, please see the Cholelithiasis and Cholecystitis and Biliary Colic articles. [emedicine.medscape.com]

References:[5][6][7][8] Pathophysiology Acute infection Hepatocytes infected by the hepatitis B virus express viral peptides on their surfaces → lymphocytes recognize HBV-derived peptides and become activated (CD8+ cytotoxic T cells) → lymphocytes attack [amboss.com]

IFN-alpha gave a sustained response in only a small proportion of patients, but worsening of compensated cirrhosis was prevented and development of hepatocellular carcinoma was inhibited, increasing the survival rate. [ncbi.nlm.nih.gov]

Prevention In some cases, preventing cholangitis might not be possible. However, as most cases are caused by gallstones blocking the bile ducts, preventing gallstones may help. [verywellhealth.com]

Vaccination and other prevention measures Vaccination is a safe and effective way of preventing the spread of hepatitis B. [southerncross.co.nz]