Immunosuppression use in primary antiphospholipid antibody-positive patients: Descriptive analysis of the AntiPhospholipid Syndrome Alliance for Clinical Trials and InternatiOnal Networking (APS ACTION) Clinical Database and Repository ("Registry").

Background/purpose: APS ACTION Registry was created to study the outcomes of patients with persistently positive antiphospholipid antibodies (aPL) with or without other systemic autoimmune disease (SAIDx). Given that immunosuppression (IS) is used for certain aPL manifestations, for example, thrombocytopenia (TP), our primary objective was to describe the indications for IS in aPL-positive patients without other SAIDx. Secondly, we report the type of IS used in patients with selected microvascular or non-thrombotic aPL manifestations.

Pregnancy outcomes in antiphospholipid antibody positive patients: prospective results from the AntiPhospholipid Syndrome Alliance for Clinical Trials and InternatiOnal Networking (APS ACTION) Clinical Database and Repository ('Registry').

Objectives: To describe the outcomes of pregnancies in antiphospholipid antibody (aPL)-positive patients since the inception of the AntiPhospholipid Syndrome Alliance for Clinical Trials and InternatiOnal Networking Registry.

Methods: We identified persistently aPL-positive patients recorded as 'pregnant' during prospective follow-up, and defined as a composite of: (1) Preterm live delivery (PTLD) at or before 37th week due to pre-eclampsia (PEC), eclampsia, small-for-gestational age (SGA) and/or placental insufficiency (PI); or (2) Otherwise unexplained fetal death after the 10th week of gestation. The primary objective was to describe the characteristics of patients with and without aPL-related composite outcomes based on their first observed pregnancies following registry recruitment.

EULAR recommendations for a core data set for pregnancy registries in rheumatology.

Background And Objective: There is an urgent need for robust data on the trajectories and outcomes of pregnancies in women with inflammatory rheumatic diseases (IRD). In particular when rare outcomes or rare diseases are to be investigated, collaborative approaches are required. However, joint data analyses are often limited by the heterogeneity of the different data sources.

Mycophenolate mofetil as steroid-sparing treatment for elderly patients with giant cell arteritis: report of three cases.

Background And Aims: Glucocorticoids have never been studied in a placebo-controlled manner in giant cell arteritis (GCA), but their effectiveness is well established. However, evidence for the efficacy of immunosuppressant drugs as steroid-sparing agents in this disease is highly desirable, especially in elderly patients. We report the use of mycophenolate mofetil (MMF) as a steroid-sparing agent in three patients (mean age 78 years) with GCA, at high risk of longterm high dose glucocorticoids because of type II diabetes mellitus, obesity, hypertension or osteoporosis.

Prevention of thrombosis relapse in antiphospholipid syndrome patients refractory to conventional therapy using intravenous immunoglobulin.

Objectives: To investigate the long-term effects of megadoses of intravenous immunoglobulin (IVIG) in a small cohort of patients with relapsing primary APS resistant to conventional treatments.

Methods: Five primary APS patients, 4 women, mean age 45.1 years (range 31-76 years), were considered eligible for IVIG therapy due to relapsing thrombotic events (4 recurrent venous thromboses, 2 ischaemic cerebral strokes, 2 pulmonary thromboembolisms, 1 thrombotic event on the vena cava filter), despite conventional therapy with anticoagulants.

Long-term effects of rituximab added to cyclophosphamide in refractory patients with vasculitis.

Background: Current therapies have changed systemic vasculitis from a disease with a high rate of mortality to a chronic curable condition. A limited percentage of patients either remains refractory to conventional treatment or experiences dose-limiting side effects.

Methods: 11 patients (4 affected by idiopathic systemic microscopic polyangiitis, 5 by Wegener's granulomatosis, and 2 by Churg-Strauss syndrome) intolerant or refractory to conventional therapies including cyclophosphamide were enrolled.

Presence of low titre of antiphospholipid antibodies in cancer patients: a prospective study.

Antiphospholipid antibodies (aPL) represent a well-defined risk factor for thrombotic events. aPL have been observed in the plasma of cancer patients, but the role and clinical relevance of aPL in this clinical setting is still unclear. This is a prospective cohort study whose aims were to: (1) compare the prevalence of aPL antibodies in cancer patients at diagnosis to matched control subjects; (2) compare thrombosis-free survival and overall survival in aPL positive and aPL negative cancer patients.

Effects of anti-CD20 monoclonal antibody as a rescue treatment for ANCA-associated idiopathic systemic vasculitis with or without overt renal involvement.

Background: Cyclophosphamide (CYC) is thought to be the most effective treatment for antineutrophil cytoplasmatic antibody (ANCA)-associated idiopathic systemic vasculitis with severe organ or life threatening presentation. The key mechanism of action of CYC is suppression of the B lymphocyte activity. However, a considerable minority of patients either remains refractory to conventional therapy or experiences dose-limiting side effects.

Rituximab as a therapeutic tool in severe mixed cryoglobulinemia.

Type II mixed cryoglobulinemia (MC) is a systemic vasculitis, associated in most cases with hepatitis C virus (HCV) infection, sustained by proliferation of oligoclonal cells. Systemic B cell depletion and clinical remission can be achieved in non-Hodgkin lymphoma by human/mouse chimeric monoclonal antibody that specifically reacts with the CD20 antigen (rituximab). Similar effects could be expected in type II MC.

[Antiphospholipid syndrome and kidney].

The diagnosis of antiphospholipid syndrome (APS) relies on clinical and laboratory criteria, which have been recently outlined in specific consensus conferences. Renal involvement in APS is not infrequent and includes different clinical patterns. For clinical purposes a distinction can be made between large vessel and microvascular involvement.

Long-term effects of anti-CD20 monoclonal antibody treatment of cryoglobulinaemic glomerulonephritis.

Background: Type II mixed cryoglobulinaemia (MC) is a systemic vasculitis, associated in most cases with hepatitis C virus (HCV) infection, and sustained by proliferation of oligoclonal cells. Systemic B-cell depletion and clinical remission can be achieved in non-Hodgkin lymphoma by a human/mouse chimeric monoclonal antibody that specifically reacts with the CD20 antigen (Rituximab). Similar effects could be expected in type II MC.