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Background Despite the WHO recommendation that the 2010–2011 trivalent seasonal flu vaccine must contain A/California/7/2009/H1N1-like virus there is no consistent data regarding its immunogenicity and safety in a large autoimmune rheumatic disease (ARD) population. Methods 1668 ARD patients (systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), ankylosing spondylitis (AS), systemic sclerosis, psoriatic arthritis (PsA), Behçet's disease (BD), mixed connective tissue disease, primary antiphospholipid syndrome (PAPS), dermatomyositis (DM), primary Sjögren's syndrome, Takayasu's arteritis, polymyositis and Granulomatosis with polyangiitis (Wegener's) (GPA)) and 234 healthy controls were vaccinated with a non-adjuvanted influenza A/California/7/2009(H1N1) virus-like strain flu. Subjects were evaluated before vaccination and 21 days post-vaccination. The percentage of seroprotection, seroconversion and the factor increase in geometric mean titre (GMT) were calculated. Results After immunisation, seroprotection rates (68.5% vs 82.9% p<0.0001), seroconversion rates (63.4% vs 76.9%, p<0.001) and the factor increase in GMT (8.9 vs 13.2 p<0.0001) were significantly lower in ARD than controls. Analysis of specific diseases revealed that seroprotection significantly reduced in SLE (p<0.0001), RA (p<0.0001), PsA (p=0.0006), AS (p=0.04), BD (p=0.04) and DM (p=0.04) patients than controls. The seroconversion rates in SLE (p<0.0001), RA (p<0.0001) and PsA (p=0.0006) patients and the increase in GMTs in SLE (p<0.0001), RA (p<0.0001) and PsA (p<0.0001) patients were also reduced compared with controls. Moderate and severe side effects were not reported. Conclusions The novel recognition of a diverse vaccine immunogenicity profile in distinct ARDs supports the notion that a booster dose may be recommended for diseases with suboptimal immune responses. This large study also settles the issue of vaccine safety. (ClinicalTrials.gov #NCT01151644)

ObjectiveTo review all published cases of the rare association between thrombotic thrombocytopenic purpura (TTP) and Sjögren's syndrome (SS). The authors report an additional case of this unique association.MethodsSystematic review of the literature and a case report. The database were articles published in PubMed/MEDLINE, Web of Science, LILACS, and SciELO, registered from 1966 to August 2020. The DESH terms were \"Sjögren's syndrome\" and \"thrombotic thrombocytopenic purpura,\" without language limitation.ResultsMost patients were female (88%), and the age varied from 30 to 75 years old. Concerning the sequence of disease appearance, SS followed by TTP was seen in seven articles, TTP and SS in three, and simultaneous appearance of both diseases in three studies. Primary SS was observed in 16 patients, and secondary SS was detected in two cases: dermatomyositis and rheumatoid arthritis. Anemia was the most common TTP manifestation, followed by thrombocytopenia, fever, consciousness alteration, renal impairment, and schistocytes' appearance on a blood smear. Treatment involved plasmapheresis, plasma exchange, rituximab, glucocorticoid, and cyclophosphamide. A good outcome was noted in most studies; few patients died.ConclusionsTTP is a rare manifestation associated with SS. After the TTP diagnosis, plasmapheresis and/or plasma exchange should be immediately implemented.

Background Reduced response to pandemic (2009) H1N1 (pH1N1) vaccine in patients with rheumatoid arthritis (RA) was recently reported. Objectives To evaluate the contribution of age, disease activity, medication and previous antibody levels to this reduced response. Methods 340 adult RA patients and 234 healthy controls were assessed before and 21 days after adjuvant-free influenza A/California/7/2009 (pH1N1) vaccine. Disease activity (DAS28), current treatment and pH1N1 antibody titres were collected. Seroprotection, seroconversion and factor increase in geometric mean titre (GMT) were calculated and adverse events registered. Results RA and controls showed similar (p>0.05) prevaccination GMT (8.0 vs 9.3) and seroprotection (10.8% vs 11.5%). After vaccination a significant reduction (p<0.001) was observed in all endpoints: GMT and factor increase in GMT, seroprotection and seroconversion rates. Disease activity did not preclude seroconversion or seroprotection and remained unchanged in 97.4% of patients. Methotrexate was the only disease-modifying antirheumatic drug associated with reduced responses (p=0.001). Vaccination was well tolerated. Conclusions The data confirmed both short-term anti-pH1N1 vaccine safety and, different from most studies with seasonal influenza, reduced seroprotection in RA patients, unrelated to disease activity and to most medications (except methotrexate). Extrapolation of immune responses from one vaccine to another may therefore not be possible and specific immunisation strategies (possibly booster) may be needed. Clinicaltrials.gov no NCT01151644.

This study aims to perform global gonadal and sexual function assessments in systemic lupus erythematosus-related antiphospholipid syndrome (SLE-APS) patients. A cross-sectional study was conducted in ten SLE-APS male patients and 20 healthy controls. They were assessed by demographic data, clinical features, urological examination, sexual function, testicular ultrasound, seminal parameters, sperm antibodies, and hormone profile. The median of current age was similar in SLE-APS patients and controls with a higher frequency of erectile dysfunction in the former group (30 vs. 0 %, p  = 0.029). The median penis circumference was significantly reduced in SLE-APS patients with erectile dysfunction compared to patients without this complication (8.17 vs. 9.14 cm, p  = 0.0397). SLE-APS patients with previous arterial thrombosis had a significantly reduced median penis circumference compared to those without this complication (7.5 vs. 9.18 cm, p  = 0.039). Comparing SLE-APS patients and controls, the former had a significant lower median of sperm concentration (41.1 vs. 120.06 × 10 6 /mL, p  = 0.003), percentages of sperm motility (47.25 vs. 65.42 %, p  = 0.047), normal sperm forms by WHO guidelines (11 vs. 23.95 %, p  = 0.002), and Kruger criteria (2.65 vs. 7.65 %, p  = 0.02). Regarding seminal analysis, the medians of sperm concentration and total sperm count were significantly lower in SLE-APS patients treated with intravenous cyclophosphamide vs. those untreated with this drug ( p  < 0.05). Therefore, we have observed a novel association of reduced penile size with erectile dysfunction and previous arterial thrombosis in SLE-APS patients. Penis assessment should be routinely done in SLE-APS patients with fertility problems. We also identified that intravenous cyclophosphamide underlies severe sperm alterations in these patients.

To report a case of triple association of juvenile systemic lupus erythematosus (SLE), juvenile dermatomyositis and urticarial vasculitis as well as a review of the relevant literature. A 12-year-old male patient diagnosed with overlap syndrome between SLE and juvenile dermatomyositis since 2004 evolved with erythematous plaques, which were compatible with an urticarial rash. Clinical, laboratory and histopathological findings indicated a diagnosis of urticarial vasculitis. The patient previously had a C1q deficiency. Using the established treatment with methylprednisolone (1 g/day for 3 days), increasing doses of deflazacort and introduction of a dapsone, as well as mycophenolate mofetil regimen, with the suspension of azathioprine resulted in complete resolution of skin lesions. Urticarial vasculitis can present in various diseases. In SLE, presentation of urticarial vasculitis in children is rarely found. The triple association of juvenile-onset SLE, juvenile dermatomyositis and urticarial vasculitis is unusual, and this is the first case described in literature.

Catastrophic antiphospholipid (Asherson's) syndrome (CAPS) is known to be a severe variant (1%) of antiphospholipid syndrome, with a high rate of mortality (50%). The distinguishing feature of CAPS is microvascular thromboses in the presence of antiphospholipid antibodies. Molecular mimicry between β2-glycoprotein I and infectious agents, endothelial cell activation and reduced fibrinolysis are the most frequently described pathophysiological mechanisms. Genetic risk factors have also been implicated in the onset of CAPS, although these have not yet been identified. There have been no randomized, controlled trials evaluating the efficacy of any medication on CAPS; however, when CAPS is suspected, aggressive multimodal treatment is required. Patients who receive a combination of anticoagulation therapy, glucocorticosteroids and plasma exchange with or without intravenous immunoglobulin show the best survival rates. Herein, we review the clinical and laboratory findings, diagnostic criteria, pathophysiology, treatment and prognosis of CAPS.

Upper airway manifestations are common features of antineutrophil cytoplasmic antibody (ANCA)-related vasculitis. Determining the presence of this antibody in patients with chronic rhinosinusitis (CRS) may allow early identification and treatment of underlying vasculitis. Methods Forty-nine consecutive CRS patients and 165 age- and sex-matched healthy controls were evaluated for vasculitis complaints. ANCA were detected by indirect immunofluorescence, and antibodies to proteinase 3 (PR3) and myeloperoxidase were determined by ELISA. Patients and controls were comparable concerning the mean age (47.2 +/- 15 years versus 45 +/- 12.5 years; p = 0.303) and female predominance (73.5% versus 60%; p = 0.502). Vasculitis-associated complaints were reported in 8/49 (16.3%) patients: 7 patients reported a 10-year history of asthma and 1 patient had red/painful eyes associated with epistaxis. ANCA was positive in 5/49 (10%) patients and absent in controls (p < 0.0001). One patient had high titer cytoplasmic ANCA/PR3 and during the investigation developed clinical features of Wegener's granulomatosis. The other four patients had perinuclear ANCA, of whom three were asymptomatic and one is currently under surveillance for Churg-Strauss syndrome. Sinus computed tomography scan revealed that patients who were ANCA(+) had more extensive disease involvement than ANCA(-) patients (Lund-Mackay score median value, 21 versus 13; p = 0.008). ANCA may identify a subset of difficult to treat CRS patients with underlying vasculitis and may be useful for establishing an early diagnosis of vasculitis in CRS.

Antiphospholipid syndrome is an autoimmune disease characterized by venous and/or arterial thrombosis and/or recurrent fetal loss and the presence of antiphospholid antibodies. Among the causes of death of antiphospholipid syndrome there are the myocardial infarction and stroke. Comorbidities could worsen the evolution of these patients, and the knowledge about these situations could prevent or minimizethe risks associated with the disease. In this article, we review the literature about cardiovascular comorbidities, such as traditional risk factors for atherosclerosis, metabolic syndrome and vitamin D deficiency, and how these could influence the prognosis of antiphospholipid syndrome patients.

The objective of this study is to determine if digital vasculitis (DV), a clinical manifestation with a high systemic lupus erythematosus disease activity index (SLEDAI) score, is associated with lupus severity. DV and other clinical manifestations defined according to the SLEDAI were evaluated in 168 consecutive patients with systemic lupus erythematosus (SLE). Two groups were defined according to presence (DV+, n = 27) or absence of DV (DV−, n = 141) at the time of evaluation. The exclusion criterion was the presence of antiphospholipid syndrome (Sapporo’s criteria). The two groups were comparable with regard to age (P = 0.09), gender (P = 1.00), white race (P = 0.81), and disease duration (P = 0.78). Compared to the DV− group, the DV+ group had a significantly higher frequency of mucocutaneous manifestations (66.7 vs. 39.0%, P = 0.01), haematological abnormalities (22.2 vs. 6.4%, P = 0.02) and constitutional symptoms (11.1 vs. 0.7%, P = 0.01). Renal and neurological involvements were similar in both groups (P = 0.57 and P = 1.00, respectively). The evaluation of each SLEDAI parameter confirmed that the DV+ group had higher frequencies of mild manifestations, such as new rash (P = 0.02), alopecia (P = 0.02), oral ulcers (P = 0.045), fever (P = 0.01) and leucopenia (P = 0.005). In contrast, both groups had similarly increased anti-dsDNA (P = 0.78) and decreased complement levels (P = 0.29). In conclusion, DV in patients with SLE identifies a subgroup of a mild disease. The high ‘weighted’ index attributed to this alteration in the SLEDAI score should therefore be revised.

Atherosclerosis is an inflammatory disease, leading to the formation of pro-inflammatory and pro-oxidative lipids that generate an immune response. Several antigens have been shown to activate the immune response and affect the development of atherogenesis. Systemic lupus erythematosus is an autoimmune and inflammatory disease strongly associated with premature development of atherosclerotic plaques. Modulation of the immune system could represent a useful approach to prevent and/or treat atherosclerosis. A vaccination-based approach might be a useful, effective tool in the modern arsenal of cardiovascular therapies and could be used on a large scale at a low cost. In non-systemic lupus erythematosus populations, vaccines against oxidized low-density lipoprotein, beta-2-glycoprotein I, heat shock proteins, lipoproteins, cholesterol, molecules involved in cholesterol metabolism, and other molecules (CD99, vascular endothelial growth factor-receptor, and interleukin-2) have been tested, with promising results. However, there are no studies of vaccination against atherosclerosis in systemic lupus erythematosus. Lupus (2009) 18, 1209—1212.