SS is a chronic systemic autoimmune disease characterized by decreased exocrine gland function. A variety of other disease manifestations may also be present, including general constitutional symptoms and extraglandular features. A multidisciplinary approach focused on both local and systemic medical therapies is needed as the disease has a wide clinical spectrum. The current treatment for SS is mainly symptomatic. However, there is evidence that systemic drugs are effective in controlling extraglandular manifestations of the disease. Overall evidence for the role of conventional immunosuppressive therapy is limited. A number of attempts to use biologic therapies have led to variable results. Biologic agents targeting B cells, such as rituximab, epratuzumab and belimumab, have shown promising results, but further studies are needed to validate the findings. Early-phase studies with abatacept and alefacept proved that T cell stimulation inhibition is another potentially effective target for SS treatment. Modulation or inhibition of other targets such as IFN, IL-6 and Toll-like receptor are also currently being investigated. We have summarized the available evidence regarding the efficacy of biologic treatments and discuss other potential therapies targeting pathways or molecules recognized as being involved in the pathogenesis of SS.
Patients with autoimmune disorders are at an increased risk of venous thromboembolism (VTE), but this association has not been consistently evaluated. We used the RIETE (Registro Informatizado Enfermedad Trombo Embólica) database to compare the rates of VTE recurrences, major bleeding, and death during the course of anticoagulation, according to the presence or absence of autoimmune disorders. Of 71 625 patients with VTE recruited in February 2018, 1800 (2.5%) had autoimmune disorders. Median duration of anticoagulant therapy was slightly longer in patients with autoimmune disorders (median, 190 vs 182 days; P = .001). On multivariable analysis, patients with autoimmune disorders had a similar risk of VTE recurrences (hazard ratio [HR]: 0.93; 95% confidence interval [CI]: 0.68-1.27) or major bleeding (HR: 1.07; 95% CI: 0.82-1.40) and a lower risk to die (HR: 0.66; 95% CI: 0.54-0.81) than those without autoimmune disorders. Patients with giant cell arteritis had the highest rates of major bleeding (8.6 events per 100 patient-years) and the lowest rate of recurrences (zero). In other subgroups, the rates of both events were more balanced. During anticoagulation, patients with or without autoimmune disorders had similar rates of VTE recurrences or major bleeding. However, there were some differences between subgroups of patients with autoimmune disorders.
Advances in our knowledge of the pathogenic mechanisms of antiphospholipid syndrome have been achieved in the past few years. Apart from the well-known role of anti-β2-glycoprotein I antibodies, complement, endocrine and genetic factors and a variety of other molecules are now under investigation. These new approaches should lead to novel explanations and potential new treatment options.
SUMMARY
The ideal duration of anticoagulation therapy in patients with autoimmune disorders and venous thromboembolism (VTE) is controversial. We used the Registro Informatizado de Enfermedad TromboEmbólica (RIETE) database to compare the incidence rate of major bleeding during anticoagulation versus the incidence rate of VTE recurrences after its discontinuation. We included 1061 patients with autoimmune disorders and VTE followed‐up after discontinuing anticoagulant therapy: rheumatoid arthritis, 321; polymyalgia rheumatica, 159; ulcerative colitis, 134; Crohn’s disease, 111; systemic lupus erythematosus (SLE), 82; giant cell arteritis, 58; ankylosing spondylitis, 39; Behcet disease, 17; other vasculitides, 140. During anticoagulation (median, 183 days), 64 patients had major bleeding. After discontinuing anticoagulation (median, 190 days), 112 patients developed symptomatic VTE recurrences. In most subgroups, the incidence rate of major bleeding during therapy was similar to the incidence rate of VTE recurrences after its discontinuation. However, in patients with SLE (10.0 major bleeds, 95% confidence interval [CI] 4.07–20.9) per 100 patient‐years vs. 3.62 VTE recurrences, 95% CI 1.15–8.74) or ankylosing spondylitis (10.9 major bleeds [95% CI 3.47–26.3] vs. 4.69 VTE recurrences, 95% CI 1.19–12.8) the incidence rates of major bleeding during anticoagulation were over twofold higher than the incidence rates of VTE recurrences after its discontinuation.
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