Bone mineral density in adults with Down syndrome: a cross-sectional study in a Brazilian sample.

BACKGROUND: We aim to investigate the relationship between bone mass in a sample of Brazilian individuals with DS and handgrip strength, body mass index (BMI) and physical exercise. METHODS: Dual-energy X-ray emission densitometry analysis of bone mass in 26 individuals with DS (8 men and 18 women with a mean age of 30.7 ± 10.3 years) was conducted. Additionally, weight and height were measured to determine BMI, palmar grip strength was measured using a Jammar dynamometer®, and physical activity was classified using the International Physical Activity Questionnaire (IPAQ). RESULTS: In this sample, 2/15 (13.3%) individuals with age between 18 to 29 years had low BMD in the spine; 2/8 (25%) of those with age between 30 and 39 years also had low BMD in the spine and 2/3 (66.6%) with age ≥40 had low BMD in the femur. There were significant correlations between palmar grip strength and Z femoral neck score in women (P = 0.02) and between BMI and Z femoral neck score in men (P = 0.04). All other correlations lacked statistical significance (P > 0.05). CONCLUSIONS: Brazilian patients with DS showed a high prevalence of low bone mass. Traditional factors such as muscle strength, BMI and physical activity appear to have little effect on bone mineral density in this population.

Telemedicina en la monitorización de pacientes con demencia: una perspectiva de cuidadores brasileños / Telemedicine in the monitoring of patients with dementia: a Brazilians caregivers’s perspective

Introducción: La pandemia de SARS-CoV-2 (COVID-19) requirió una atención ampliada a través de la telemedicina en una variedad de especialidades médicas. El objetivo del estudio es analizar las preferencias de los cuidadores de pacientes con demencia sobre el uso de la telemedicina en el seguimiento de estos pacientes. Sujetos y métodos: Los cuidadores y familiares a cargo de pacientes con demencia completaron un cuestionario autoaplicable en línea, que se distribuyó en grupos de Facebook y WhatsApp entre abril y octubre de 2020. Resultados: Se obtuvieron 130 respuestas; la mayoría de los cuidadores eran mujeres (83,8%), de las cuales el 60% eran hijas de pacientes. La mayoría de los pacientes también eran mujeres (75,3%). La enfermedad de Alzheimer causó demencia en el 73% de los casos. Internet era accesible para el 98% de los encuestados, pero menos de la mitad (33,8%) había utilizado la telemedicina al menos una vez. Aunque el 66,9% de los cuidadores estaría dispuesto a utilizar la telemedicina si el médico se la ofreciera, el 63% cree que el coste de las videoconsultas debería ser inferior al de las presenciales. Durante la pandemia de COVID-19, el 85,3% de los cuidadores necesitó contactar con el médico: poco más de la mitad de estas consultas fueron presenciales (52,2%), pero se prefirió la telemedicina (62,3%) si era posible. Conclusión: Los cuidadores aceptaron y se interesaron por la telemedicina como herramienta para el seguimiento de los pacientes con demencia, especialmente los que tenían dificultad para caminar.(AU)

Introduction: The SARS-CoV-2 (COVID-19) pandemic warranted expanded care via telemedicine across a breath of medical specialties. The aim of the study is to analyze the preferences of caregivers of patients with dementia on the use of telemedicine in monitoring these patients. Subjects and methods: Caregivers and family members responsible for patients with dementia completed an online self-applicable questionnaire, which was distributed in Facebook and WhatsApp groups between April and October 2020. Results: 130 responses were obtained; most caregivers were women (83.8%), of whom 60% were daughters of patients. Most patients were also women (75.3%). Alzheimer’s disease caused dementia in 73% of cases. The Internet was accessible to 98% of respondents, yet less than half (33.8%) had used telemedicine at least once. Although 66.9% of caregivers would be willing to use telemedicine if the doctor offered it, 63% believed that the cost of video consultations should be lower than those face-to-face. During the COVID-19 pandemic, 85.3% of caregivers needed to contact the physician: just over half of these consultations were face-to-face (52.2%), but telemedicine was preferred (62.3%) if possible. Conclusion: Caregivers accepted and were interested in telemedicine as a tool for monitoring patients with dementia, especially those with walking difficulties.(AU)

Telemedicine in the monitoring of patients with dementia: a Brazilians caregivers's perspective. / Telemedicina en la monitorización de pacientes con demencia: una perspectiva de cuidadores brasileños.

INTRODUCTION: The SARS-CoV-2 (COVID-19) pandemic warranted expanded care via telemedicine across a breath of medical specialties. The aim of the study is to analyze the preferences of caregivers of patients with dementia on the use of telemedicine in monitoring these patients. SUBJECTS AND METHODS: Caregivers and family members responsible for patients with dementia completed an online self-applicable questionnaire, which was distributed in Facebook and WhatsApp groups between April and October 2020. RESULTS: 130 responses were obtained; most caregivers were women (83.8%), of whom 60% were daughters of patients. Most patients were also women (75.3%). Alzheimer's disease caused dementia in 73% of cases. The Internet was accessible to 98% of respondents, yet less than half (33.8%) had used telemedicine at least once. Although 66.9% of caregivers would be willing to use telemedicine if the doctor offered it, 63% believed that the cost of video consultations should be lower than those face-to-face. During the COVID-19 pandemic, 85.3% of caregivers needed to contact the physician: just over half of these consultations were face-to-face (52.2%), but telemedicine was preferred (62.3%) if possible. CONCLUSION: Caregivers accepted and were interested in telemedicine as a tool for monitoring patients with dementia, especially those with walking difficulties.

TITLE: Telemedicina en la monitorización de pacientes con demencia: una perspectiva de cuidadores brasileños.Introducción. La pandemia de SARS-CoV-2 (COVID-19) requirió una atención ampliada a través de la telemedicina en una variedad de especialidades médicas. El objetivo del estudio es analizar las preferencias de los cuidadores de pacientes con demencia sobre el uso de la telemedicina en el seguimiento de estos pacientes. Sujetos y métodos. Los cuidadores y familiares a cargo de pacientes con demencia completaron un cuestionario autoaplicable en línea, que se distribuyó en grupos de Facebook y WhatsApp entre abril y octubre de 2020. Resultados. Se obtuvieron 130 respuestas; la mayoría de los cuidadores eran mujeres (83,8%), de las cuales el 60% eran hijas de pacientes. La mayoría de los pacientes también eran mujeres (75,3%). La enfermedad de Alzheimer causó demencia en el 73% de los casos. Internet era accesible para el 98% de los encuestados, pero menos de la mitad (33,8%) había utilizado la telemedicina al menos una vez. Aunque el 66,9% de los cuidadores estaría dispuesto a utilizar la telemedicina si el médico se la ofreciera, el 63% cree que el coste de las videoconsultas debería ser inferior al de las presenciales. Durante la pandemia de COVID-19, el 85,3% de los cuidadores necesitó contactar con el médico: poco más de la mitad de estas consultas fueron presenciales (52,2%), pero se prefirió la telemedicina (62,3%) si era posible. Conclusión. Los cuidadores aceptaron y se interesaron por la telemedicina como herramienta para el seguimiento de los pacientes con demencia, especialmente los que tenían dificultad para caminar.

Pre-donation deferral of blood donors in a Brazilian blood bank: a 10-year experience.

OBJECTIVES: To study the main causes of pre-donation blood donors' deferral in a Brazilian blood bank. BACKGROUND: Blood donor selection is the most important process to maintain transfusion safeness. Pre-donation deferral aims to avoid the transmission of infectious diseases in the serological window, as well as to preserve blood donors' health. METHODS: This was a retrospective study undertaken in a single blood centre in Curitiba, Brazil, taking into account the number of blood donations per year, the number of annual donations by gender and the total number of blood donors deferred annually prior to blood donation from 1 January 2007 to 31 December 2016. RESULTS: Pre-donation blood donors' deferral ranged from 12·1 to 15·7% of donors. The main reason was related to donors' health (22·5-51·4%) followed by behavioural risk (17·6-29%). Issues related to blood donors' health diminished, and those related to behavioural risk increased with time. Blood donors deferred because of anaemia diminished with time and were more common in women than men (P < 0·001). CONCLUSIONS: Pre-donation blood donors' deferral ranges from 12·1 to 15·7% in our region. The most common cause was blood donors' health followed by behavioural risk. Anaemia was more common in women. Knowing this specific population better could avoid wasting blood bags and help to minimise costs and still maintain transfusion safety.

Complement Factor H as a potential atherogenic marker in chronic Chagas' disease.

We aimed to investigate the association between plasma levels of complement Factor H (FH) with cardiac involvement, inflammatory and cardiometabolic parameters in patients with chronic Chagas' disease (CD). FH plasmatic levels were determined in 80 chronic CD patients. Glycaemic index, lipidogram (high-density lipoprotein cholesterol HDL-C, low-density lipoprotein cholesterol LDL-C, triglycerides and total cholesterol) and Ultrasensitive C-Reactive Protein (uCRP) values were obtained from medical records. Height, weight, body mass index (BMI) blood pressure and left ventricular ejection fraction (LVEF) were obtained from echocardiography examinations. Comparisons between chronic CD clinical forms were performed using Mann-Whitney test and correlation Spearman's test. FH levels were correlated positively with triglycerides (P = .001, r = .39), LDL-C (P = .009, r = .3), cholesterol (P = .02, r = .28), uCRP (P = .029, r = .31) and BMI (P = .008, r = .34); and negatively with HDL-C (P = .03, r = -.25) levels. Dyslipidemic patients showed higher FH levels compared to normolipidemic, although no difference for FH levels was observed between chronic CD clinical forms. Alternative pathway of complement may be a link between immune response and metabolic disorders, with important immunoregulatory role in chronic CD.

Anti-nuclear antibodies in patients with breast cancer.

To study the prevalence of anti-nuclear antibodies (ANA) in breast cancer patients and its association with tumour characteristics. Ninety-one patients with breast mass detected by image studies and assigned to conduct diagnostic biopsy and eventual surgical treatment were studied for demographical, tumour data and presence of ANA. Serum of positive ANA patients was screened for the extractable nuclear antigen (ENA) profile. As comparison, 91 healthy individuals matched for age and from the same geographical area were included. In this sample 72 of 91 (79·1%) had malignant lesions (83% ductal infiltrative carcinoma). ANA was positive in 44·4% of patients with malignant tumour and in 15·7% of those with benign lesions (malignant versus benign with P = 0·03). Controls had ANA positivity in 5·4%, and when compared with tumour samples showed P < 0·0001. The most common immunofluorescence pattern was a fine dense speckled pattern. In the ANA-positive patients with malignant lesions, seven had positivity for ENA profile (three for anti-RNP and anti-Sm, one for just anti-RNP, two for anti-Ro and anti-La e two for just anti-La). It was not possible to associate ANA positivity with tumour histological characteristics or staging or with patient's age. A negative association of ANA with hormonal (oestrogen or oestrogen plus progesterone) receptor status was found (P = 0·01). In this sample, there was a high prevalence of ANA positivity in breast cancer patients with a negative association with the presence of hormonal receptors. More studies are needed to understand the real value of this finding.

The lupus patient with positive rheumatoid factor.

Background Patients with systemic lupus erythematosus (SLE) may form clusters with clinical manifestations and autoantibodies. Objective The objective of this report is to study whether SLE patients with positive rheumatoid factor (RF) have a special clinical and/or serological profile. Methods A retrospective study of 467 SLE patients seen at a single rheumatology unit was conducted. Epidemiological data (age, gender, age at disease onset, ethnic background and tobacco use), clinical data (malar rash, photosensitivity, oral ulcers, discoid lesions, serositis, glomerulonephritis, convulsions, psychosis, hemolytic anemia, leukopenia, lymphocytopenia, arthritis and hypothyroidism) and serological profile (anti-dsDNA, anti-Ro/SS-A, anti-La/SS-B, anti-RNP, anti-Sm, IgG aCL, IgM aCL, lupus anticoagulant, direct Coombs and RF) were collected. Patients with positive and negative RF were compared. Results RF was found in 24.9% of the sample. In univariate analysis, RF was positively associated with butterfly rash ( p = 0.04), anti-Ro ( p = 0.03), anti-Sm antibodies ( p = 0.01) and hypothyroidism ( p = 0.01) and negatively associated with glomerulonephritis ( p = 0.003). Logistic regression showed that only glomerulonephritis ( p = 0.03; OR = 0.45; 95% CI = 0.21-0.93) and anti-Ro ( p = 0.009; OR = 2.3; 95% CI = 1.24-4.57) were independent associations. Conclusion In our sample RF was associated with protection from glomerulonephritis and with higher prevalence of anti-Ro antibodies.

Anti-parietal cell antibodies in patients with autoimmune thyroid diseases.

BACKGROUND: Autoimmune thyroid disease (ATD) patients may have a higher prevalence of anti-parietal cell antibodies (APCA) than normal population. OBJECTIVE: To study the prevalence of APCA in a cohort of ATD patients to know its association with patient's clinical profile and gastrointestinal complaints. METHODS: APCA was sought for by indirect immunofluorescence test in 243 ATD patients: 136 (55.9%) with Graves' disease and 107 (44.0%) with Hashimoto's thyroiditis. A structured questionnaire for gastrointestinal symptoms, previous history of thrombosis, arthralgia and other autoimmune diseases in the patients and their families was applied. Positive and negative APCA individuals were compared. Positive patients were invited to perform upper gastrointestinal endoscopy and biopsy of duodenum segments. Sera from 100 healthy individuals from the same geographic area were used as controls. RESULTS: APCA was present in 20.1% (49/243) of ATD patients: 21.3% (29/136) in the Graves' sample and 18.6% (20/107) in the Hashimoto's sample (p = 0.61). Patients with positive APCA had more anemia (p = 0.03; OR = 2.89; 95% CI = 1.03-8.07) and less heartburn (p = 0.01; OR = 0.4; 95% CI = 0.20-0.83). Among the group of 49 APCA-positive patients, 24 agreed with upper endoscopy and it was found that 54.1% had atrophic gastritis. CONCLUSIONS: There is a high prevalence of positive APCA in ATD patients. APCA are more common in those with anemia and less common in those with complaints of heartburn. Almost half of positive APCA patients had atrophic gastritis.

Mannose-binding lectin serum levels in patients with systemic lupus erythematosus: association with thrombocytopaenia and seizure.

The complement system contributes to the pathogenesis of systemic lupus erythematosus (SLE). Mannose-binding lectin (MBL) is a key molecule of the lectin pathway of complement and seems to be related to the clinical manifestations of this disease. We evaluated the serum levels of MBL and its relationship with disease onset and clinical findings in SLE patients. Serum samples were analysed in 195 patients and 145 healthy controls from southern Brazil. Patients with high MBL levels (above 2000 ng/ml) showed a significant increase in the frequency of thrombocytopaenia ( p = 0.007; OR = 2.71; 95% CI = 1.32-5.55); and seizures ( p = 0.034; OR = 2.61; 95% CI = 1.07-6.37). A positive correlation between disease activity and MBL levels (>2000 ng/ml; p = 0.031, rho = 0.279) as well as of MBL concentration with accumulated organ damage ( p = 0.021; rho = 0.232) was observed. Our results suggest a role for MBL in the development of clinical manifestations such as thrombocytopaenia and seizures in SLE patients. These findings corroborate the participation of the lectin pathway of complement in the pathophysiologic mechanisms underlying clinical manifestations of SLE.

Lymphocytopenia is associated with anti-Beta-2 glycoprotein-1 in patients with 220 systemic lupus erythematosus.

BACKGROUND: Anti B2-Glicoprotein 1 (B2-GPI) is an antiphospholipid antibody that may be present in primary or secondary antiphospholipid syndrome (APS). Systemic Lupus erythematosus (SLE) is the main disease associated with secondary APS. OBJECTIVE: To study the prevalence of anti B2-GPI in SLE patients. METHODS: Anti B2-GPI (IgM/IgG) was studied by ELISA in 88 patients with SLE of both genders; 18.6% of which with secondary APS. Charts were reviewed for clinical and serological profile. RESULTS: Anti B2-GPI was present in 18.6% of the whole sample and in 29.4% of those with secondary APS. At univariated analysis, the presence of anti B2-GPI was more common in patients with serositis (p=0.04), lymphocytopenia (p=0.003) and anti cardiolipin (aCl) IgM antibodies (p=0.04). In a logistic regression study, only the associations with lymphocytopenia (OR=8.2; 95%CI=2.1-39.3) and aCl IgM (p=0.04; OR=3.4; 95%CI=1.05-11.1) remained significant. CONCLUSION: There is a 18.6% prevalence of positive anti B2-GPI in SLE population that is associated with the presence of aCl IgM and lymphocytopenia.

BF*F allotype of the alternative pathway of complement: A marker of protection against the development of antiphospholipid antibodies in patients with systemic lupus erythematosus.

BACKGROUND: B factor (BF) from the alternative complement pathway seems to participate in the pathophysiology of systemic lupus erythematosus (SLE) and antiphospholipid syndrome (APS). OBJECTIVE: To study the allotypic variability of BF in SLE and their associations with clinical and autoantibodies profile. METHODS: BF allotypes were determined by high-voltage agarose gel electrophoresis, under constant cooling, followed by immunofixation with anti-human BF antibody, in 188 SLE patients and 103 controls. Clinical and serological data were obtained from medical examination and records. RESULTS: No significant differences of BF variants between patients and controls were found, neither in relation to epidemiologic or clinical manifestations. Associations of phenotype BF SS07 and allotype BF*S07 were found with anticardiolipin IgM (aCl-IgM) antibodies (p = 0.014 and p = 0.009 respectively), but not with aCl-IgG, lupus anticoagulant (LA), anti ß2GPI or clinical APS. A significant decrease in BF*F allotype (p = 0.043) and BF SF phenotype (p = 0.018) was detected in patients with anti-phospholipid antibodies as a whole (aCl-IgG, aCl-IgM, LA and anti ß2GPI). CONCLUSIONS: There is a link between phenotype BF SS07 and allotype BF*S07 with aCl-IgM in SLE patients; BF*F allotype could be considered a marker of protection against the development of antiphospholipid antibodies in these patients.

Association of complement factor B allotypes and serum biomarkers in rheumatoid arthritis patients and their relatives.

The aim of the study was to investigate the allotypic variability of complement factor B (BF) in patients and relatives with rheumatoid arthritis (RA) and its association with serological biomarkers and clinical features of the disease. BF allotypes were determined by high-voltage agarose gel electrophoresis in serum samples of 180 patients with RA, 198 relatives and 98 controls from Southern Brazil. Anticyclic citrullinated peptide (anti-CCP), antimutated citrullinated vimentin (anti-MCV) and IgA-rheumatoid factor (RF) were determined by ELISA and IgM-RF by latex agglutination in all samples. No significant differences were found in the allotypic variants of BF between patients with RA, relatives and controls, nor associations with gender and age of RA onset. BF*S07 allotype was significantly associated with extra-articular manifestations (EAMs; Secondary Sjögren Syndrome, pneumonitis, rheumatoid nodules) in patients with RA (P = 0.02; OR = 6.62). Patients with phenotype BF F had lower positivity for anti-MCV biomarker (P = 0.02; OR = 0.22) and those with allotype BF*S had higher prevalence of this autoantibody (P = 0.02; OR = 3.77). An increased frequency of RF-IgA was detected in relatives of patients with RA with BF FS07 phenotype (P = 0.02; OR = 7.78). Complement BF variability did not influence the development of RA in the studied patients, but BF variants may act as markers of disease prognosis, such as development of EAMs, corroborating with the role of the alternative pathway in the pathogenesis of RA.

Rheumatic manifestations in brazilian patients with AIDS.

INTRODUCTION: Patients with AIDS (acquired immunodeficiency syndrome) may have rheumatic complaints such as arthritis and arthralgia, dry eyes, increased salivary glands, lower back pain, enthesitis etc. Autoantibodies like ANA (antinuclear antibody) and RF (rheumatoid factor) may also be present. OBJECTIVE: To study the prevalence of rheumatic complaints in AIDS patients and correlate them with the presence of ANA and RF. METHODS: We studied 69 patients with AIDS (28.9% women and 71.0% men) with a mean age of 40.8 ± 8.9 years, median disease duration of 60 months, for rheumatic complaints, ANA, ENA-6 (anti-Ro, anti-La, anti-Sm, anti-RNP, anti-Scl70 and anti-Jo1) and RF. We collected demographic data, CD4+ and CD8+ cell count and values of viral load. RESULTS: Arthralgia was present in 39.1%, sicca symptoms in 21.7%, inflammatory lumbar pain in 13.4%, enthesopathy in 6.6%, parotid enlargement in 1.4%, RF in 10.1% and ANA in 8.6%. All patients were negative for ENA-6. ANA was more common in older patients (p = 0.03) and in those with higher viral load (p = 0.006). No association was found with the presence of RF. CONCLUSIONS: The most common manifestation in this context was arthralgia. ANA presence was associated with age of the patients and viral load.

Anti-CCP antibodies in Brazilian children and adults with juvenile idiopathic arthritis.

Patients with juvenile idiopathic arthritis (JIA) may need further care in the adult clinic as this disease frequently has continuous inflammatory activity during adult life. To identify which pediatric JIA patients will need continuing care into adulthood. We compared the clinical, serological, and demographic data of 45 JIA patients followed up by the pediatric clinic to those of 49 JIA patients in the adult rheumatology clinic. Patients in the adult clinic have older age at disease onset (p < 0.0001) and higher prevalence of positive anti-cyclic citrullinated peptide (CCP) (p = 0.05). No differences were observed in JIA form, presence of rheumatoid factor (RF), uveitis, and gender. Anti-CCP and older age at disease onset may identify pediatric JIA patients that will need further care in the adult clinic.

Low hepatitis B vaccine response in children with Down syndrome from Brazil.

BACKGROUND: It has been reported that vaccination against hepatitis B is less effective among people with Down syndrome than in the general population. We aimed to evaluate the rate of seroconversion to hepatitis B vaccine in children with Down syndrome from Brazil. METHODS: A total of 120 people with Down syndrome were included. All of them received the vaccine at intervals of 0, 30 and 180 days and serum samples were tested for the presence of antibodies to the hepatitis B surface antigen (anti-HBs) 30 days after the last dose. RESULTS: In the studied group, 58.3% (70/120) were male and 41.7% (50/120) female, with the median age of 5 years (range 2-15 years). Fifty-eight of 120 (48.3%) developed anti-HBs after vaccination. No association was found between gender and/or age and vaccine response. CONCLUSIONS: The low rate of seroconversion in response to hepatitis B vaccine suggests that all patients with Down syndrome immunized against hepatitis B should be followed and monitored by clinicians.

Spectrum of autoantibodies for gastrointestinal autoimmune diseases in systemic lupus erythematosus patients.

BACKGROUND: Organ-specific autoimmune diseases may appear in patients with systemic lupus erythematosus (SLE). Gastrointestinal symptoms are well documented in SLE and may be similar to those related to autoimmune gastrointestinal diseases. OBJECTIVE: Our aim was to search for gastrointestinal organ-specific autoantibodies in 194 patients with systemic lupus and 103 healthy controls from Southern Brazil. Methods Anti-endomysium antibodies (IgA-EmA), anti-gastric parietal cells (GPC) antibodies, anti-smooth muscle antibodies (ASMA), anti-mitochondrial antibodies (AMA) and anti-LKM-1 (liver-kidney microsomal) were searched for using indirect immunofluorescence in the sera of patients and controls. RESULTS: The total positivity of antibodies in SLE patients was 14.4% (28/194) and differed significantly from healthy individuals (0.97%; p<0.001). IgA-EmA was more common in lupus patients than in controls (11/194; p=0.009), and one of these patients had dermatitis herpetiformis. Clinical association revealed that IgA-EmA was more common in SLE patients with discoid lesions. The frequency of anti-GPC (p=0.10), ASMA (p=0.16) and AMA (p=0.55) did not differ significantly between groups. No patient presented LKM-1 autoantibodies. One patient presenting anti-GPC was diagnosed with atrophic gastritis and pernicious anemia. CONCLUSION: Only IgA-EmA was significantly associated with lupus and with the presence of discoid lesions. Until now, no obvious association with celiac disease has been found.

Antinucleosome in systemic lupus erythematosus. A study in a Brazilian population.

Antinucleosome antibodies have been found with variable prevalence in systemic lupus erythematosus (SLE) and were associated with more severe disease. This research aims to study the prevalence of antinucleosome antibodies in a sample of Brazilian adult SLE patients and their association with clinical findings and disease activity. Ninety-two adult patients (81 females and 11 males, with mean age of 37.29 ± 10.98 years) with SLE were studied for clinical and antibody profile, disease activity by SLE disease activity index (SLEDAI), and presence of antinucleosome antibodies by ELISA. The prevalence of antinucleosome antibodies was 61.9% (mean titer, 87.8 ± 62.6 U). No relationship was found of antinucleosome presence and any of the studied clinical features. A positive association was detected with anti-DNA (p = 0.001) and SLEDAI (p < 0.0001), but not with anti-Sm, anti-Ro, anti-La, and anti-RNP. No specific disease feature could be associated with the presence of antinucleosome; however, a positive relationship was detected with disease activity measured by SLEDAI and with anti-DNA presence.

Circulating levels of mannan-binding lectin (MBL) and MBL-associated serine protease 2 in endemic pemphigus foliaceus.

Endemic pemphigus foliaceus (EPF) is an autoimmune disease, which occurs in Brazil and other regions of South America. Mannose-binding lectin (MBL) and MBL-associated serine protease (MASP-2) play a key role in innate immunity, and its deficiency has been related to increased susceptibility to infection and autoimmune diseases. MBL and MASP-2 serum levels were measured in 114 patients with EPF and in 100 healthy individuals in Brazil. MBL and MASP-2 levels were measured by sandwich assays (time-resolved immunofluorimetic assay) using monoclonal antibodies. No difference was observed in the MBL level in patients with EPF compared with controls [mean +/- SEM 1230.07 +/- 132.18 ng/mL (median 789.0 ng/mL) vs. 1036.98 +/- 117.99 ng/mL (median 559.5 ng/mL), P = 0.32]. Non-significant lower MASP-2 levels were observed in EPF [274.34 +/- 15.66 ng/mL (median 239.5 ng/mL ) vs. 304.72 +/- 15.28 ng/mL [median 261.0 ng/mL ), P = 0.06]. MBL deficiency (< 10 ng/mL) or MASP-2 deficiency (< 100 ng/mL) did not differ significantly between patients and controls. These data indicate that MBL and MASP-2 deficiency are not associated with susceptibility to EPF.