BackgroundPulmonary fibrosis is one of the gravest manifestations of Systemic Sclerosis (SSc) and conventional DMARDs therapy has not shown any particular positive effect.ObjectivesOur goal was to see whether the elimination pf B - lymphocytes through use of anti – CD20 Mab, Rituximab (RTX) would offer to the improvement of the pulmonary function of SSc patients.MethodsWe studied 23 SSc patients with pulmonary fibrosis, who received treatment with RTX (n=12) or DMARDs treatment (n=11) for 1 to 3 years (1,9 years). Conventional therapy included azathioprine (n=4), mycophenalate (n=6) and methotrexate (n=2). RTX – treated patients were recorded with FVC improvement in the first year of treatment (FVC 81,3 +/- 12,6 vs FVC 87,4 +/- 11,3 out on the onset of the study and at the first year respectively, p=0,02) when on the other hand DMARDs treated patients didn't show any FVC improvement at all.ResultsAll RTX – treated patients did not present any lung HRCT deterioration imaging, in contrast to DMARDs – treated patients who were also submitted to lung HRCT each year of the study, all showing signs of CT imaging deterioration. Similar findings, as far as FVC was concerned, were recorded at the 3rd year of the study (RTX patients, n=6 and DMARDs patients, n=11). 3rd year RTX FVC was 92,6 +/-13,2 vs 80,7 +/-11,8 at the onset of the study, p=0,04 when DMARDs treated patients were all presented with worst 3rd year FVC compared to their primary FVC, p<0,01.ConclusionsOur small cohort of SSc patients with pulmonary fibrosis shows that it is possible that rituximab may be proven helpful to at least prevent the deterioration of the interstitial pulmonary fibrosis of Systemic Sclerosis. The pathophysiology of this particular fibrosis and the possible molecular role of B-lymphocytes in the inversion of this procedure need yet to be furtherly explored.Disclosure of InterestNone declared
BackgroundResistant – recurring pericarditis (RRP) is often associated with autoinflammatory syndromes. Conventional therapy with glucocorticoids and colchicine is often enough unsuccessful, thus nowadays the use of anakinra in such cases is becoming common every – day practice.ObjectivesOur goal was to determine whether RRP was associated with gene mutations relevant with autoinflammatory diseases and then compare the effectiveness of canakimumab and anakinra.MethodsWe studied 18 patients with RRP (all women, from 14 to 36 years old) determing the existence of mutation from the infevers database (25 genes, from MEFV and TNFRSF1A to MVK and SLC 29A3, all related with autoinflammatory diseases). All the patients were proven positive to one of the above mutations (all heterozygous) and then treatment with anakinra (n=9, 100mg sc once daily) and canakimumab (n=9, 150mg sc q4wk) was administered.ResultsDuring the study (3 years), all anakinra patients stopped receiving per os glucocorticoids and were free of any new pericarditis episodes. 3 of them (33%) stopped any treatment after a year of anakinra therapy and none was presented with any skin reactions.On the contrary, all canakimumab patients were not able to free themselves of the glucocorticoid treatment, since at least one new episode of pericarditis to each patient was recorded. Two of them were free of pericarditis the last year of the study (though being treated with 2,5mg prednisolone per os daily).Statistically speaking, the use of IL-1R antagonist in recurrent – resistant pericarditis associated with autoinflammatory mutations was proven much more successful (p<0,01) than the use of the IL-1b Mab.ConclusionsThough the sample of patients (n=18) was too small in order to set us able to reach any safe conclusions, it is quite possible that anakinra may be one effective solution that can prevent the long term administration of corticoids to patients with recurrent pericarditis Canakimumab was not recorded with similar positive results.Disclosure of InterestNone declared
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