Systemic lupus erythematosus (SLE) is a chronic, multisystemic autoimmune disease that occurs predominantly in women of fertile age. The association of SLE and pregnancy, mainly with active disease and especially with nephritis, has poorer pregnancy outcomes, with increased frequency of preeclampsia, fetal loss, prematurity, growth restriction, and newborns small for gestational age. Therefore, SLE pregnancies are considered high risk condition, should be monitored frequently during pregnancy and delivery should occur in a controlled setting. Pregnancy induces dramatic immune and neuroendocrine changes in the maternal body in order to protect the fetus from immunologic attack and these modifications can be affected by SLE. The risk of flares depends on the level of maternal disease activity in the 6–12 months before conception and is higher in women with repeated flares before conception, in those who discontinue useful medications and in women with active glomerulonephritis at conception. It is a challenge to differentiate lupus nephritis from preeclampsia and, in this context, the angiogenic and antiangiogenic cytokines are promising. Prenatal care of pregnant patients with SLE requires close collaboration between rheumatologist and obstetrician. Planning pregnancy is essential to increase the probability of successful pregnancies.
There is an increased risk of developing aPL in various infections, particularly in viral infections. The most frequent infection related to aPL has been hepatitis C virus. These antibodies may be associated with thromboembolic events, including catastrophic APS. There is a link between vaccinations, such as the tetanus toxoid and aPL, due to molecular mimicry between the two molecules. Accumulated evidence supports that the presence of aPL is associated with a variety of infections, including viruses, bacteria, fungi, and parasites, and the main mechanism to explain this correlation is molecular mimicry. Moreover, a link between vaccinations, such as the tetanus toxoid, and APS has also been described.
Susac syndrome (SS) is an autoimmune disease characterized by the clinical triad of encephalopathy, branch retinal artery occlusions and neuro-sensorial hearing loss; it is due to a microangiopathy affecting the precapillary arterioles of the brain, retina and inner ear. SS is characterized by typical radiological features on magnetic resonance imaging (MRI) which, together with clinical symptoms, may permit a diagnosis. Branch retinal artery occlusions (BRAOs) are best evaluated using fluorescein angiography (FA) which may show the typical multifocal fluorescence. SS is an autoimmune endotheliopathy that requires treatment with immunosuppressive agents: steroids, azathioprine, mycophenolate mofetil, methotrexate, cyclophosphamide and intravenous immunoglobulin, usually in combination. Plasma exchange is also useful. In addition, antiplatelet agents may be a useful adjunct. Correct immunosuppressive therapy results in significant clinical and radiological improvement. An early diagnosis and treatment are important to delay the disease progression and prevent permanent disability.
The aim of the current study was to analyze the role of traditional and systemic lupus erythematosus (SLE)-related risk factors in the development of vertebral fractures. A cross-sectional study was performed in women with SLE attending a single center. A vertebral fracture was defined as a reduction of at least 20% of vertebral body height. Two hundred ten patients were studied, with median age of 43 years and median disease duration of 72 months. Osteopenia was present in 50.3% of patients and osteoporosis in 17.4%. At least one vertebral fracture was detected in 26.1%. Patients with vertebral fractures had a higher mean age (50 +/- 14 vs. 41 +/- 13.2 years, p = 0.001), disease damage (57.1% vs. 34.4%, p = 0.001), lower bone mineral density (BMD) at the total hip (0.902 +/- 0.160 vs. 982 +/- 0.137 g/cm(2), p = 0.002), and postmenopausal status (61.9% vs. 45.3%, p = 0.048). Stepwise logistic regression analysis revealed that only age (p = 0.001) and low BMD at the total hip (p = 0.007) remained as significant factors for the presence of vertebral fracture. The high prevalence of vertebral fractures in the relatively young population implies that more attention must be paid to detect and treat vertebral fractures.
The objective of this study was to investigate the efficacy and safety of anti-CD20 treatment in Hispanic patients with refractory systemic lupus erythematosus and to determine whether baseline parameters predict disease flare. Fifty-two patients with systemic lupus erythematosus, 13 with active lupus nephritis, eight with thrombocytopenia, three with leukocytopenia, 25 with severe musculoskeletal involvement and three with skin involvement) refractory to conventional therapy were treated with anti-CD20 treatment (rituximab; MabThera, Roche) plus ongoing immunosuppressive treatment. Disease activity was assessed monthly using the SLEDAI validated for the Mexican population with a follow-up period of 6 months. At 6 months of follow-up, significant clinical improvements were detected, with a reduction in the global SLEDAI validated for the Mexican population score. Five of the 13 patients with lupus nephritis (38.4%) had a complete renal response and five (38.4%) had a partial response. Rituximab was also effective in patients with autoimmune thrombocytopenia, inducing a significant increase in platelet counts (p = 0.012). Nineteen of 25 patients with severe musculoskeletal involvement had remission of arthritis. Only one of the three patients with skin involvement had no lesions at 6 months. Rituximab treatment also allowed a reduction of the oral prednisone dose in the majority of patients. No baseline predictors of flare were found. Treatment was discontinued after the first infusion in two patients due to serum sickness and in another due to pulmonary infection. In conclusion, the addition of rituximab to conventional immunosuppressive therapy may be an effective strategy for lupus nephritis, autoimmune thrombocytopenia and inflammatory polyarthritis in patients with refractory systemic lupus erythematosus.
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