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Liver disease associated with anti-liver-kidney microsome antibody in children
Insights
Autoimmune liver disease in children, marked by anti-LKM antibodies, presents varied symptoms and often co-occurs with other autoimmune conditions. Early immunosuppressive treatment is crucial for managing this potentially fatal disease.
Area of Science:
- Pediatric autoimmune liver disease
- Hepatology
- Immunology
Background:
- Autoimmune hepatitis can affect children, presenting with diverse clinical symptoms.
- High titers of anti-liver-kidney microsome antibody (anti-LKM) are characteristic of this condition.
Purpose of the Study:
- To describe the clinical presentation, associated conditions, and treatment outcomes of pediatric inflammatory liver disease linked to anti-LKM antibodies.
Main Methods:
- Retrospective analysis of 20 children diagnosed with anti-LKM antibody-associated inflammatory liver disease over 10 years.
- Evaluation of clinical symptoms, laboratory findings, histological data, extrahepatic manifestations, and treatment responses.
Main Results:
- Common symptoms included fatigue, jaundice, hepatomegaly, and splenomegaly.
- Cirrhosis was prevalent (19/20 children), with 11 exhibiting extrahepatic autoimmune diseases (e.g., type 1 diabetes, autoimmune thyroiditis).
- Immunosuppressive therapy (prednisone and azathioprine) improved liver function in 16/18 patients, though relapses occurred upon discontinuation.
Conclusions:
- Pediatric anti-LKM antibody-positive autoimmune liver disease has varied presentations and is frequently associated with other autoimmune disorders.
- Early initiation of immunosuppressive therapy is vital for managing this potentially life-threatening condition.
- Long-term treatment is often necessary to maintain stable hepatic function.
Abstract:
In the past 10 years we have examined 20 children with inflammatory liver disease associated with high serum titers of anti-liver-kidney microsome antibody (anti-LKM). The first hepatic symptoms were progressive fatigue and jaundice, the fortuitous finding of hepatomegaly or splenomegaly with raised transaminase activity, or an acute hepatitis-like illness. At the time of diagnosis, hepatomegaly was present in 18 children, splenomegaly in 16, jaundice in nine, and ascites in two. Serum alanine transferase activities were elevated in all but two, who had already received steroids. Serum total gammaglobulin values were greater than 2.0 gm/dl in 16 children, prothrombin activity less than or equal to 60% in six, and serum titer of anti-LKM between 1:100 and 1:100,000. All children but one had cirrhosis, and histologic signs of aggressivity were present in 14. In 11 children one or more extrahepatic diseases were present, including type 1 diabetes, vitiligo, glomerulonephritis, autoimmune hemolytic anemia, hypoglycemia with hyperinsulinism, autoimmune thyroiditis, chronic mucocutaneous candidiasis with hypoparathyroidism, and multiple cutaneous and visceral telangiectasias. Treatment with prednisone and azathioprine improved the liver condition in 16 of the 18 patients given treatment. In eight of them discontinuation of treatment resulted in rapid relapse; 14 are still receiving treatment and have stable hepatic function with follow-up from 8 months to 6 1/2 years. Only two are free of treatment. Four children died, two in spite of immunosuppressive therapy, one during a relapse, and one of extrahepatic disease. These results indicate that this autoimmune inflammatory liver disease may have onset early in life, with several clinical patterns; is frequently associated with certain types of extrahepatic manifestations of autoimmune origin; and is a potentially fatal disease for which immunosuppressive treatment must be started early.