We studied eight affected and four unaffected individuals from a Colombian family with autosomal dominant diffuse high bone density. Affected individuals have normal, proportional height and high serum alkaline phosphatase activity. Radiographically, affected members exhibit generalized, symmetrically diffuse endosteal hyperostosis of the long bones and skull with narrow medullary cavities and loss of the diploë, respectively. There is no periosteal reaction or decreased hematopoiesis. Furthermore, osteosclerosis affects vertebral bodies, ribs, pelvis, mandible, clavicles, and scapulae. Bone mineral density is 2.4-7.3 SD above the mean for age and gender in affected individuals. Affected vs. unaffected individuals' Z-scores were (mean +/- SD) 5.03 +/- 1.77 vs. 0.08 +/- 0.97, respectively, P = 0.0004). Three affected subjects older than 40 yr old lost bone mass in 6 yr. No dysmorphism, abnormal facial features, bone fractures, or cranial nerve involvement was found. The pattern of inheritance, the absence of asymmetries and malformations, the increased serum alkaline phosphatase, the peak bone mass that appears to decrease physiologically with age, and the involvement of cortical and trabecular bone suggest a new variant of hyperostosis/osteosclerosis that affects the entire skeleton.

Conclusion From our study was found that there is a directly proportional relationship between hypocomplementemia and the severity of haematological involvement in SLE patients.The more severe it is the haematological involvement, the more affected seem to be the complement serum levels.

and the Charlson index (RR: 2.5; 95% CI: 1.3 to 4.8, p=0.009). The accumulated damage (SDI>0) was closely associated with significance: RR: 2.0; 95% CI: 1.0 to 4.0, p=0.07. The use of immunosuppressants was associated with a significant increase in the risk of mycobacterial infection: RR: 4.3; 95% CI: 2.2 to 8.3, p=0.31.Two patients (4.8%) died (1respiratory and 1extrapulmonary). The mean survival after diagnosis in these cases was 21 days. Conclusion M.I. in RELESSER affects 1.15% of patients. Its incidence rate is 1 per 1000 patients/year (95% CI: 0.7 to 1.4). Extrapulmonary localization affects more than half of the patients and is associated with the use of immunosuppressants. Previous admission by SLE activity, renal involvement, severity of SLE, and increased number of associated comorbidities are factors associated with the existence of mycobacterial infection.

Methods A total of 1,392 SLE patients were included in the analysis. At the first visit when vitamin D was measured, 76.7% had levels of 25-hydroxyvitamin D<40 ng/mL. The SLE patients were: 92% female, mean age 42.9 years, and ethnicity 50% Caucasian, 41% African American. 27% patients had a history of thrombosis; 7% stroke, 4% MI and 14% DVT. Results Vitamin D, measured either as a continuous variable or as 'low' (<40 ng/mL) vs normal, was associated with any thrombosis and with DVT.We next looked prospectively: this analysis excluded thrombotic events before the first vitamin D measurement. It allowed for vitamin D to be a time-varying variable, as replacement therapy was given if it was low. After adjustment for race, age and sex, the adjusted hazard ratio remained significant for any thrombosis: 1.75 (1.04,2.92). Conclusion Low vitamin D was significantly associated with any thrombosis and with DVT (even after adjustment for lupus anticoagulant). In prospective models it remained significantly associated with any thrombosis. As supplementation with vitamin D was proven to reduce thrombosis in an oncology randomised clinical trial, vitamin D replacement should become routine in SLE patients at risk for thrombosis.

psychosis, seizures requiring therapy for 6 months, cerebrovascular accident ever, cranial or peripheral neuropathy, transverse myelitis). We excluded from the analysis patients with neurologic involvement at entry or those who were lost to follow up before 6 months have elapsed from baseline or who had died during that time period. Data were recorded in an ARTHROS database. Statistical analysis: Patients who developed and those who did not develop NP-damage were compared using the log-rank test. Independent predictors of NP-damage accrual were identified using a Cox proportional hazard regression model. Results During a median follow-up time of 47 months, 79 (7.2%) patients developed NP-damage. In the univariable analyses, variables predictive of NP-damage were: cardiovascular disease (4.16 per 100 patient-year of follow up [% pyf] vs. 1.62% pyf in patients without cardiovascular disease, p < 0.001), renal disease (2.92% pyf vs. 1.73% pyf, p = 0.038) and lymphopenia (2.71% pyf vs. 1.90% pyf, p = 0.012). In the multivariable analysis only cardiovascular disease (Yes vs. No) was retained in the model: HR 2.554 (95% CI: 1.580-4.128). During follow-up, mortality was higher in those who developed as compared to those who did not develop NP-damage (12/79, 15.2% vs. 34/ 1021, 3.3%; p < 0.0001). Conclusions Cardiovascular disease was predictive of the occurrence of NP-damage. Furthermore, the occurrence of NP-damage was significantly associated with a higher mortality. A better control in the early stages of neurological manifestations (early diagnosis and treatment) is needed to reduce NP-damage and improve survival. Acknowledgements On behalf of GLADEL.