Blau syndrome with hypertension and hepatic granulomas: a case report and literature review.

Background: Blau syndrome (BS) is a monogenic disorder caused by NOD2 gene variants characterized by the triad of granulomatous polyarthritis, rash, and uveitis. Atypical symptoms were recognized in one-third to one-half of individuals with BS. This study aims to describe the clinical features of BS patients with hypertension and digestive system involvement. Methods: The complete clinical data of a BS patient complicated with hypertension and hepatic granulomas were collected and documented. We also performed a literature search to find all reported cases of BS with hypertension and digestive system involvement. Results: We reported the case of a 19-year-old man who presented with early onset symmetric polyarthritis and hypertension at age 5 and hepatic granulomas and cirrhosis at age 19. He was diagnosed with BS by the finding of a variant of the NOD2 gene (R334W). Through the literature review, 24 patients with BS were found who were reported to have hypertension, and 38 patients were found who had different digestive system manifestations such as hepatic granulomas, hepatosplenomegaly, diverticulitis, and intestinal granuloma. Among the 38 BS patients with digestive system involvement, 14 had hepatic granulomas proven by liver biopsy. Conclusions: Hypertension and digestive system involvement are rare manifestations of BS. Clinicians, especially rheumatologists, must be aware of atypical symptoms of BS.

Influence of Iguratimod on Bone Metabolism in Patients with Rheumatoid Arthritis: A Meta-analysis.

Background: Influence of iguratimod on bone mineral density (BMD) and biomarkers of bone metabolism in patients with rheumatoid arthritis (RA) remains not determined. Accordingly, a meta-analysis was performed for systematical evaluation. Methods: Relevant randomized controlled trials (RCTs) were retrieved by searching of PubMed, Embase, Cochrane's Library, China National Knowledge Infrastructure (CNKI), and Wanfang databases. A random-effect model was used to pool the results. Results: In total, 24 RCTs including 2439 patients with RA contributed to the meta-analysis. Pooled results showed that compared to methotrexate alone, additional use of iguratimod 25 mg Bid for 12∼24 weeks significantly improved lumbar-spine BMD (mean difference [MD]: 0.12, 95% confidence interval [CI]: 0.04 to 0.20, p=0.002, I 2 = 39%) in patients with RA. Moreover, treatment with iguratimod was associated with increased serum osteoprotegerin (MD: 180.36 pg/ml, 95% CI: 122.52 to 238.20, p < 0.001, I 2 = 48%), and decreased serum receptor activator for nuclear factor kappa-B ligand (MD: -10.65 pmol/l, 95% CI: -15.59 to -5.72, p < 0.001, I 2 = 53%). In addition, iguratimod was associated with increased bone formation markers such as the serum N-terminal middle molecular fragment of osteocalcin (MD: 4.23 ng/ml, 95% CI: 3.74 to 4.71, p < 0.001, I 2 = 35%) and total procollagen type I amino-terminal propeptide (MD: 9.10 ng/ml, 95% CI: 7.39 to 10.80, p < 0.001, I 2 = 86%), but decreased the bone resorption marker such as serum ß-C terminal cross-linking telopeptide of type 1 collagen (MD: -0.18 pg/ml, 95% CI: -0.21 to -0.14, p < 0.001, I 2 = 70%). Conclusions: Iguratimod could prevent the bone loss and improve the bone metabolism in patients with RA.

Cost-effectiveness of Anbainuo plus methotrexate compared to conventional disease-modifying antirheumatic drugs for rheumatoid arthritis patients in China.

BACKGROUND: This study aimed to evaluate the cost-effectiveness of Anbainuo (ABN) plus methotrexate (MTX) vs. conventional disease-modifying antirheumatic drugs (cDMARDs) in Chinese rheumatoid arthritis (RA) patients. METHODS: A total of 90 RA patients who underwent ABN + MTX [assigned as ABN + MTX group (n=47)] or cDMARDs [assigned as control group (n=43)] treatment were analyzed. Disease activity was assessed at baseline (M0), 3rd month (M3), 6th month (M6), and 12th month (M12) after treatment. Drug, other medical, indirect, and total costs were calculated. Then, pharmacoeconomic analyses were performed with the threshold of cost-effectiveness set as 3 times of the mean gross domestic product (GDP) per capita in China during the study period. RESULTS: Treatment response rate was similar between the 2 groups, while disease remission and low disease activity (LDA) rates were increased in the ABN + MTX group compared to control group. Drug cost, other medical costs, and total cost were higher in the ABN + MTX group than control group, while indirect cost was similar between the 2 groups. Meanwhile, the quality-adjusted life-years (QALY) in ABN + MTX group and control group were 0.72 and 0.48 years, respectively. The incremental cost-effectiveness ratios (ICER) of ABN + MTX group compared to control group among the entire participant cohort, moderate-disease-activity participants, and severe-disease-activity participants were ¥135,486.7, ¥146,450.4, and ¥124,987.2/QALY, respectively, which were all below the cost-effectiveness threshold. Further sensitivity analyses revealed that the cost-effectiveness of ABN + MTX vs. cDMARDs was relatively robust, while among all the indexes, ABN price and Health Assessment Questionnaire Disability Index (HAQ-DI) score change for the ABN + MTX group affected ICER most. CONCLUSIONS: Treatment with ABN + MTX offers acceptable cost-effectiveness compared to cDMARDs treatment in Chinese RA patients.

Association Study of Single-Nucleotide Polymorphisms of STAT2/STAT3/IFN-γ Genes in Cervical Cancer in Southern Chinese Han Women.

OBJECTIVE: Interferon-γ (IFN-γ) and signal transducers and activators of transcription (STATs) each play an important role in carcinogenesis associated with viral infection. Cervical cancer is almost invariably associated with infection by human papillomavirus (HPV), and previous studies suggested that dysregulation of the signal pathway involved in IFN-γ and STATs is associated. Our objective was to evaluate the association of SNPs in STAT2, STAT3, and IFN-γ with cervical cancer susceptibility in Chinese Han women in Hunan province. MATERIALS AND METHODS: Genomic DNA was extracted from peripheral blood samples of 234 cervical cancer patients and 216 healthy female controls. STAT2 and STAT3 genotyping was performed using polymerase chain reaction-restriction enzyme (PCR-RE) analysis. IFN-γ genotyping was detected by PCR-amplification of specific allele (PASA). RESULTS: For STAT2 rs2066807 polymorphisms, there was no significant difference of genotype distribution (P=0.827) and allele frequencies (P=0.830, OR=1.09, 95% CI: 0.51-2.31) between cases and controls. For STAT3 rs957970 polymorphisms, there was no significant difference of genotype distribution (P=0.455) and allele frequencies (P=0.560, OR=0.92, 95% CI: 0.71-1.20) between cases and controls. For IFN-γ +874A/T polymorphisms, there was no significant difference of genotype distribution (P=0.652) and allele frequencies (P=0.527, OR=1.12, 95% CI: 0.79-1.59) between cases and controls. CONCLUSION: These results suggest that polymorphisms in STAT2, STAT3 and IFN-γ genes are not likely to be strong predictors of cervical cancer in Han women in southern China.