Background. The purpose of this study was to analyze the risk of aortic events (death, dissection, or aortic rupture) associated with Marfan syndrome and decide on the optimal timing for preventive surgery on the aortic root.Methods. From January 2004 to June 2015, 397 patients from Marfan Units were studied by echocardiographic and computed tomography and magnetic resonance imaging of aorta and periodic annual monitoring. Mean follow-up was 5.6 ± 2.7 years. The annual incidence of aortic events was assessed according to aortic diameters for the optimal time for prophylactic surgery to be decided on.Results. Mean age at the first visit was 28.4 ± 14.5 years, with mean sinuses of Valsalva diameter of 37.1 ± 6.6 mm., Eleven aortic events occurred during follow-up: seven deaths and four acute aortic dissections. Mean annual risk of an aortic event was 0.5% (risk of death 0.32% and risk of aortic dissection 0.18%). The increase in aortic diameters was associated with increased risk of 0.2% per
Material and MethodsThis multicenter, retrospective, observational study was conducted in MS patients, who fulfilled the Ghent international criteria (genetic study if appropriate). All patients were recruited at the
Primary intraosseous arteriovenous malformations are rare. This case shows the scarce specificity of clinical findings, radiology, and bone scan in the diagnosis of these lesions when they are localized in vertebral territory. Surgical excision of the lesion relieves pain but does not completely correct the scoliotic attitude.
Background
Benlysta is a human monoclonal antibody that is indicated as add-on treatment in adult patients with active, autoantibody-positive (antinuclear antibodies ANA ≥ 1/80) systemic lupus erythematosus (SLE) with a high degree of disease activity despite standard treatment.
Purpose
To investigate the benefit of use of belimumab for the treatment of SLE in a tertiary care hospital.
Materials and methods
A retrospective observational study of patients undergoing belimumab treatment between January 2012 and July 2013. The following data were reviewed from Selene (medical history software): sex; age; dose; ANA and complement levels; articular, cutaneous, haematological or other conditions; concomitant treatments; adverse reactions; suspension and cause.
Results
6 patients with active SLE (100% with ANA ≥ 1/80) were treated with belimumab (average age 38, 100% women) with a standard dose of 10 mg/kg. All revealed articular involvement, five cutaneous involvement, 2 haematological involvement, and 2 renal involvement (lupus nephritis).
All patients were on a stable SLE treatment regimen consisting of (alone or in combination): corticosteroids (prednisone), anti-malarials (hydroxychloroquine) or immunosuppressive (mycophenolate mofetil).
The most common adverse reactions were: asthenia (4 patients), lymphopenia (2), neutropenia (2), anaemia (1), cutaneous recurrences (3), nausea (3), infections (cystitis (2), respiratory infection (1)), arthralgia (1).
50% had to discontinue treatment due to lack of response (2) or prolonged adverse reactions (neutropenia) (1).
Conclusions
Belimumab is a recently launched drug which may be useful as an add-on treatment for those patients with active SLE. 50% of patients treated with belimumab revealed sustained improvement in SLE disease activity, with less fatigue, arthralgia and cutaneous exacerbations than that which they had under standard treatment.
No conflict of interest.
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