Presentation
We describe a sibship of two patients with type I polyglandular autoimmune syndrome who presented with autoimmune chronic active hepatitis. [ncbi.nlm.nih.gov]
Entire Body System
- 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]
- Ascites
A 65-year-old-woman presented with edema, ascites, proteinuria and abnormal liver function tests. A small amount of mixed cryoglobulin was detected in her serum. [ncbi.nlm.nih.gov]
[…] lobular hepatitis Toxic liver disease with chronic active hepatitis Toxic liver disease with chronic lobular hepatitis Toxic liver disease, ascites, chronic active hepatitis Applies To Chronic hepatitis: active aggressive Recurrent hepatitis ICD-9-CM [icd9data.com]
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]
Portal hypertension : ascites, hypersplenism, lower oesophageal and rectal varices. Hypoalbuminaemia. Coagulopathy. Osteoporosis. [patient.info]
Fluid in your abdomen (ascites). Liver disease can cause large amounts of fluid to accumulate in your abdomen. Ascites can be uncomfortable and may interfere with breathing and is usually a sign of advanced cirrhosis. Liver failure. [mayoclinic.org]
- 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]
- Sepsis
"Sepsis." CDC.gov. 23 Jan 2018. [verywellhealth.com]
(fever, tachycardia, hypotension, tachypnea) is a common presentation Reynold’s Pentad: Charcot’s triad + sepsis and AMS Acute Cholangitis Infographic (mediconet.blogspot.com) References Oyama LC: Disorders of the liver and biliary tract, in Marx JA, [coreem.net]
A pentad may also be seen, in which mental status changes and sepsis are added to the triad. A spectrum of cholangitis exists, ranging from mild symptoms to fulminant overwhelming sepsis. [emedicine.medscape.com]
Complications Patients with severe sepsis can develop liver abscesses and liver failure, bacteraemia and Gram-negative sepsis. [patient.info]
[…] ascending cholangitis Nonspecific cholestasis and acute / chronic inflammation, though ductular cholestasis is highly suggestive of sepsis Board review question #1 Neutrophils filling the lumens of native interlobular bile ducts are most suggestive of [pathologyoutlines.com]
Gastrointestinal
- 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]
Liver, Gall & Pancreas
- Hepatomegaly
[…] features Patients have a wide spectrum of clinical manifestations, from asymptomatic to symptomatic decompensated cirrhosis Many patients are asymptomatic or have mild nonspecific complaints such as fatigue Physical findings are typically few; may include hepatomegaly [pathologyoutlines.com]
Hepatomegaly, splenomegaly, jaundice, portal hypertension and, in more advanced cases, spider angiomata, ascites and muscle atrophy are observed. [orpha.net]
Initial hepatomegaly may be followed by a small liver in well-established cirrhosis. Splenomegaly (portal hypertension). Hirsutism. Investigation of chronic hepatitis Urinalysis : bilirubin and urobilinogen. [patient.info]
</li></ul><ul><li>Mild hepatomegaly </li></ul><ul><li>Peritonitis (uncommon ) </li></ul> 8. [slideshare.net]
- 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]
Musculoskeletal
- 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]
Skin
- 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]
Urogenital
- 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]
Workup
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]
Serum
- Hypoalbuminemia
[…] factors from literature to suggest poor prognosis 10,12,13,14 high fever >39°C shock organ dysfunction advanced age >75 years mental confusion malignancy as etiology medical comorbidities hyperbilirubinemia ≥2.2 mg/dL reduced platelet count <150 × 109/L hypoalbuminemia [radiopaedia.org]
Prognosis depends on the cause of biliary obstruction (neoplasms have poorer prognosis), severity at presentation (Reynolds’ pentad can increase mortality), underlying comorbid factors (cirrhosis with hypoalbuminemia and coagulopathy, malignancy), amenability [cancertherapyadvisor.com]
[…] or the onset of hepatocellular carcinoma as a complication of cirrhosis Seropositive results for antinuclear antibodies (ANAs), smooth-muscle antibodies (SMAs), or liver-kidney microsomal type 1 (LKM-1) or anti–liver cytosol 1 (anti-LC1) antibodies Hypoalbuminemia [emedicine.medscape.com]
For this, the following topics were considered: presence of ascites, encephalopathy, and other conditions, such as telangiectasia, palmar erythema, rarefication of hair in men, jaundice, gynecomastia, flapping, hyperbilirubinemia, hypoalbuminemia, and [ncbi.nlm.nih.gov]
- Hyperglobulinemia
Additionally, some authorities require the presence of immunologic features, particularly high titers (usually more than 1:40) of certain non organic specific autoantibodies and polyclonal hyperglobulinemia. [hepatitiscentral.com]
Biopsy
- 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]
Treatment
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]
Prognosis
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]
Etiology
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]
Epidemiology
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
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]
Prevention
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]