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A Case of Autoimmune Hepatitis - pete davidson auto immune disease


A Case of Autoimmune Hepatitis-pete davidson auto immune disease

Proceedings of UCLA Healthcare
-VOLUME 19 (2015)-
CLINICAL VIGNETTE
A Case of Autoimmune Hepatitis
Mary C. Cambou and Adela M. Greeley, M.D.
Case Presentation
A 21-year-old previously healthy male with a two-month autoimmune (including Anti-Liver Kidney Microsomal-1
history of worsening jaundice was sent to the ER for Antibody (Anti-LKM-1)), infectious, and drug-induced tests
aminotransferases elevated to the 2000s. He first noticed skin were negative. An abdominal US with Doppler was significant
yellowing two months earlier. He subsequently developed only for liver span 19.2 cm, without evidence of Budd-Chiari
fatigue, mild abdominal pain (worse on the right side), and syndrome or cirrhosis.
intermittent nausea for one month. Initial evaluation by his
primary doctor revealed mildly elevated aminotransferases, a He remained stable without encephalopathy or bleeding and
negative hepatitis panel, and CT abdomen and abdominal US was discharged after three days with hepatology follow-up.
revealed only mild hepatic inflammation. He was referred to An outpatient transjugular liver biopsy was performed two
an outside gastroenterologist who performed an EGD with weeks later. Pathology was significant for acute hepatitis with
biopsy the day prior to admission. At that time, repeat liver bridging and focal panlobular necrosis, suggestive of
function tests demonstrated markedly elevated transaminases, autoimmune hepatitis or drug-induced liver injury. The
and he was advised to present to the ED immediately for hepatology service started the patient on prednisone 40 mg PO
further evaluation. daily for autoimmune hepatitis.
The patient reported a 15-pound weight loss secondary to Discussion
anorexia and dark urine and loose stools of varying colors. He
denied emesis, fevers, confusion, recent travel, seafood Introduction: Autoimmune hepatitis (AIH) is a relatively
consumption, sick contacts, herbal supplement use, rare, chronic inflammatory process of unknown etiology. AIH
acetaminophen or NSAID use, and EtOH or illicit drug use, is marked by significantly elevated transaminases in a
including mushrooms. He was taking bupropion intermittently hepatocellular pattern, detection of serum antibodies, and
prior to the onset of his symptoms and used marijuana characteristic histologic findings on liver biopsy.1-3 The AIH
occasionally. Vaccinations were up-to-date. Past medical and diagnosis is further divided into two categories, Type 1 and
surgical histories were non-contributory. Per mother, there Type 2, based on antibody profiles, which are typically
was no known family history of hemochromatosis, reflective of the severity of the clinical presentations.1,2 Early
autoimmune disease, hepatocellular carcinoma, or liver diagnosis influences prognosis as untreated AIH can lead to
disease. cirrhosis, fulminant hepatic failure, and hepatocellular
carcinoma.1-3
Temperature was 36.7oC, HR 67, RR 18, BP 132/58, SpO2
96% on room air. Physical exam was significant for scleral Epidemiology and Genetics: The prevalence of AIH is
icterus without Kayser-Fleischer rings, mild distention, and estimated to be approximately 1 in 10,000 among Caucasian
tenderness in the RUQ without rebound or guarding. His liver and Japanese populations.3 Women are at increased risk of
span measured greater than 10 cm, and he had mild AIH, comprising up to 75% of reported cases.1,3 While AIH
splenomegaly. His skin was diffusely jaundiced, without has historically been associated with young women, recent
bronzing or malar rash. Joints were free of erythema or epidemiological research suggests that AIH Type 1 affects all
effusions. The neurologic exam was unremarkable, without ages1,4 with evidence of increased presentation of AIH Type 1
confusion or asterixis. later in life.5 Significant variations in the clinical presentations
of AIH Type 1 and Type 2 exist among different populations
Laboratory findings were significant for AST/ALT 2103/2739 on a global scale, likely secondary to genetic variations in the
IU/L, Conjugated/Total Bilirubin 22.5/25.6 mg/dL, INR 1.7, HLA genes.2,6
and MELD Score 24. ANA was strongly positive (titer 1:320).
Smooth muscle Ab was slightly positive (titer 1:40). Ferritin Type 1 AIH is characterized by the presence of ANA, Anti-
was markedly elevated (9539 ng/mL), Total Serum Iron was Smooth Muscle Antibody (ASMA), Anti-Soluble Liver
mildly elevated (194 ug/dL), TIBC was decreased (25 Gastroenterology. 2010 Jul;139(1):58-72.e4. doi:
times the upper limit of normal), antibody titers 1:80, 10.1053/j.gastro.2010.04.053. Epub 2010 May 5.
histologic confirmation, and absence of another cause. The Review. PubMed PMID: 20451521.
International Autoimmune Hepatitis Group devised two sets of 7. Abdollahi MR, Somi MH, Faraji E. Role of
criteria for AIH diagnosis, an extensive and simplified scoring international criteria in the diagnosis of autoimmune
hepatitis. World J Gastroenterol. 2013 Jun
21;19(23):3629-33. doi: 10.3748/wjg.v19.i23.3629.
PubMed PMID: 23801865; PubMed Central PMCID:
PMC3691026.
Submitted January 8, 2015

What do we know about environmental exposures in autoimmune diseases? Although the study involves populations in rural settings, the exposures being studied – pesticides, solvents, nitrates, metals, 22 Progress in Autoimmune Diseases Research mycotoxins, and silica – are present in other environments, so the results will be applicable to broader populations.