Autoimmune Hepatitis with Elevated Serum IgG4 Levels Have a High Prevalence of Cirrhosis at Diagnosis

Abstract

Background. Some autoimmune hepatitis (AIH) patients have elevated serum IgG4 levels, and the clinical characteristics of such patients are currently incompletely characterized. Aim. To analyze the clinical features and possible pathogenesis of AIH with elevated serum IgG4 levels. Methods. According to their serum IgG4 value, patients were divided into elevated IgG4 (IgG4 > 1.35 g/l) and normal IgG4 (IgG4 ≤ 1.35 g/l) groups. Results. Among the 152 patients included in this study, those in the elevated IgG4 group had the following characteristics: older onset age (56 ± 11.43 years vs. 49.49 ± 13.04 years, P = 0.005 ), higher proportion of males (34.15% vs. 12.61%, P = 0.002 ), higher prevalence of cirrhosis (56.10% vs. 36.04%, P = 0.026 ), lower prevalence of extrahepatic autoimmune diseases (9.76% vs. 27.3%, P = 0.023 ), and higher levels of IL-17 and IL-22 ( P < 0.05 ). Logistic regression analysis results showed that elevated serum IgG4 levels and male sex were risk factors for AIH cirrhosis (male: odds ratio (OR) = 4.293, 95% confidence interval (CI): 1.592–11.575, P = 0.004 ; and elevated serum IgG4: OR = 2.566, 95% CI: 1.065–6.187, P = 0.036 ). No significant differences were found for the remission rate within 6 months between the two groups (69.70% vs. 76.14%, P = 0.470 ). Conclusion. The male proportion and cirrhosis prevalence were higher in AIH with elevated serum IgG4 levels at the time of diagnosis. Male sex and elevated serum IgG4 levels are independent risk factors for AIH cirrhosis, and TH17 cells are more likely involved in the pathogenesis of this type of AIH.

Implications of Gender on the Outcome in Patients With Autoimmune Hepatitis

“…The current study cohort showed a five-year mortality of around 14% without gender predilection (Figure 2). Serum immunoglobulin-4 is associated with higher mortality in patients with autoimmune liver disease, especially in primary sclerosing cholangitis [21,22]. However, its significance in AIH remains to be explored.…”

Pleuritis crónica por enfermedad relacionada con IGG4

“…Por otra parte, cabe destacar que el compromiso pleural puede presentarse de forma aislada o asociado a compromiso de otros órganos, más frecuentemente de origen hepatobiliar, renal, pulmonar y de glándulas salivales 11,12 . En este caso, el paciente se presenta con antecedentes previos de hepatopatía crónica no filiada e insuficiencia renal crónica de causa etiopatogénica poco clara, no pudiéndose descartar su relación con ER-IgG4 en el contexto clínico, serológico y anatomopatológico que se nos plantea 13,14 . Dado que se trata una enfermedad con una gran heterogeneidad clínica, recientemente se han identificado cuatro fenotipos clínicos de ER-IgG4 mediante análisis de clases latentes 11,12 , con el objetivo de ofrecer un marco sistemático que permita facilitar el reconocimiento temprano de la afección, identificar factores de riesgo y desarrollar estrategias de tratamiento personalizadas.…”

Is simultaneous presence of IgG4-positive plasma cells and giant-cell hepatitis a coincidence in autoimmune hepatitis? A case report

“…After a median of 139 mo of long-term observation, the recurrence rate of IgG4-AIH was lower than that of classical AIH. Xue et al [ 15 ] observed 152 patients with AIH. They analyzed 111 patients with AIH in the low IgG4 group (< 135 mg/mL) and 41 patients in the high IgG4 group (> 135 mg/mL) and found that those in the high IgG4 group presented a higher proportion of cirrhosis than those in the low IgG4 group.…”