Cryoglobulinemic vasculitis in primary Sj\uf6gren's Syndrome: Clinical presentation, association with lymphoma and comparison with Hepatitis C-related disease

Objective: To describe the clinical spectrum of cryoglobulinemic vasculitis (CV) in primary Sj\uf6gren's syndrome (pSS), investigate its relation to lymphoma and identify the differences with hepatitis C virus (HCV) related CV. Methods: From a multicentre study population of consecutive pSS patients, those who had been evaluated for cryoglobulins and fulfilled the 2011 classification criteria for CV were identified retrospectively. pSS-CV patients were matched with pSS patients without cryoglobulins (1:2) and HCV-CV patients (1:1). Clinical, laboratory and outcome features were analyzed. A data driven logistic regression model was applied for pSS-CV patients and their pSS cryoglobulin negative controls to identify independent features associated with lymphoma. Results: 1083 pSS patients were tested for cryoglobulins. 115 (10.6%) had cryoglobulinemia and 71 (6.5%) fulfilled the classification criteria for CV. pSS-CV patients had higher frequency of extraglandular manifestations and lymphoma (OR=9.87, 95% CI: 4.7\u201320.9) compared to pSS patients without cryoglobulins. Purpura was the commonest vasculitic manifestation (90%), presenting at disease onset in 39% of patients. One third of pSS-CV patients developed B-cell lymphoma within the first 5 years of CV course, with cryoglobulinemia being the strongest independent lymphoma associated feature. Compared to HCV-CV patients, pSS-CV individuals displayed more frequently lymphadenopathy, type II IgMk cryoglobulins and lymphoma (OR = 6.12, 95% CI: 2.7\u201314.4) and less frequently C4 hypocomplementemia and peripheral neuropathy. Conclusion: pSS-CV has a severe clinical course, overshadowing the typical clinical manifestations of pSS and higher risk for early lymphoma development compared to HCV related CV. Though infrequent, pSS-CV constitutes a distinct severe clinical phenotype of pSS

Lack of replication of higher genetic risk load in men than in women with systemic lupus erythematosus

Introduction: We aimed to replicate a recent study which showed higher genetic risk load at 15 loci in men than in women with systemic lupus erythematosus (SLE). This difference was very significant, and it was interpreted as indicating that men require more genetic susceptibility than women to develop SLE. Methods: Nineteen SLE-associated loci (thirteen of which are shared with the previous study) were analyzed in 1,457 SLE patients and 1,728 healthy controls of European ancestry. Genetic risk load was calculated as sex-specific sum genetic risk scores (GRS(s)). Results: Our results did not replicate those of the previous study at either the level of individual loci or the global level of GRS(s). GRS(s) were larger in women than in men (4.20 ± 1.07 in women vs. 3.27 ± 0.98 in men). This very significant difference (P < 10(-16)) was more dependent on the six new loci not included in the previous study (59% of the difference) than on the thirteen loci that are shared (the remaining 41%). However, the 13 shared loci also showed a higher genetic risk load in women than in men in our study (P = 6.6 × 10(-7)), suggesting that heterogeneity of participants, in addition to different loci, contributed to the opposite results. Conclusion: Our results show the lack of a clear trend toward higher genetic risk in one of the sexes for the analyzed SLE loci. They also highlight several limitations of assessments of genetic risk load, including the possibility of ascertainment bias with loci discovered in studies that have included mainly women

Nutritional risk factors for iron-deficiency anaemia in children 12-24 months old in the area of Thessalia in Greece

Iron-deficiency anaemia (IDA) is a common problem all over the world, which mainly attacks pregnant women, infants and children. The main objectives were to assess the prevalence of IDA in children 12-24 months old in the area of Thessalia located in the central part of Greece and to identify, by means of a simple questionnaire, its nutritional risk factors. The research was applied as a cross-sectional and case-control study. In the first part of the study, the haemoglobin (Hb) levels were estimated by a mobile photometer analyser in 938 children (approximately one-third of the total children population). In the second part, children with Hb &lt; 11 g/dl were compared with their random selected healthy controls in haematological, anthropometric and nutritional parameters. The estimated laboratory values were Hb, haematocrit, mean corpuscular volume, mean corpuscular Hb, mean corpuscular Hb concentration, zinc protoporphyrin, serum iron, serum ferritin, transferring saturation, total iron binding capacity and Hb electrophoresis. Finally, 75 children (34 boys, 41 girls; mean age 17.51 ± 3.5 months), who were found to have IDA, constituted the case group while 75 healthy children constituted the control group. The studied nutritional variables through the application of a standardized food frequency questionnaire were: duration of breast feeding, milk that the child drinks during the research, age of solid food introduction, child&apos;s health status according to the mother&apos;s point of view, child&apos;s appetite and frequency of bread, cereals, meat, fish, egg, legumes, chocolate, marmalade, vegetables, fruit and tea intake. The prevalence of IDA in the region was 7.99%. The carriers of b-thalassaemia (2.13%) were excluded from the study. Significant statistical difference has been observed between the two groups (P &lt; 0.001) in all haematological and anthropometric parameters except head circumference (P = 0.85). Concerning the nutritional indices the two groups were statistically different (P &lt; 0.001) in the following: the cases were breastfed less, were drinking fresh cow&apos;s milk and tea, were eating meat, vegetables and fruit less often, had a bad appetite and were more likely to get sick. In conclusion, the prevalence of IDA in this area of Greece is approximately similar to other areas in the developed world. The application of simple food frequency questionnaires for the detection of the nutritional IDA risk factors could prognose and prevent anaemia. © 2005 Taylor &amp; Francis Group Ltd