Background and aim: There is a clinical subgroup of patients with a combination of autoimmune liver disease, an “overlap”, with features of cholestasis (CBP or CEP) in combination with HAI. Its management is relevant since, without an adequate treatment, these patients are in increased risk for developing cirrhosis and liver failure, have lower therapeutic response and their prognosis is worse than those with isolated autoimmune hepatitis, their behavior tends to be more aggressive with higher rates of cirrhosis and need for liver transplantation. Aim: To describe the clinical and biochemical characteristics and the natural history of a cohort of patients with overlap syndrome at Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán in the period January 2008 - December 2018.
Material and methods: Clinical, radiologic and histopathological records of patients with overlap syndrome were reviewed. Variables included; diagnosis (CBP / HAI or CEP / HAI), date of diagnosis, symptoms, laboratory values, metabolic comorbidities, other autoimmune diseases, characteristics of liver biopsy, time-associated variables: development of cirrhosis, decompensation, death, liver transplantation, biochemical response and relapse.
Results: Fifty patients were included in the study, 90% were women, mean age was 43.2 years (SD 11.0). The predominant comorbidity was arterial hypertension; fatigue and pruritus were the main symptoms; an important association with thyroid disease was evident, antinuclear antibodies were serologically observed in 100% of cases and mitochondrial antibodies in 57%. According to International's group criteria 14 were definitive cases and 32 were probable, with Paris criteria 33 were diagnosed. The most common outcomes were cirrhosis (50%) and disease relapse (60%). The median progression to cirrhosis was 73 months, survival and decompensation medians were not available. The percentage of biochemical response for CBP was 35.4% and for HAI 47%.
Conclusions: Overlap Syndrome at Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán predominantly affects middle-aged women with autoimmune comorbidities, the most common outcomes were cirrhosis and disease relapse.
Conflicts of interest: The authors have no conflicts of interest to declare.
