Liver hemangioma and vascular liver diseases in patients with systemic lupus erythematosus

AIM:To investigate whether systemic lupus erythematosus (SLE) is associated with benign focal liver lesions and vascular liver diseases, since these have been occasionally reported in SLE patients. METHODS:Thirty-five consecutive adult patients with SLE and 35 age-and sex-matched healthy controls were evaluated. Hepatic and portal vein patency and presence of focal liver lesions were studied by colour-Doppler ultrasound, computerized tomography and magnetic resonance were used to refine the diagnosis, clinical data of SLE patients were reviewed. RESULTS:Benign hepatic lesions were common in SLE patients (54% vs 14% controls, P < 0.0001), withhemangioma being the most commonly observed lesion in the two groups. SLE was associated with the presence of single hemangioma [odds ratios (OR) 5.05; 95% confidence interval (CI) 1.91-13.38] and multiple hemangiomas (OR 4.13; 95% CI 1.03-16.55). Multiple hemangiomas were associated with a longer duration of SLE (9.9 ± 6.5 vs 5.5 ± 6.4 years; P = 0.04). Imaging prior to SLE onset was available in 9 patients with SLE and hemangioma, showing absence of lesions in 7/9. The clinical data of our patients suggest that SLE pos- sibly plays a role in the development of hemangioma. In addition, a Budd-Chiari syndrome associated with nodular regenerative hyperplasia (NRH), and a NRH associated with hepatic hemangioma were observed, both in patients hospitalized for abdominal symptoms, suggesting that vascular liver diseases should be specifically investigated in this population. CONCLUSION:SLE is associated with 5-fold increased odds of liver hemangiomas, suggesting that these might be considered among the hepatic manifestations of SLE.

Abatacept is effective in Chinese patients with LRBA and CTLA4 deficiency

CTLA4 deficiency and LRBA deficiency are a group disorders of immune dysregula-tion that affect CTLA4 pathway.The patients mainly present with autoimmunity,antibody defi-ciency and recurrent infections.Here we reported three Chinese patients with LRBA and CTLA4 mutations.They all presented with chronic diarrhea,hypokalemia,organomegaly,recurrent in-fections,and hypogammaglobulinemia.Reduced Treg cells and increased percentage of circu-lating follicular helper T(cTfh)cells were revealed in these patients.Although steroid and immunoglobulin therapy were given,the enteropathy was persistent.Therefore,abatacept treatment was provided to these patients.They showed a marked improvement of enteropathy and gastrointestinal endoscopy showed alleviated inflammatory lesion and follicular hyperpla-sia.Furthermore,the frequency of cTfh cells was reduced after abatacept therapy.Taken together,targeted therapy with abatacept is a promising treatment modality for patients with LRBA and CTLA4 deficiency.The findings also suggest that the frequency of cTfh cells could serve as a marker for tracking disease activity and the response to abatacept therapy.