Autoimmune liver diseases in systemic rheumatic diseases

Abstract

Systemic rheumatic diseases (SRDs) are chronic, inflammatory, autoimmune disorders with the presence of autoantibodies that may affect any organ or system. Liver dysfunction in SRDs can be associated with prescribed drugs, viral hepatitis, alternative hepatic comorbidities and coexisting autoimmune liver diseases (AILDs), requiring an exclusion of secondary conditions before considering liver involvement. The patterns of overlap diseases depend predominantly on genetic determinants with common susceptible loci widely distributing in both disorders. In AILDs, it is important to identify the overlapping SRDs at an early stage since such a coexistence may influence the disease course and prognosis. Commonly co-occurring SRDs in AILDs are Sjögren syndrome (SS), rheumatoid arthritis (RA) or systemic lupus erythematosus (SLE) in autoimmune hepatitis (AIH), and SS, RA or systemic sclerosis in primary biliary cholangitis. Owing to different disease complications and therapies, it is imperative to differentiate between SLE liver involvement and SLE-AIH overlap disease. Therapeutic options can be personalized to control coexisting conditions of liver autoimmunity and rheumatic manifestations in AILD-SRD overlap diseases. The collaboration between hepatologists and rheumatologists can lead to significant advances in managing such a complex scenario. In this review, we provide a comprehensive overview on coexisting AILDs in different SRDs and the therapeutic approach in managing these overlap diseases.

Keywords: Autoimmune liver disease; Drug-induced liver injury; Liver function test; Overlap disease; Systemic rheumatic disease; Viral hepatitis.

Figure 1

Liver biopsied tissues from a patient with systemic lupus erythematosus liver involvement (lupus hepatitis). The portal area with minimal non-specific lymphocytic infiltration is shown. Hematoxylin and eosin staining, 400 × magnification.

Figure 2

Liver biopsied tissues from 2 patients with systemic lupus erythematosus-autoimmune hepatitis overlap disease. A and B: Case 1 (A) lymphoplasmacytic infiltration with interface activity and (B) lymphoplasmacytic infiltration with interface activity. Plasma cells are indicated by blue arrows and rosette formations by black arrows; C and D: Case 2 (C) lymphoplasmacytic infiltration with interface activity, and (D) rosette formation (arrows). Hematoxylin and eosin staining, 400 × magnification.