Utility of neutrophil-to-lymphocyte ratio plus C-reactive protein for infection in systemic lupus erythematosus.

OBJECTIVE: To analyze the utility of neutrophil-to-lymphocyte ratio (NLR) plus C-reactive protein (CRP) to differentiate between infection and active disease in patients with SLE. METHODS: A cross-sectional study of a cohort of patients with SLE was carried out. Blood samples from four groups (patients without infection or active disease, patients with infection, patients with active disease, and patients with both infection and active disease) before therapeutic interventions were analyzed. We excluded patients with current malignancy, pregnancy, ischemic heart disease or use of antimicrobials during previous 7 days. Hematological cell count, CRP and cultures were obtained. We constructed receiver operating characteristic curves; sensitivity, specificity, positive predictive value (PPV) and negative predictive value (NPV) were calculated. RESULTS: Forty patients were included. NLR cut-off ≥6.3 had sensitivity 70%, specificity 85%, PPV 83% and NPV 74% to detect patients with non-viral infections. A CRP cut-off ≥7.5 mg/L had sensitivity 90%, specificity 75%, PPV 78% and NPV 88% to detect infections regardless of SLE activity. Combination of CRP plus NLR improves the specificity to 90% and PPV to 88%. Excluding the group with both infection and active disease, CRP plus NLR expands specificity to 95% and NPV to 90%. CONCLUSION: In our experience, levels of CRP, particularly CRP plus NLR, were useful in differentiating patients with SLE from those with suspected non-viral infection regardless of the activity of the disease.

Inflammatory biomarkers, disease activity index, and self-reported disability may be predictors of chronic arthritis after chikungunya infection: brief report.

The chikungunya virus (ChikV) is a reemerging mosquito-borne pathogen that causes disabling chronic arthritis. The relationship between clinical evolution and inflammatory biomarkers in patients with ChikV-induced arthritis has not been fully described. We performed a prospective case series to evaluate the association among joint involvement, self-reported disability, and inflammatory biomarkers. Patients with ChikV infection were followed for 1 year. Joint involvement and self-reported disability were evaluated with disease activity index 28 (DAS-28) and World Health Organization Disablement Assessment Schedule II (WHODAS-II). Interleukin-6 (IL-6), C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), and rheumatoid factor (RF) were used as biomarkers. Ten patients with mean age 48 ±15.04 years were included. Symptoms at diagnosis were fever, arthralgias, myalgias, rash, arthritis, nausea, vomiting, and back pain. Polyarticular involvement was present in seven cases. At diagnosis, measures were as follows: DAS-28, 5.08±1.11; WHODAS-II score, 72.3±10.3 %; CRP, 5.09±7.23 mg/dL; ESR, 33.5±17.5 mm/h; RF, 64±21.7 IU/mL; and IL-6, 17.6±10.3 pg/mL. Six patients developed subacute and chronic symptoms. During follow-up, DAS-28 index, WHODAS-II score, ESR, and IL-6 were statistically different in patients with subacute and chronic symptoms compared to those who resolved in the acute phase (p < 0.05). DAS-28 index, WHODAS-II score, and IL-6 were related to chronicity of articular symptoms and could be used as predictors of ChikV-induced arthritis.

Active haematological manifestations of systemic lupus erythematosus lupus are associated with a high rate of in-hospital mortality.

The aim of this study was to estimate the impact of the haematological manifestations of systemic lupus erythematosus (SLE) on mortality in hospitalized patients. For that purpose a case-control study of hospitalized patients in a medical referral centre from January 2009 to December 2014 was performed. For analysis, patients hospitalized for any haematological activity of SLE ( n = 103) were compared with patients hospitalized for other manifestations of SLE activity or complications of treatment ( n = 206). Taking as a variable outcome hospital death, an analysis of potential associated factors was performed. The most common haematological manifestation was thrombocytopenia (63.1%), followed by haemolytic anaemia (30%) and neutropenia (25.2%). In the group of haematological manifestations, 17 (16.5%) deaths were observed compared to 10 (4.8%) deaths in the control group ( P < 0.001). The causes of death were similar in both groups. In the analysis of the variables, it was found that only haematological manifestations were associated with intra-hospital death (odds ratio 3.87, 95% confidence interval 1.8-88, P < 0.001). Our study suggests that apparently any manifestation of haematological activity of SLE is associated with poor prognosis and contributes to increased hospital mortality.

Effect of ezetimibe plus pravastatin on endothelial dysfunction in patients with systemic lupus erythematosus.

BACKGROUND: Patients with systemic lupus erythematosus (SLE) have a higher risk for cardiovascular disease (CVD), not fully explained by the conventional risk factors. These patients have endothelial dysfunction (ED) as an early process of atherosclerosis, which can be reversed with therapy. OBJECTIVE: To determine the effect of ezetimibe plus pravastatin on endothelial function in patients with SLE after 12 months of treatment. PATIENTS AND METHODS: An open study, before and after, which assessed the effect of ezetimibe plus pravastatin treatment, was performed. Twenty two patients (21 women and one man) with diagnosis of SLE were studied, with a mean age 40 ± 5 years. Endothelial dysfunction was evaluated using vascular ultrasound of the brachial artery in order to measure the flow-mediated vasodilation (FMV) basal and after 12 months of treatment with pravastatin 40 mg/day plus ezetimibe 10 mg/day. In addition, a lipid profile: total cholesterol (TC), HDL-cholesterol (HDL-C), LDL-cholesterol (LDL-C), and serum C-reactive protein (CRP), was done. RESULTS: We found a basal FMV of 7.58% and 18.22% after 12 months of treatment, with an improvement of 10.64 points 95% CI (7.58-13.58), p < 0.001. TC decreased from 201.3 ± 58.9 mg/dL to 158.06 ± 50.13 mg/dL (p < 0.01); LDL-C from 125.78 ± 44.4 mg/dL to 78.8 ± 32.9 mg/dL (p < 0.001); HDL-C increased from 49.0 ± 16.8 mg/dL to 52.2 ± 13.8 mg/dL (p = 0.077). The basal and final concentrations of CRP were 4.49 and 2.8, respectively, with a mean decrease of 2.11 mg/dL, 95% CI (0.908-3.32), p < 0.002. Both drugs were well tolerated. CONCLUSION: Ezetimibe plus pravastatin significantly improved FMV in patients with SLE, decreasing ED and the lipid profile. This treatment ameliorated an early process of atherosclerosis and a risk factor for CVD.

Primigravida is associated with flare in women with systemic lupus erythematosus.

The objective of this study was to identify risk factors associated with flare during pregnancy in women with systemic lupus erythematosus (SLE). We performed a retrospective analysis of pregnant women with SLE in a referral hospital. Flare was considered according to predetermined definitions. We analyzed 15 clinical, biochemical and immunological variables with a potential predictive value for relapse during pregnancy. We included 124 lupus pregnancies in 120 women. The relapse rate during pregnancy was 37.9% (47 episodes). The most common manifestations of flare were renal, joint, cutaneous and hematological. Patients with flare during pregnancy developed a higher frequency of preeclampsia and preterm delivery. In multivariate analysis, primigravida was a risk factor associated with any type of flare during pregnancy (OR 2.3, 95% CI 0.99-5.52, p = 0.05); on the other hand, primigravida (OR 3.6, 95% CI 1.19-11.3, p = 0.02), activity prior to pregnancy (OR 3.7, 95% CI 0.97-14.1, p = 0.05), and previous renal disease (OR 5.8, 95% CI 1.95-17.6, p = 0.001) were the principal risk factors associated with renal flare. The first pregnancy in women with SLE is associated with any type of flare. Disease activity is associated with preeclampsia and preterm delivery. Close monitoring is mandatory to identify relapses and timely treatment.

Carotid atherosclerosis is not associated with lower bone mineral density and vertebral fractures in patients with systemic lupus erythematosus.

BACKGROUND: Low bone mineral density (BMD) and vertebral fractures (VF) have been associated with atherosclerosis in the general population. We sought to investigate the relationship between BMD and VF and carotid atherosclerosis in women with systemic lupus erythematosus (SLE). METHODS: We studied 122 women with SLE. All patients had BMD, carotid intima-media thickness (IMT), and carotid artery atherosclerotic plaque assessment by ultrasound. RESULTS: Mean age at study entry was 44 years and mean disease duration was 11 years. Carotid plaque was found in 13 (11%) patients (9 postmenopausal and 4 premenopausal). Patients in the highest IMT quartile were more likely to be older (p = 0.001), have a higher body mass index (p = 0.008), and exhibit dyslipidemia at study entry (p = 0.041), compared with the lower three quartiles. BMD at the lumbar spine was lower in patients in the highest IMT quartile compared with the lower quartiles in the multivariate logistic analysis, however, there was no association between lumbar or total hip BMD and IMT (p = 0.91 and p = 0.6, respectively). IMT measurements did not differ according to the presence or absence of VF (0.08 ± 0.12 vs. 0.06 ± 0.03 mm, p = 0.11). A trend towards higher incidence of VF was found in patients with carotid plaque compared with those without (33% vs. 21%; p = 0.2). CONCLUSIONS: In patients with SLE, the presence of carotid atherosclerosis is not associated with low BMD or VF.

Fulminant systemic vasculitis in systemic lupus erythematosus. Case report and review of the literature.

Vasculitis in systemic lupus erythematosus (SLE) has a broad spectrum of clinical manifestations from cutaneous to visceral involvement and its prognosis ranges from mild to life-threatening. We report the case of a previously healthy 17-year-old woman with eight months' history of arthralgias and myalgias. Subsequently, she developed facial and lower limbs edema, and hair loss. Two weeks before admission to a secondary level hospital, she developed fever up to 40°C followed by abdominal pain, rectal bleeding, hematemesis and blisters on both legs, reason for which she was hospitalized. With active bullous SLE with rapidly progressive glomerulonephritis suspected, she was treated with methylprednisolone pulses without response. After one week of treatment, she was transferred to a tertiary level hospital. On admission she presented acute arterial insufficiency of the lower extremities, respiratory failure with apnea, metabolic acidosis and shock; six hours later she died. Autopsy findings showed active diffuse lupus nephritis and diffuse systemic vasculitis that involved vessels from the skin, brain, myocardium, spleen, iliac and renal arteries. In addition, serositis of the small intestine and colon, acute and chronic pericarditis, pericardial effusion and myocarditis were found. Immunologic tests confirmed SLE diagnosis. In this case the fulminant course was the result of SLE high disease activity, visceral vasculitis of several organs and late diagnosis, referral and treatment. Early diagnosis, and opportune referral to the rheumatologist for intensive treatment can improve the outlook in these patients.

Oxidative stress in Mexicans with diffuse cutaneous systemic sclerosis.

To compare oxidative stress (OS) biomarkers and antioxidant capacity of plasma (ACP) between dcSSc (diffuse cutaneous systemic sclerosis) and healthy Mexicans and their possible relationship with autoantibodies, C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), and uric acid (UA). We included 28 dcSSc and 28 healthy individuals. Patients were grouped in early and late dcSSc and were excluded if they had infections, neoplasias, comorbidity, or antioxidant treatment. Lipoperoxidation products (malondialdehyde), protein oxidation products (carbonyls, dityrosines), ACP, CRP, ESR, and UA were investigated. Age was 47.5 ± 10 in dcSSc versus 48 ± 7 years in controls. In dcSSc, OS was higher and ACP was decreased versus controls (p < 0.001). OS was similar in early and late dcSSc. Anti-Scl-70 (anti-topoisomerase I) was associated with a higher OS (p < 0.05). Eight dcSSc patients had hyperuricemia (28.5 %). A significant correlation between UA and malondialdehyde, dityrosines and carbonyls levels (r = 0.52, r = 0.78 and r = 0.69, p < 0.01) respectively, was found in dcSSc group. A high level of ESR was present in 71 % and CRP in 40 % of dcSSc patients. Mexican dcSSc patients had elevated lipid/protein OS with respect to healthy controls. These OS biomarkers have direct correlation with UA levels. ESR and CRP were elevated in a great number of dcSSc patients. These biochemical markers suggest that dcSSc patients have a continuous stimulus for endothelial dysfunction and accelerated atherogenesis.

Cerebral blood flow abnormalities in neurologically asymptomatic patients with primary antiphospholipid syndrome.

OBJECTIVE: To evaluate cerebral blood flow abnormalities in primary antiphospholipid syndrome (PAPS) patients without ongoing neurological manifestations. PATIENTS AND METHODS: We included 28 PAPS patients and 28 healthy controls. Carotid Doppler ultrasound, and echocardiographic evaluation were done. Transcranial Doppler ultrasonography measured mean flow velocity (MFV) in the carotid siphon, middle, anterior, posterior, intracranial vertebral arteries, and basilar artery (11 cerebral arteries). Results were considered abnormal when the MFV was out of the normal range according to age and/or flow asymmetry and/or more than four arterial segments affected. RESULTS: The mean age of patients was 41.4 ± 11.2 and 39.3 ± 8.6 years in controls. Disease duration was 11 ± 2.7 years. A significant increase in MFV in 7/11 cerebral arteries in PAPS patients, mainly in the middle and anterior cerebral arteries was found compared with controls. A significant association between lupus anticoagulant, history of stroke and obesity with a greater number of affected arteries was found. We did not find an association between MFV and abnormal echocardiography, arterial hypertension and carotid intima-media thickness. CONCLUSIONS: Asymptomatic patients with PAPS can have significantly increased MFVs. These alterations may be the consequence of accelerated atherosclerosis, PAPS vasculopathy or both. Whatever the cause, these findings can represent a risk for stroke in PAPS patients that needs the trial of other therapeutic options.

Human adjuvant disease induced by foreign substances: a new model of ASIA (Shoenfeld's syndrome).

OBJECTIVE: To investigate the clinical, laboratory and histological manifestations of patients who received illegal injections of foreign substances for cosmetic purposes. PATIENTS AND METHODS: We studied patients who met the following inclusion criteria: 1) history of application of foreign substances for cosmetic purposes, 2) clinical data of autoimmune disease or non-specific autoimmune manifestation (i.e. arthralgias, myalgia, malaise, fever, and weight loss), 3) detection of autoantibodies in patients' sera, 4) histological evidence of chronic inflammation and/or granulomatous reaction to foreign body. RESULTS: Fifty female patients aged 44.4 ± 10 years were studied. The mean time between application of foreign substances and onset of symptoms was 4.5 ± 4.3 years. Patients were followed for 12 ± 7.5 years. Forty-one patients were injected with mineral oil, nine patients received other substances: three iodine gadital, one guayacol, one guayacol plus silicone fluid, two collagen, two silicone fluid. The sites of application were: buttocks (36), legs and/or thighs (11), breasts (eight) hands and face (one), face (two) (seven patients received an injection to more than one site). Thirty patients presented with non-specific autoimmune manifestations, whereas 20 patients fulfilled the criteria for a defined autoimmune disease such as systemic lupus erythematosus, rheumatoid arthritis, systemic sclerosis, overlap syndrome, autoimmune hemolytic anemia, autoimmune thyroiditis, autoimmune hepatitis, and ulcerative colitis. CONCLUSIONS: Cases of human adjuvant disease following illegal injections of oil substances for cosmetic purposes are reported. Patients presented with defined autoimmune diseases as well as with non-specific autoimmune manifestations. Illegal injection of these substances could lead to serious local and systemic complications, even to death. These cases represent another model of Autoimmune/inflammatory Syndrome Induced by Adjuvants (ASIA). The use of these substances should be prohibited.

Still's disease, lupus-like syndrome, and silicone breast implants. A case of 'ASIA' (Shoenfeld's syndrome).

In recent years, four conditions, siliconosis, Gulf War syndrome (GWS), macrophagic myofasciitis syndrome (MMF) and post-vaccination phenomena, were linked to a previous exposure to an adjuvant, suggesting a common denominator, and it has been proposed to incorporate comparable conditions under a common syndrome entitled Autoimmune/inflammatory Syndrome Induced by Adjuvants (ASIA). We report a case of a female who at the age of 11 years was diagnosed with Still's disease. At the age of 22 she underwent silicone breast implants and presented with a transient lupus-like syndrome. Then, at 25 years old she had a severe activation of Still's disease in association with rupture of silicone breast implants. When the prostheses were removed, the clinical picture improved. This case fulfills the criteria for ASIA and complements seven previous reports of Still's disease in association with silicone breast implants.

Antimitochondrial antibodies in patients with chronic hepatitis C virus infection: description of 18 cases and review of the literature.

To describe the clinical and immunologic patterns of disease expression of patients with chronic hepatitis C virus (HCV) infection and positive antimitochondrial antibodies (AMA). We investigated the presence of AMA in 237 consecutive HCV patients with extrahepatic manifestations from an International Registry. AMA were detected by indirect immunofluorescence in triple rat tissue (liver, stomach and kidney), aceton-fixed criosections and FITC-conjugated rabbit anti-human immunoglobulins. We found positive AMA in 18 (8%) out of 237 HCV patients. All patients were female with a mean age at protocol inclusion of 65.8 years (ranging from 37 to 87 years). Twelve (67%) patients fulfilled classification criteria for systemic autoimmune diseases (SAD), including Sjögren's syndrome (n = 7), systemic sclerosis (n = 3) and systemic lupus erythematosus (n = 2). Fourteen (78%) of the HCV-AMA patients presented at least one of the highly suggestive characteristics of primary biliary cirrhosis (PBC): 9 (50%) had a specific M2 pattern, 6 (33%) had more than twice normal levels of alkaline phosphatase, 5 (28%) had raised IgM levels and 4 (22%) a histological pattern compatible with PBC. Five (28%) patients developed neoplasia after detection of AMA. Seven (39%) patients died, due to neoplasia (n = 4), cirrhotic complications (n = 2) and hepatopulmonary syndrome (n = 1). We describe a subset of HCV patients with positive AMA who presented a broad spectrum of clinical features, including liver, autoimmune and neoplasic manifestations. Two-thirds of these patients presented an associated SAD, mainly Sjögren's syndrome or systemic sclerosis, together with a high frequency of multiple autoantibodies and an increased prevalence of cirrhosis and neoplasia.

The impact of gender on clinical manifestations of primary antiphospholipid syndrome.

The objective of the study was to determine the clinical differences at diagnosis and during follow-up between male and female patients with primary antiphospholipid syndrome (PAPS). We analysed 68 patients, 30 males and 38 females diagnosed and followed between 1990 and 2003. Patients with antiphospholipid syndrome associated with systemic lupus erythematosus at onset and during follow-up were excluded. The mean age at diagnosis was 31.4 +/- 11 years in males and 35.7 +/- 11 years in females (NS). The follow-up after diagnosis was 8.7 +/- 3.1 years in males and 9.2 +/- 2.9 years in females (NS). We did not find significant differences between the two groups with respect to venous and arterial thrombosis. However, in female patients, stroke was more prevalent than in male patients (12/38 versus 3/30, P = 0.03). In contrast, we found a significant prevalence of severe gastrointestinal complications in male compared to female patients (7/30 versus 1/38, P = 0.009). One male patient died due to catastrophic antiphospholipid syndrome. This study suggests that clinical course in patients with PAPS may be different with significant prevalence of central nervous system involvement in females and gastrointestinal involvement in males. Factors such as accelerated atherosclerosis, hormones, related to gender could be the explanation of these findings.

Systemic autoimmune diseases co-existing with chronic hepatitis C virus infection (the HISPAMEC Registry): patterns of clinical and immunological expression in 180 cases.

OBJECTIVES: To describe the clinical and immunologic characteristics of a large series of patients with systemic autoimmune diseases (SAD) associated with chronic hepatitis C virus (HCV) infection. METHODS: We analysed 180 patients diagnosed with SAD and chronic HCV infection seen consecutively at our centres during the last 10 years. The clinical and immunological patterns of disease expression were compared with 180 SAD-matched patients without chronic HCV infection. RESULTS: A total of 180 HCV patients fulfilled the classification criteria for the following SAD: Sjogren's syndrome (n = 77), systemic lupus erythematosus (n = 43), rheumatoid arthritis (n = 14), antiphospholipid syndrome (n = 14), polyarteritis nodosa (n = 8) and other SAD (n = 24). One hundred and thirty (72%) patients were female and 50 (28%) male, with a mean age at SAD diagnosis of 50 years. The main immunologic features were antinuclear antibodies in 69% of patients, cryoglobulinaemia in 62%, hypocomplementaemia in 56% and rheumatoid factor (RF) in 56%. Compared with the SAD-matched HCV-negative group, SAD-HCV patients presented a lower prevalence of females (P = 0.016), an older age at SAD diagnosis (P = 0.039) and a higher prevalence of vasculitis (P < 0.001) and neoplasia (P < 0.001). Immunologically, SAD-HCV patients presented a lower prevalence of antinuclear (P = 0.036), anti-extractable nuclear antigen (P = 0.038) and anti-DNA (P = 0.005) antibodies, and a higher frequency of RF (P = 0.003), hypocomplementaemia (P < 0.001) and cryoglobulins (P < 0.001). CONCLUSIONS: In comparison with an SAD-matched HCV-negative population, SAD-HCV patients were older and more likely to be male, with a higher frequency of vasculitis, cryoglobulinaemia and neoplasia. This complex pattern of disease expression is generated by a chronic viral infection that induces both liver and autoimmune disease.

Controlled clinical trial of IV cyclophosphamide versus IV methylprednisolone in severe neurological manifestations in systemic lupus erythematosus.

BACKGROUND: Severe neurological involvement in systemic lupus erythematosus (NPSLE) is one of the most dreadful complications of the disease. OBJECTIVE: To identify the best drug, dose, and treatment. PATIENTS AND METHODS: The study was a controlled clinical trial at two tertiary care centres of patients with SLE according to the ACR criteria, with incident (no more than 15 days) onset of severe NP manifestations such as seizures, optic neuritis, peripheral or cranial neuropathy, coma, brainstem disease, or transverse myelitis. Induction treatment with 3 g of IV methylprednisolone (MP) followed by either IV monthly cyclophosphamide (Cy) versus IV MP bimonthly every 4 months for 1 year and then IV Cy or IV MP every 3 months for another year. The primary end point was response to treatment: at least 20% improvement from basal conditions on clinical, laboratory, or specific neurological testing variables. RESULTS: Overall, a response rate of 75% was observed. Of the 32 patients studied, 18/19 receiving Cy and 7/13 receiving MP responded to treatment (p<0.03). CONCLUSIONS: Cy seems to be more effective than MP in the treatment of acute, severe NPSLE.

Prolactin effect on CD69 and CD154 expression by CD4+ cells from systemic lupus erythematosus patients.

OBJECTIVE: The aim was to explore the role of prolactine (PRL) in the lymphocyte activation process in active and inactive systemic lupus erythematosus (SLE) patients in an in vitro model. METHODS: Peripheral blood mononuclear cells (PBMNC) were isolated from SLE patients and healthy individuals. The mRNA for prolactine and its receptor, obtained by standard techniques with an appropriate primer, were subjected to PCR and visualised. The PBMC were cultured with: a) medium alone as a negative control, b) unspecific mitogen as a positive control (PMA-ionomycin for CD154 or concanavalin A for CD69), c) PRL alone, d) mitogen plus PRL, e) mitogen plus antibody anti-PRL (1:50) and f) mitogen plus an unrelated antibody. Then CD69 and CD154 were determined by flow cytometry analysis. RESULTS: Twelve inactive and 15 active SLE patients were studied. 25% of the active patients displayed hyperprolactinemia. Under basal conditions, CD69 expression was associated with disease activity. In contrast, CD154 did not show this association. The PBMNC activated in vitro were capable of producing and secreting prolactine as measured by mRNA and Nb2 assay. In the same way the mRNA for prolactine receptor was visualized. Cells from SLE patients cultivated with PRL alone did not display increased CD69 or CD154 expression. The addition of PRL to the unspecific stimulated culture did not have an additive effect. In contrast, the addition of antibodies against PRL, in order to block the autocrine prolactine, resulted in a striking reduction in CD69 and CD154 expression. CONCLUSIONS: PRL is produced and secreted by the immune cell and acts just after the first trigger signal of activation in an autocrine way. The expression of CD69 and CD154 molecules depend partially on the prolactine.

Clinical spectrum of males with primary antiphospholipid syndrome and systemic lupus erythematosus: a comparative study of 73 patients.

The objective of this study was to compare the clinical findings, laboratory data, functional outcome and chronic damage in male patients with primary antiphospholipid syndrome (PAPS) and systemic lupus erythematosus (SLE). We studied 29 male patients with PAPS and 44 with SLE. Clinical findings, laboratory data, lupus damage index (SLICC/ACR DI), and functional outcome in PAPS, were analysed in each group. The mean age at diagnosis was 29.8 +/- 10.4 years in patients with PAPS and 26 +/- 10.1 years in SLE patients. The duration of disease was 4.5 +/- 2.6 versus 5.2 +/- 3.8 years in patients with PAPS and SLE, respectively (P = NS). In patients with PAPS the most frequent clinical manifestations were venous thrombosis, thrombocytopenia, and pulmonary thromboembolism. Patients with SLE had joint, skin and renal involvement more frequently than those with PAPS (P = 0.0001). All PAPS patients had anticardiolipin antibodies (aCL), and 14 patients (48%) had lupus anticoagulant (LA). All SLE patients had antinuclear antibodies (ANAs). Anti-dsDNA antibodies were positive in 39% of SLE patients. Five patients died: one with 'catastrophic' APS and four with SLE. SLICC/ACR-DI score in SLE patients was 1.9 (SD = 1). In PAPS patients poor functional outcome was due to myocardial infarction, pulmonary thromboembolism, stroke and mesenteric thrombosis. Lupus nephritis was the principal organ damage in SLE. In conclusion, in male patients with PAPS and SLE, the clinical manifestations were significantly different. Arterial thrombosis was the major cause of functional impairment and permanent organ damage in PAPS. Renal involvement was the major cause of chronic damage in SLE.

Increased carotid artery intima-media thickness may be associated with stroke in primary antiphospholipid syndrome.

OBJECTIVE: To investigate the prevalence and clinical significance of carotid artery intima-media thickness (IMT) in patients with primary antiphospholipid syndrome (APS). METHODS: 28 patients with primary APS with at least a five year follow up, and 28 healthy subjects, matched by age and sex, were included in the study. Colour Doppler with high resolution B mode carotid ultrasonography and spectral analysis were performed in patients and controls. Information on cardiovascular risk factors and the clinical course were collected. RESULTS: The mean (SD) age of patients and controls (12 male, 16 female in each group) was 40 (8.5) years; the mean (SD) disease duration 7.7 (3) years. Carotid artery IMT was found in 23/28 patients (2.6 (1.14) mm) and 7/28 controls (1.2 (0.44)) (p=0.0001). A decrease in the lumen diameter was also found in 11/28 patients with primary APS without carotid atherosclerotic plaque, and 2/28 controls (p=0.004). Hyperlipidaemia, diabetes, smoking, obesity, and hypertension were not associated with carotid artery IMT. Patients with carotid artery IMT had arterial vascular disease more often than patients without: 9/23 v 0/5 (p<0.009). These patients had stroke (seven patients), myocardial infarction (one), and mesenteric thrombosis (one). Subjects with IMT had a threefold higher risk for stroke than those without IMT (95% CI 0.78 to 14.3). CONCLUSIONS: Patients with primary APS have a high prevalence of carotid artery IMT and a decreased lumen diameter. IMT in primary APS may be associated with stroke. Patients with primary APS with IMT must be considered as carriers of atherosclerosis.

Neurological involvement as a poor prognostic factor in catastrophic antiphospholipid syndrome: autopsy findings in 12 cases.

CAPS is an uncommon disease, characterized by clinical evidence of multiple organ involvement and histopathological evidence of multiple vessel occlusions, in patients with either primary or secondary antiphospholipid syndrome. The present series describes the clinical manifestations and autopsy findings of 12 patients with CAPS. Neurological involvement was considered the main cause of death in all of them. CNS pathology revealed thrombotic microangiopathy as well as small and large vessel occlusions in several brain areas. Neurological involvement in CAPS is strongly associated with thrombotic microangiopathy and should be considered a potential cause of death in these patients.