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We report on 11 cases of specific polysaccharide antibody deficiency (SPAD) revealed in adulthood by severe infections with encapsulated bacteria. Given that immunoglobulin replacement therapy can effectively prevent the recurrence of bacterial infections in this context, SPAD should be considered once other antibody deficiencies have been ruled out. [ABSTRACT FROM AUTHOR]

• Anti-Ro52 antibodies were strongly associated with the prevalence of interstitial lung disease at the diagnosis of connective tissue disease, even after adjusting for the coexistence of anti-Ro60 antibodies. • Anti-Ro52 antibodies positivity should lead clinicians to careful screening of interstitial lung disease at the diagnosis of connective tissue disease. • Anti-Ro52 antibodies could represent a useful severity marker of connective tissue diseases. Clinical significance of anti-Ro52 antibodies in connective tissue diseases (CTD) is controversial. Anti-Ro52 antibodies might be associated with a more severe CTD phenotype, especially interstitial lung disease (ILD). The aims of this study were to evaluate ILD prevalence and severity, the prevalence of micro- or macroangiopathy and CTD-associated cancers in CTD with anti-Ro52 antibodies. CTD patients with anti-Ro52 antibody screening by immunoblot at diagnosis were enrolled. Two groups were retrospectively formed according to the presence of anti-Ro52 antibodies with an unbiased 1:1 matching on CTD types. Unsupervised multiple correspondence analysis and hierarchical clustering analysis were used to aggregate anti-Ro52 positive patients in subgroups. 408 CTD patients were included. Anti-Ro52 antibodies were detected in 33 % of CTD patients. Anti-Ro52 antibodies were associated with ILD at CTD diagnosis (47.8% vs. 23.0%, OR 3.3 95% IC 1.4 to 8.0, p = 0.008), even after adjusting for the presence of anti-Ro60 antibodies, especially in patients with antisynthetase syndrome, primary Sjögren syndrome and systemic sclerosis. Micro- or macroangiopathy was more frequent in anti-Ro52 positive CTD patients (18.6% vs. 9.7%, p = 0.02) and CTD patients with anti-Ro52 antibodies experienced more frequent relapses and required more immunosuppressive drugs. Clusters 4 and 5 identified anti-Ro52 positive CTD patients with severe ILD and with clinical features of systemic sclerosis or antisynthetase syndrome respectively. We found that anti-Ro52 antibodies were independently associated with ILD in CTD patients irrespective of CTD type. Anti-Ro52 antibodies could be associated with severity and a more relapsing disease course in CTD patients. [ABSTRACT FROM AUTHOR]

Context.--Distinguishing the different types of amyloid is clinically important because treatments and outcomes are different. Mass spectrometry is the new gold standard for amyloid typing, but it is costly and not widely available. Therefore, immunolabeling remains the first step in identifying the most common types of amyloidosis. In amyloid subtyping, direct immunofluorescence works well when applied to frozen sections, but immunohistochemistry on formalin-fixed, paraffin-embedded material often yields poor results, particularly for light chain amyloidosis. Recently, paraffin immunofluorescence has been described as a valuable salvage technique in renal pathology when frozen sections are not available but it has not been evaluated for extra-renal diseases. Objectives.--To evaluate the use of paraffin immunofluorescence for light-chain detection in extra-renal amyloidosis and other light-chain--associated diseases. Design.--First, we compared the staining intensity of both light chains between paraffin immunofluorescence and immunohistochemistry on a retrospective cohort of 28 cases of amyloidosis that have been previously typed. Then, we studied the role of paraffin immunofluorescence as an addition to our classical immunohistochemistry panel for amyloidosis typing. Results.--In the retrospective cohort, we found that paraffin immunofluorescence outperformed immunohistochemistry for light-chain detection. Then, in the prospective part of the study, we showed that the proportion of correctly classified cases increased from 50% to 71.9% with the adjunction of second-intention paraffin immunofluorescence to the immunohistochemistry procedure. Conclusions.--We therefore view paraffin immunofluorescence as a significant addition to the routine workflow for detection of light-chain--related diseases. [ABSTRACT FROM AUTHOR]

The diseases caused byseveral Salmonella serovars in poultry results in important aconomical lossesfor this industrie in developing countries. The consumption of Salmonella contaminated poultry products by humanscan produce diarrhea; therefore Salmonella contaminated poultry products has become an important public health problem revealing the necesity to develop a vaccine against the different Salmonella serovars for poultry . In order to develope this vaccine will be necesary to obtein immunogens capable of inducing a protective respònse aginst these bacteria. In the present work we describe the purification and immunochemical characteristics of porins from Salmonella enterica serovar gallinarum, Salmonella enterica serovar pullorum and Salmonella enterica serovar enteritidis. The porins are proteins highly conserved of gram negative bacteria. They are found like homotrimers in the outer membrane, acting like difussion channels. Several experimental models had showned that these proteins are important antigens in the induction of host protective immune responses. The outer membrane proteins (OMP's) were isolated by solubilization of the cellular envelopes in a buffer of high ionic strength and detergent (0.3M NaCl, 2%SDS). This enriched porin preparation was further purified by gel filtration using a Sephacryl S-200 column; we obtained an Absorbance peak (280) that was analyzed for porin pressence by discontinuous poliacrylamide-SDS gels and by western blot ussing specific porin antibodies. We obtained two bands with 35 and 37 Kda that reacted with the specific antibodies. The S.enterica serovars porins showed homology between them and with the previously characterized porins fro S.typhi and S.typhimurium. By bidimensional electrophoresis we found out that each serovar porin has a particular bidimentional pattern, these porins were acidic proteins with isoelectric points between 4.7-5.5; this distinctive bidimentional pattern of each porin will allow us to issolate and sequence them in the near future, So far we have been able to purified and characterized porins from important poultry Salmonella enterica serovars than are being used as antigens to develope an specific vaccine. [ABSTRACT FROM AUTHOR]

Objective: In systemic sclerosis (SSc) and idiopathic inflammatory myopathies (IIM), auto-antibodies are used in daily practice as potent biomarkers of clinical phenotypes. This study aimed at estimating the prevalence of myositis-specific (MSA) and myositis-associated (MAA) auto-antibodies in a well-characterised SSc patients cohort using two different immunoblot assays, and studying their clinical associations.Methods: In this cross-sectional study, the sera of 300 consecutive patients were tested at the same time with myositis antibodies Euroimmun® and D-tek® immunoblot assays.Results: Prevalence of MSA/MAA, MSA and MAA were 17.0%, 8.0% and 9.7%, respectively. When combining results of both tests, anti-PM/Scl 100 were found in 5.0% (95% confidence interval 2.8; 8.1); anti-PM/Scl 75 and anti-TIF1γ in 3.7% (1.8; 6.5); anti-Ku 3.0% (1.4; 5.6); anti-MDA5 in 1.3% (0.4; 3.4); anti-Mi-2 β, anti-NXP2, anti-PL-7 and anti-SRP in 0.7% (0.08; 2.4); anti-EJ and anti-PL-12 in 0.3% (0.01; 1.8) of patients. No reactivity against SAE1, Jo-1 or OJ was observed. Anti-PM/Scl 75 antibodies were associated with interstitial lung disease (80% vs. 42%) and myositis (27% vs. 3%); anti-Ku antibodies were associated with myositis (33% vs. 3%).Conclusion: In this cross-sectional study of 300 SSc patients, the prevalence of MSA/MAA, MSA and MAA using immunoblot assays were 17.0%, 8.0% and 9.7%, respectively. MAA positivity was associated with ILD and myositis, but this study did not highlight any clinical associations with MSA positivity. [ABSTRACT FROM AUTHOR]