Our aim was to investigate the prevalence and clinical relevance of inherited complement and antibody deficiency states in a large series of patients with various autoimmune rheumatologic diseases (ARD) with juvenile onset. A total number of 117 consecutive patients from 2 tertiary referral hospitals were included in the study. All patients underwent genetic screening for type I C2 deficiency and C4 allotyping. Serum levels of immunoglobulin classes measured systematically throughout their regular medical care were recorded retrospectively. Our cohort of patients included 84 with juvenile idiopathic arthritis (JIA), 21 with systemic lupus erythematosus (SLE), 6 with systemic vasculitis, 2 with juvenile scleroderma, 2 with idiopathic uveitis, 1 with mixed connective tissue disease and 1 with SLE/scleroderma overlap syndrome. We have found 16 patients with evidence of primary immunodeficiency in our series (13.7 %), including 7 with C4 deficiency, 5 with selective IgA deficiency, 3 with C2 deficiency and 2 with unclassified hypogammaglobulinemia (one also presented C4D). Of the 84 patients with JIA, 4 (4.8 %) had a complement deficiency, which was less prevalent than in the SLE cohort (23.8 %), but all of them have exhibited an aggressive disease. Most of our patients with primary antibody deficiencies showed a more complicated and severe disease course and even the co-occurrence of two associated autoimmune diseases (SLE/scleroderma overlap syndrome and SLE/autoimmune hepatitis type 1 overlap). Our findings among others demonstrate that complement and immunoglobulin immunodeficiencies need careful consideration in patients with ARD, as they are common and might contribute to a more severe clinical course of the disease.
Hepatocellular carcinoma (HCC) is probably the epitome of a screening target, with a well-defined high-risk population, accessible screening methods, and multiple curative-intent treatments available for early disease. Per major societies guideline consensus, biannual ultrasound (US) surveillance of the at-risk patients is the current standard of care worldwide. Yet, despite its documented success in the past decades, this standard is far from perfect. While the whole community is working to further tighten the knots, a worrying number of cases still slip through this safety net. Consequently, these patients lose their chance to a curative solution which leads to a high disease burden with disproportionate mortality. While US will probably remain the fundamental staple in the screening strategy, key questions are seeking better answers. How can its caveats be addressed, and the technique be improved? When are further steps needed? How to increase accuracy without giving up on accessibility? This narrative review discusses the place of US surveillance in the bigger HCC picture, trying to navigate through its strengths and limits based on the most recent available evidence.
Aim: Percutaneous radiofrequency (RFA) and microwave ablation (MWA) are currently the best treatment options forpatients with liver metastases (LM) who cannot undergo a liver resection procedure. Presently, few studies have evaluated theefficacy of tumor ablation in beginner’s hands but none at all in hepatic metastasis. Our aim was to report the initial experiencewith ultrasound as a tool to guide tumor ablation in a low volume center with no experience in tumor ablation.Material and methods: We conducted a retrospective cohort study, on a series of 61 patients who had undergone percutaneous US-guided ablations for 82 LM between 2010 and 2015. Long term outcome predictors were assessed using univariate and multivariate analysis.Results: Complete ablation was achieved in 86.9% of cases (53/61). All MWA sessions (20/20) attained ablation margins >5mm, compared to 79% (49/62) for RFA sessions (p=0.031). Ablation time was significantly shorter for MWA, with a median duration of 10 minutes (range: 6-12) vs. 14 minutes (range: 10-19.5, p=0.003). There was no statistically significant difference in local tumor progression (LTP)-free survival rates between MWA and RFA (p=0.154). On univariate analysis, significant predictors for local recurrence were multiple metastases (p=0.013) and ablation margins <5 mm (p<.001), both retaining significance on multivariate analysis. Significant predictors for distant recurrence on both univariate and multivariate analysis were multiple metastases (p<0.001) and non-colorectal cancer metastases (p<0.05).Conclusion: A larger than 5 mm ablation size is critical for local tumor control. We favor the use of MWA due to its ability to achieve ablation in significantlyshorter times with less incomplete ablations.