The traditional concept that effector T helper (Th) responses are mediated by Th1/Th2 cell subtypes has been broadened by the recent demonstration of two new effector T helper cells, the IL-17 producing cells (Th17) and the follicular helper T cells (Tfh). These new subsets have many features in common, such as the ability to produce IL-21 and to express the IL-23 receptor (IL23R), the inducible co-stimulatory molecule ICOS, and the transcription factor c-Maf, all of them essential for expansion and establishment of the final pool of both subsets. Tfh cells differ from Th17 by their ability to home to B cell areas in secondary lymphoid tissue through interactions mediated by the chemokine receptor CXCR5 and its ligand CXCL13. These CXCR5+ CD4+ T cells are considered an effector T cell type specialized in B cell help, with a transcriptional profile distinct from Th1 and Th2 cells. The role of Tfh cells and its primary product, IL-21, on B-cell activation and differentiation is essential for humoral immunity against infectious agents. However, when deregulated, Tfh cells could represent an important mechanism contributing to exacerbated humoral response and autoantibody production in autoimmune diseases. This review highlights the importance of Tfh cells by focusing on their biology and differentiation processes in the context of normal immune response to infectious microorganisms and their role in the pathogenesis of autoimmune diseases.

Objective The objective of this study was to evaluate the association between Fc gamma receptor IIIb polymorphism and susceptibility to systemic lupus erythematosus and clinical traits of the disease.Methods Genomic DNA was obtained from 303 consecutive systemic lupus erythematosus patients and 300 healthy blood donors from the southeastern region of Brazil. The polymorphic region of the FCGR3B gene was sequenced and the alleles FCGR3B*01, FCGR3B*02 and FCGR3B*03 were analyzed.Results The FCGR3B*01 allele was more frequent in systemic lupus erythematosus patients (43.1%) while the FCGR3B*02 allele prevailed among controls (63.7%) (P=0.001). The FCGR3B*03 allele was found equally in both groups. The FCGR3B*01/*01 (20.7%) and FCGR3B*01/*02 (41.1%) genotypes were more frequent among systemic lupus erythematosus patients (P=0.028 and P=0.012, respectively) while the FCGR3B*02/*02 genotype was more frequent in controls (45.5%) (P<0.001). One variant of the FCGR3B*01 allele previously described in Germany was found in only one control. A new variant of the FCGR3B*01 allele with two substitutions (A227G/G277A) was found in one control. Three variants of the FCGR3B*02 allele previously described in African-Americans, Brazilians, Chinese and Japanese were found in ten 10 patients and two controls. In addition, several single nucleotide polymorphisms at non-polymorphic positions were identified in both patients and controls. Conclusion Susceptibility to systemic lupus erythematosus was associated with the FCGR3B*01 allele, as well as with the FCGR3B*01/*01 and FCGR3B*01/*02 genotypes. No association was found between FCGR3B genotypes and clinical manifestations, disease severity or the presence of autoantibodies.

β2 glycoprotein I (β2GPI) is a phospholipid binding protein that plays an important role in endothelial stability, blood coagulation, clearance of apoptotic debris and other physiologic processes. Anti-β2GPI antibodies occur in normal individuals and transiently during the course of infections, but are also associated with thrombotic events in autoimmune disease: the antiphospholipid syndrome (APS). A total of 31 out of 37 treated leprosy patients previously found to present high titers of IgM anti-β2GPI and/or anticardiolipin antibodies (aCL) remained positive for IgM antiphospholipid antibodies (aPL), and exhibited high titers of anti-β2GPI. The 37 patients were part of the 77 aPL-positive patients from a previous study that evaluated 158 leprosy patients. The median time elapsed between the first and second sample was 66 months. None of the 37 patients had any thrombotic event and 24 had a reactional state and were still requiring the use of prednisone, thalidomide or both. None of them fulfilled World Health Organization criteria for leprosy recurrence.

Background Cardiovascular disease (CVD) is an important cause of morbidity and mortality in systemic lupus erythematosus (SLE) and disturbances in endothelial function (EF) are implicated in its pathogenesis (1,2). EF also depends on endothelial progenitor cells (EPCs) that enhance angiogenesis, promote vascular repair and have potential as a marker of CVD (3,4,5). SLE patients have endothelial dysfunction and fewer EPCs (6,7). Objectives To evaluate the effect of supervised physical exercise (SPE) on quality of life, exercise tolerance, body composition, endothelial function, EPCs number and on vascular endothelial growth factor (VEGF) level in SLE patients. Methods Prospective, controlled, nonrandomized study. Women with SLE were allocated according to availability to participate in exercise group (EG) or control group (CG). Intervention: SPE was performed for 1 hour, 3X/week, for 16 weeks. Patients were evaluated at baseline (T0) and after 16 weeks (T16): exercise tolerance by cardiopulmonary exercise test; quality of life by SF-36; body composition by DEXA; high-resolution ultrasound of brachial artery in resting conditions, after reactive hyperaemia (flow-mediated dilation-FMD) and after oral glyceryl trinitrate (GTMD) was performed to assess endothelial function; EPCs were evaluated by flow cytometry using anti-CD34 (FITC), anti-CD133 (PE) and anti-KDR (APC); and VEGF was assessed by ELISA (R&D Systems, Minneapolis, USA). Results 535 SLE patients were invited, 239 manifested interest, but 127 were excluded due to exclusion criteria. 55 patients dropped out due to personal reasons. Twenty four patients completed the evaluations (mean age 33.2±8.2 years and mean disease duration of 99±77.9 months). Thirteen patients were assigned in the EG and eleven in the CG. Both groups were comparable and homogeneous regarding demographic variables and cardiovascular traditional risk factors. After 16 weeks, we observed a significant increase in FMD (7.7±7.2% vs 16.9±8.8%, p=0.005) in EG without changes in the GC (4.1±4.4% vs 7.4±5.7%, p=0.62). In the EG, we also found a significant improvement in exercise tolerance (12±2.1min vs 13.5±2min, p=0.021), maximum speed (7.6±1km/h vs 8.3±1km/h, p=0.049), threshold speed (5.5±0.6km/h vs 5.9±0.6km/h, p=0.012), functional capacity (66.2±23.8 vs 82.1±11.6, p=0.035) and vitality (72.9±31.4 vs 78.8±19.7, p=0.007). EPCs were analyzed in 10 patients of the EG and in seven of the CG. We observed a significant increase in number of CD34/CD133/KDR positive cells at T16 in the EG (0.38±0.37 vs. 1.57±1.38, p=0.005), with no difference in the CG (0.62±0.83 vs. 0.82±0.58, p=0.176). There was no difference on body composition and VEGF levels in both groups comparing T0 and T16. Conclusions Despite the small sample, this is the first study demonstrating that SPE can improve EF and EPCs number in SLE patients. The higher number of EPCs may be one of the mechanisms associated with EF improvement after an exercise program. Physical exercise can be a useful strategy to prevent CVD morbidity and mortality in SLE patients. References Kao et al. Curr Opin Rheumatol. 2003. Pearson JD. Lupus. 2000. Asahara et al. Science. 1997. Hill et al. N Engl J Med. 2003. Werner et al. N Engl J Med. 2005. Lima et al. J Rheumatol. 2002. Westerweel et al. Ann Rheum Dis. 2007. Disclosure of Interest None Declared

Background The literature shows that acute exercise increases IL-6 [1,2] and IL-10 [3,4] and TNF-a [5,6] serum levels in healthy subjects and can worse the inflammatory response in patients with diabetes mellitus type I, cystic fibrosis and obstructive pulmonary disease [7]. Some studies showed resting SLE patients have higher levels of IL-6 [8,9] and IL-10 [8,10] compared to healthy controls. Although being established in the literature that SLE patients have benefits with the aerobic training [11] mainly related to cardiovascular risk factors, there is no study evaluating the effect of acute exercise on the cytokines levels in SLE patients what could cause some concerning about the risk of worsening inflammatory cytokines Objectives To assess the levels of IL-10, IL-6 and TNF-a at baseline and after acute exercise in SLE patients Methods Thirty female SLE patients (≥4 ACR criteria) with inactive or mild disease activity, and 30 age-and gender-matched healthy controls were included. At baseline and soon after the ergospirometric test 10 ml of peripheral blood were collected for IL-10, IL-6 and TNF-a measurement by ELISA technique (R&D Systems, Inc.-Minneapolis, USA). The disease activity was evaluated by SLEDAI. The Student’s t-tests and Mann-Whitney test were used for intra and inter-group comparisons. P values <0.05 were considered significant. All participants signed Institutional Ethic Committee approved consent form. Results The mean age of patients and controls were comparable (32.58±7.44 y.o vs 30.43±7.76 y.o, p=0,275). The mean disease duration was 105.5±85.55 months and the mean SLEDAI score was 1.96±2.32. SLE patients presented worst ergospirometric parameters compared to controls: VO2máx (ml/kg/min) (25.27±5.20 vs 32.74±5.85, P<0.001); VO2max (l/min) (1.61±0.36 vs 1.98±0.36 P<0.001); maximum heart rate (174.75±2.34 vs 185.15±2.07 P=0.002); maximum ventilation (VEmax) (66.33±15.68 vs 80.48±18.98, P=0.001) and maximum speed (Km/h) (7.68±1.24 vs 9.4±1.22 P<0.001). At baseline SLE patients presented higher levels of IL-6 [2.85±2,68 vs 1.58±1.62 p=0.005] and IL-10 [1.00±2.1 vs -0.44±1.20 p=0.002] comparing to controls. The acute exercise did not significantly alter the levels of IL-6, IL-10 and TNF-a in SLE patients. On the other hand, in the control group, the acute exercise increased IL-6 level [1.8±1.62 vs 1.91±1.52; p=0.001] without significant change on IL-10 and TNF-α levels. Conclusions IL-6 and IL-10 levels were higher in SLE patients than in controls. Acute physical exercise increased IL-6 levels in healthy women, but, in SLE patients did not significantly changed the levels of IL-6, IL-10 or TNF-a. We concluded does not appear that acute exercise increase inflammatory cytokines and disease exacerbation in SLE patients with inactive or mild disease activity. References Nieman et al. Med. Sci. Sports Exerc., 2005. Pedersen. J Exp Biol. 2011. Yakeu et al. Atherosclerosis. 2010. Paalani et al. Ethn Dis 2011. Sandri et al. Eur J Cardiovasc Prev Rehabil. 2011. Hallberg et al. J Affect Disord. 2010. Ploeger et AL. Exerc Immunol Rev, 2009. Avrămescu et al. Rom J Morphol Embryol. 2010. Mellor-Pita et al. Cytometry B. Clin Cytom. 2009. Gigante et al. Eur Rev Med Pharmacol Sci. 2011. Carvalho et al Arthritis Care Res 2005. Disclosure of Interest None Declared

OBJETIVOS: Determinar a frequência do fator reumatoide (FR-IgM), anticorpos antipeptídeos citrulinados cíclicos (anti-CCP), antinucleares (AAN), anticitoplasma de neutrófilos (ANCA), anticardiolipina (aCL) e anti-β2 glicoproteína I (anti-β2GPI) em pacientes com hanseníase, com e sem comprometimento articular, avaliando a possível associação entre estes autoanticorpos e as manifestações articulares, a forma clínica, a reação hansênica, o tratamento com poliquimioterapia (PQT) e a alta. PACIENTES E MÉTODOS: 158 pacientes com hanseníase foram distribuídos em dois grupos; 73 pacientes com (Grupo I) e 82 sem comprometimento articular (Grupo II). Compuseram o Grupo III 129 indivíduos saudáveis. MÉTODOS: aglutinação com partículas de látex para FR-IgM, imunofluorescência indireta para AAN e ANCA, e, ELISA para anti-CCP, aCL e anti-β2GPI. RESULTADOS: Dentre 158 pacientes com hanseníase, 56 apresentavam a forma virchowiana (VV). A frequência de anticorpos anti-CCP, FR e AAN nos Grupos I e II foi semelhante à do Grupo III. ANCA não foi detectado em nenhum dos grupos. Anticorpos aCL foram mais frequentes nos pacientes com hanseníase (Grupos I e II) que em controles sadios (15,8% vs. 3,1%; P < 0,001), não sendo observada diferença entre Grupos I e II (P = 0,67). Anticorpos anti-β2GPI também foram mais frequentes nos pacientes que nos controles (46,2% vs. 9,4; P < 0,001), sem diferença significativa entre os Grupos I e II. Houve predomínio do isotipo IgM com relação ao IgG tanto para aCL (88% vs. 16%, P = 0,001), quanto para anti-β2GPI (97,3% vs. 12,3%, P < 0,001). Nenhum paciente apresentou manifestações sugestivas de trombose vascular. CONCLUSÃO: A frequência de anticorpos aCL e anti-β2GPI foi significativamente maior nos pacientes com hanseníase que nos controles saudáveis. Entretanto, a positividade dos demais autoanticorpos foi semelhante à dos controles. Não foi observada associação entre autoanticorpos e comprometimento articular, episódios reacionais, tratamento com PQT ou alta, nem com a forma clínica da hanseníase, exceto para anticorpos aCL que foram mais frequentes na forma clínica VV.OBJECTIVE: Determine the frequency of rheumatoid factor (IgM-RF) and anti-cyclic citrullinated peptide antibodies (anti-CCP), antinuclear antibodies (ANA), antineutrophil cytoplasmic antibodies (ANCA), anticardiolipin antibodies (aCL), and anti-β2 glycoprotein I antibodies (anti-β2GPI) in leprosy patients, with and without joint involvement, and to evaluate the possible association among those antibodies and articular manifestations, clinical type, reactional episodes, polychemotherapic treatment (PCT), and discharge from PCT. PATIENTS AND METHODS: One hundred and fifty-eight leprosy patients were divided in two groups of 73 patients (Group I) and 82 patients (Group II). Group III was composed of 129 healthy individuals. Methods: Semi-quantitative latex agglutination test for IgM-RF, indirect immunofluorescence for ANA and ANCA, and ELISA for anti-CCP, aCL, and anti-β2GPI. RESULTS: Fifty-six (35.4%) of 158 leprosy patients had lepromatous leprosy (LL). The frequency of anti-CCP, RF, and ANA antibodies in Groups I and II was similar to that of Group III. Antineutrophil cytoplasmic antibodies were not detected in any patient. Anticardiolipin antibodies were more frequent in leprosy patients (Groups I and II) than in control group (15.8% vs. 3.1%; P < 0.001), and differences between Groups I and II (P = 0.67) were not observed. Anti-β2GPI antibodies were also more common in leprosy patients than in control group (46.2% vs. 9.4%; P < 0.001), without differences between Groups I and II. A predominance of IgM isotype over IgG isotype was observed both for aCL (88% vs. 16%; P = 0.001) and anti-β2GPI (97.3% vs. 12.3%; P < 0.001). Patients did not present manifestations suggestive of vascular thrombosis. CONCLUSION: The frequency of aCL and anti-β2GPI antibodies was significantly increased in leprosy patients than in healthy individuals. However, positivity to other autoantibodies was similar to that observed in the control group. An association between autoantibodies and joint involvement, reactional episodes, polychemotherapic treatment, discharge, and clinical type of leprosy was not observed, except for aCL antibodies, which were more frequent in lepromatous leprosy.

Objective: Determine the frequency of rheumatoid factor (IgM-RF) and anti-cyclic citrullinated peptide antibodies (anti-CCP), antinuclear antibodies (ANA), antineutrophil cytoplasmic antibodies (ANCA), anticardiolipin antibodies (aCL), and anti-β2 glycoprotein I antibodies (anti-β2GPI) in leprosy patients, with and without joint involvement, and to evaluate the possible association among those antibodies and articular manifestations, clinical type, reactional episodes, polychemotherapic treatment (PCT), and discharge from PCT. Patients and methods: One hundred and fifty-eight leprosy patients were divided in two groups of 73 patients (Group I) and 82 patients (Group II). Group III was composed of 129 healthy individuals. Methods: Semi-quantitative latex agglutination test for IgM-RF, indirect immunofluorescence for ANA and ANCA, and ELISA for anti-CCP, aCL, and anti-β2GPI. Results: Fifty-six (35.4%) of 158 leprosy patients had lepromatous leprosy (LL). The frequency of anti-CCP, RF, and ANA antibodies in Groups I and II was similar to that of Group III. Antineutrophil cytoplasmic antibodies were not detected in any patient. Anticardiolipin antibodies were more frequent in leprosy patients (Groups I and II) than in control group (15.8% vs. 3.1%; P < 0.001), and differences between Groups I and II (P = 0.67) were not observed. Anti-β2GPI antibodies were also more common in leprosy patients than in control group (46.2% vs. 9.4%; P < 0.001), without differences between Groups I and II. A predominance of IgM isotype over IgG isotype was observed both for aCL (88% vs. 16%; P = 0.001) and anti-β2GPI (97.3% vs. 12.3%; P < 0.001). Patients did not present manifestations suggestive of vascular thrombosis. Conclusion: The frequency of aCL and anti-β2GPI antibodies was significantly increased in leprosy patients than in healthy individuals. However, positivity to other autoantibodies was similar to that observed in the control group. An association between autoantibodies and joint involvement, reactional episodes, polychemotherapic treatment, discharge, and clinical type of leprosy was not observed, except for aCL antibodies, which were more frequent in lepromatous leprosy.

The objective of the present research was to evaluate the usefulness of anti-cyclic citrullinated peptide (anti-CCP) antibodies and the IgM rheumatoid factor (IgM RF) test for the differential diagnosis of leprosy with articular involvement and rheumatoid arthritis (RA). Anti-CCP antibodies and IgM RF were measured in the sera of 158 leprosy patients (76 with and 82 without articular involvement), 69 RA patients and 89 healthy controls. Leprosy diagnosis was performed according to Ridley and Jopling classification criteria and clinical and demographic characteristics of leprosy patients were collected by a standard questionnaire. Leprosy patients with any concomitant rheumatic disease were excluded. Serum samples were obtained from all participants and frozen at -20 degrees C. Measurement of anti-CCP antibodies and IgM RF were performed by ELISA, using a commercial second-generation kit, and the latex agglutination test, respectively. Anti-CCP antibodies and IgM RF were detected in low frequencies (2.6 and 1.3%, respectively) in leprosy patients and were not associated with articular involvement. Among healthy individuals both anti-CCP antibodies and IgM RF were each detected in 3.4% of the subjects. In contrast, in the RA group, anti-CCP antibodies were present in 81.2% and IgM RF in 62.3%. In the present study, both anti-CCP antibodies and IgM RF showed good positive predictive value for RA, helping to discriminate between RA and leprosy patients with articular involvement. However, anti-CCP antibodies were more specific for RA diagnosis in the population under study.

The aim of this study was to evaluate traditional risk factors for coronary artery disease (CAD), homocysteine, anti-oxidized low-density lipoprotein (anti-oxLDL), anti-lipoprotein lipase (anti-LPL) and endothelin-1 (ET-1) in patients with primary anti-phospholipid syndrome (APS), furthermore verify possible association among these variables and arterial thrombosis. Thirty-eight women with primary APS and 30 age-and-sex-matched controls were evaluated. Patients presented higher-LDL and triglycerides levels and lower-HDL levels than controls. Anti-LPL antibodies were not detected in both groups. The mean number of risk factors was higher in patients than in controls ( P = 0.030). Anti-oxLDL antibodies, homocysteine and ET-1 mean levels were similar between groups, but abnormal homocysteine levels were found only among primary APS patients ( P = 0.031). Hypertension and the presence of at least one risk factor for CAD were more prevalent in patients with arterial involvement than those without. Homocysteine levels and mean number of risk factors for CAD were significantly higher in patients with arterial thrombosis than controls. In a multivariate analysis hypertension was the only independently associated with arterial thrombosis (OR 14.8, 95% CI = 2.1—100.0, P = 0.006). This study showed that in primary APS patients other risk factors besides anti-phospholipid antibodies contribute for the occurrence of arterial events and the most important factor was hypertension. Lupus (2007) 16, 782—787.

The aim of this study was to characterize a novel human autoantibody-autoantigen system represented as cytoplasmic discrete speckles (CDS) in indirect immunofluorescence (IIF). A distinct CDS IIF pattern represented by 3-20 discrete speckles dispersed throughout the cytoplasm was identified among other cytoplasmic speckled IIF patterns. The cytoplasmic domains labelled by human anti-CDS-1 antibodies did not co-localize with endosome/lysosome markers EEA1 and LAMP-2, but showed partial co-localization with glycine-tryptophan bodies (GWB). CDS-1 sera did not react with several cellular extracts in immunoblotting and did not immunoprecipitate recombinant GW182 or EEA1 proteins. The typical CDS-1 IIF labelling pattern was abolished after delipidation of HEp-2 cells. Moreover, CDS-1 sera reacted strongly with a lipid component co-migrating with phosphatidylethanolamine (PE) in high performance thin-layer chromatography (HPTLC)-immunostaining of HEp-2 cell total lipid extracts. The CDS-1 major molecular targets were established by electrospray ionization-mass spectrometry (ESI-MS), HPTLC-immunostaining and chemiluminescent enzyme-linked immunosorbent assay as diacyl-PE species, containing preferentially a cis-C18 : 1 fatty acid chain at C-2 of the glycerol moiety, namely 1,2-cis-C18 : 1-PE and 1-C16 : 0-2-cis-C18 : 1-PE. The clinical association of CDS-1 sera included a variety of systemic and organ-specific autoimmune diseases but they were also observed in patients with no evidence of autoimmune disease.

Cajal bodies (CB) are ubiquitous nuclear structures involved in the biogenesis of small nuclear ribonucleoproteins and show narrow association with the nucleolus. To identify possible relationships between CB and the nucleolus, the localization of coilin, a marker of CB, and of a set of nucleolar proteins was investigated in cultured PtK2 cells undergoing micronucleation. Nocodazol-induced micronucleated cells were examined by double indirect immunofluorescence with antibodies against coilin, fibrillarin, NOR-90/hUBF, RNA polymerase I, PM/Scl, and To/Th. Cells were imaged on a BioRad 1024-UV confocal system attached to a Zeiss Axiovert 100 microscope. Since PtK2 cells possess only one nucleolus organizer region, micronucleated cells presented only one or two micronuclei containing nucleolus. By confocal microscopy we showed that in most micronuclei lacking a typical nucleolus a variable number of round structures were stained by antibodies against fibrillarin, NOR-90/hUBF protein, and coilin. These bodies were regarded as CB-like structures and were not stained by anti-PM/Scl and anti-To/Th antibodies. Anti-RNA polymerase I antibodies also reacted with CB-like structures in some micronuclei lacking nucleolus. The demonstration that a set of proteins involved in RNA/RNP biogenesis, namely coilin, fibrillarin, NOR-90/hUBF, and RNA polymerase I gather in CB-like structures present in nucleoli-devoid micronuclei may contribute to shed some light into the understanding of CB function.

Objective. To determine frequency, origin, and clinical associations of, elevated serum neuron specific enolase (NSE) in systemic sclerosis (SSc).Methods. Serum was obtained from 75 patients with SSc, 20 systemic lupus erythematosus, 8 polymyositis, 10 idiopathic interstitial lung disease, and 10 healthy volunteers. NSE status was determined in serum (in all individuals) and in platelet lysate (in volunteers and 30 patients with SSc).Results. Elevated serum NSE (mean 22.6 ng/ml, range 12.1-68.2 ng/ml) was observed in 26 patients with SSc (34.6%). Those with diffuse SSc had higher serum NSE than those with limited disease (16.5 +/- 13.4 vs 9.6 +/- 5.0 ng/ml, p = 0.006). No association was found between serum NSE and lung or esophagus involvement. Patients with long-standing disease had lower serum NSE than those with early disease (10.8 +/- 7.3 vs 16.1 +/- 13.6 ng/ml, p = 0.05). Serum NSE was 19.4 +/- 13.0 ng/ml in patients with total skin score (TSS) > 20, 8.3 +/- 2.1 ng/ml in patients with TSS < 5, and 6.0 +/- 3.1 ng/ml in volunteers (p = 0.01). NSE platelet lysate concentration was 3.6 +/- 2.9 ng/ml in patients with TSS > 20, 12.4 +/- 4.1 ng/ml in those with TSS < 5, and 14.1 +/- 6.5 ng/ml in healthy individuals (p < 0.001). Volunteers and SSc patients with low TSS had comparable S/PL-NSE index (serum/platelet lysate NSE concentration) (0.42 +/- 0.16 and 0.75 +/- 0.33, respectively), both lower than SSc patients with high TSS (7.45 +/- 5.57) (p < 0.001).Conclusion. Elevated serum NSE was observed in one-third of SSc patients but not in other autoimmune rheumatic diseases. The inverse relationship between serum and platelet lysate NSE concentration suggests platelet activation as the origin of high serum NSE in SSc. NSE S/PL was the best discriminatory variable between healthy volunteers and SSc patients as well as between patients with high and low TSS. High serum NSE and high NSE-S/PL index seemed to be associated with SSc disease activity. Further work is warranted to investigate a possible role for this marker in assessing disease activity and therapy response.

We analyzed the frequency and clinical correlates of antiperinuclear factor (APF) and antibodies to the stratum corneum of rat esophagus in 86 children with juvenile idiopathic arthritis (JIA), 32 children with juvenile systemic lupus erythematosus, and 52 healthy children. Forty-two patients with JIA (49%) were positive for APF. No association was observed between APF and current age, sex, JIA subtype, age at disease onset, or disease duration. APF was found in one patient with juvenile systemic lupus erythematosus and in no healthy child. Antibodies to the stratum corneum of rat esophagus were detected in 3 patients with polyarticular JIA. APF, may be a valuable tool in the differential diagnosis of JIA.

The objective of the present study was to investigate the prevalence, clinical characteristics, and HLA associations of C2 deficiency in the Brazilian population. The frequency of C2 deficiency profile (C2Q° profile) was 2.2% among 1503 blood donors and 6.6% among 166 patients with systemic lupus erythematosus (SLE). A higher incidence of clinical manifestations possibly related to immune complex disease was observed among blood donors with C2Q° profile and their relatives with C2Q° profile when compared to the normal C2 relatives. The comparison of clinical and laboratory features between SLE patients with C2Q° profile and those with normal C2 revealed earlier disease onset, higher frequency of oral ulcerations and lower frequency of anti-native DNA antibodies in the first group. The HLA study conducted on 18 individuals with C2Q° profile (11 blood donors and 7 SLE patients) confirmed the previously reported association with the antigens HLA-A25, B18 and DR2, supporting the concept that probably most C2 deficiency cases, throughout the world, are due to a single mutation in the C2 gene in linkage disequilibrium with the A25B 18DR2 haplotype.