Decompensated Cirrhosis as Presentation of LKM1/LC1 Positive Type 2 Autoimmune Hepatitis in Adulthood. A Rare Clinical Entity of Difficult Management

Abstract

Background: Autoimmune hepatitis (AIH) is a chronic and aggressive liver disease that rapidly evolves into cirrhosis and end-stage liver disease if not timely diagnosed and treated with immunosuppressive therapy. AIH is classified into type 1 and type 2 according to the autoantibody pattern, with smooth muscle antibodies and/or antinuclear antibodies as serological markers of AIH-1, while antiliver cytosol antibody type 1 and/or antiliver/kidney microsomal antibody type 1 characterize type 2 AIH, which mainly affects children, including infants, and adolescents. Case Summary: We describe a case of type 2 AIH, clinically onset in a 34-year-old woman with decompensated cirrhosis. Only a thorough analysis of the autoantibody profile allowed for a diagnosis of an AIH-2 evolved into cirrhosis. The patient received a moderate corticosteroid therapy without achieving optimal disease control. We discuss the controversial decision of whether or not to treat the patient with immunosuppressive therapy, which should be balanced with the potential risk of infectious and other complications. A review of the literature on the management of patients with autoimmune cirrhosis is also presented. Conclusions: AIH-2 can be clinically onset in adult patients with cirrhosis and its complications, without being preceded by major clinical signs. Due to the difficult management of cirrhosis with immunosuppressive treatments, a patient-tailored strategy with a case-by-case approach is needed to prevent major complications such as infections, potentially precluding liver transplantation the only curative therapy.

The evolving phenotype of autoimmune hepatitis across the millennium: The 40‐year experience of a referral centre in Italy

“…A possible explanation for this decrease could be the prompt identification and correct treatment of the disease in its early phase during its acute presentation, thus preventing multiple and possibly overlooked episodes of recurrent acute hepatitis 37,38 and thus its progression towards fibrosis and cirrhosis. 39 Conversely, recent epidemiological reports show cirrhosis detected at diagnosis among 34%-35% of patients, 20 a finding suggesting not only a role for the different genetic predisposition in disease expression, but also a delayed access to diagnosis and cure in different countries and populations. [40][41][42] As far immunologic features are concerned, we identified a progressive decline of gamma-globulin and IgG levels at diagnosis over the four decades.…”

“…In parallel with the rise of the acute phenotype, we identified a sharp reduction of cirrhosis at the time of diagnosis, a figure that in the last decade dropped to 8.7%. A possible explanation for this decrease could be the prompt identification and correct treatment of the disease in its early phase during its acute presentation, thus preventing multiple and possibly overlooked episodes of recurrent acute hepatitis 37,38 and thus its progression towards fibrosis and cirrhosis 39 …”

Autoimmune Hepatitis with Acute Presentation: Clinical, Biochemical, and Histological Features of 126 Patients

“…Other second-level serological tests for autoantibodies detection were not done, since they were not available in our country at the time of the study period inclusion. This is an important limitation, since some second-level autoantibodies that can be very helpful for diagnosis, such as anti-LKM1, anti-SLA, and anti-LC1, could be detected only thanks to high-sensitive second-level tests, as recently demonstrated in the case of anti-LC1 antibodies that can be negative by standard indirect immunofluorescence but easily detected by immunoblotting [ 22 ].…”

Identification of Subclasses of Decompensated Cirrhotic Patients with Acute Kidney Injury with Different Responses to Fluid Input

“…We identified liver cirrhosis patients by using the International Classification of Diseases, Ninth Revision, Clinical Modification diagnosis codes. Decompensated liver cirrhosis was defined as the presence or previous history of complications of cirrhosis, namely ascites, jaundice, SBP, hepatopulmonary syndrome, HRS, variceal bleeding, and hepatic encephalopathy [15, 16]. Acute-on-chronic liver failure was defined according to the EF-CLIF criteria [17].…”