Autoimmune liver disease (primary biliary cholangitis/autoimmune hepatitis-overlap) associated with sarcoidosis (clinical cases and literature review)

Abstract

Clinical features of overlap autoimmune hepatitis/primary biliary cholangitis and morphological-proved sarcoid lesions (lungs, lymph nodes, skin) were performed. Data of long-term clinical observation presented in comparison with the results of laboratory datas, instrumental and morphological studies of liver tissue, lungs, skin. The modern aspects of pathogenesis of association autoimmune and granulomatous diseases arediscussed on the example of clinical cases of combination of cholestatic variants of autoimmune hepatitis and generalized sarcoidosis.

A rare coexistence of sarcoidosis with overlap syndrome and sarcoidosis with primary biliary cholangitis and Sjogren's syndrome: Two distinct case reports

“…In 10–25 % of patients with sarcoidosis, hepatic manifestations can lead to occasional elevation of aminotransferase levels, less frequently cholestatic markers, and up to 20 % may present with hepatosplenomegaly. Signs of liver cirrhosis related to granulomatous lesions with severe impairment of liver functions are rare [ 10 ].…”

“…Nevertheless, while suggestions have been put forth regarding several potential mechanisms, research in this field remains limited to this day. On one hand, in the case of sarcoidosis combined with the overlap PBC/AIH, it can be envisaged that non-immune biliary necrosis of hepatocytes, leading to the release of hepatic autoantigens, could trigger pathological changes in the lungs and liver, especially in the presence of genetic predisposition [ 10 ]. On the other hand, sarcoidosis is a systemic inflammatory disease that can affect various organs, including the liver.…”

“…The criteria have shown efficacy in identifying patients who have undergone corticosteroid treatment for the inflammatory component of their disease, demonstrating high specificity but moderate sensitivity. These criteria align with the treatment indications for AIH as defined in the current American Association for the Study of Liver Diseases (AASLD) practice guidelines [ 10 ].…”

Mechanism of TGF‑β1 inhibiting Kupffer cell immune responses in cholestatic cirrhosis

“…Inflammatory responses are often found in the process of fibrosis (6) as an increase in the levels of inflammatory cytokines and a decrease in anti-inflammatory cytokines (7,8). Macrophages are multifunctional immune cells in the innate immune system, which remove apoptotic cells by phagocytose, present antigens, and produce pro-inflammatory cytokines and chemokines, such as interleukin-1β (IL-1β), IL-6, and tumor necrosis factor-α (TNF-α) (9,10).…”