Management of refractory anti-phospholipid syndrome

Thrombotic recurrences and bleeding events in APS vascular patients: A review from the literature and a comparison with the APS Piedmont Cohort

“…Second, the possibility that a clinical subset of APS vascular exists, i.e., those who have recurrences despite correct OAT, must be seriously considered. This fact represents a treatment failure, and some studies have shown that this is not at all a rare event [9], [12] and [37]. In particular, this happens in higher risk patients for aPL profile (triple positivity, Miyakis types 1 and 2a) [38].…”

Treatment with rituximab in patients with mixed cryoglobulinemia syndrome: Results of multicenter cohort study and review of the literature

“…Since 1999 anecdotal observations followed by two pilot studies showed the efficacy and safety of anti-CD20 monoclonal antibody (rituximab) treatment in patients with cryoglobulinemic vasculitis, often resistant or intolerant to other therapies [9][10][11][12]. The safety and efficacy of this drug have been reported in various vasculitides and other systemic autoimmune diseases [13][14][15][16][17][18][19][20][21][22][23]. The usefulness of monoclonal antibodies directed to CD20 antigen, a trans membrane protein expressed on pre-B lymphocytes and mature B lymphocytes, on both cutaneous and visceral organ involvement of MCs, including low grade B-cell lymphoma, has been reported by various Authors, generally referred to small patients' series [24][25][26][27][28][29][30][31].…”

Refractory Thromobotic Anti-Phospholipid Syndrome Manifesting With Budd Chiari Syndrome and Pulmonary Embolism.

“…The desired INR for these patients has been debated. An INR of 2.0 -3.0 has been suggested as a target in patients who present with venous thrombosis while an INR of 3.0 -4.0 is desired in those with arterial thrombosis [4]. However, there are no enough studies available to support this recommendation [5] .…”

“…However, there are no enough studies available to support this recommendation [5] . Moreover, such high INR is associated with increased risk of bleeding [4]. There is only scarce evidence on the treatment approach for patients with refractory APS (who have recurrent obstetric complications or recurrent thrombosis despite adequate anti-coagulation treatment) [4], such as our case.…”