Atypical presentation of acute rheumatic fever (ARF) in a 25-year-old woman in the Caribbean: a challenging diagnosis.

A 25-year-old woman presented a challenging diagnosis of acute rheumatic fever (ARF). Initial symptoms included dry cough and three minor Jones criteria (unabating fever (38.4°C, 0d), elevated acute phase reactants (C-reactive protein, 13d) and joint pain (monoarthralgia) in her neck (0d)). ARF was diagnosed only after presentation of two major Jones criteria (polyarthritis/polyarthralgia (16d) and erythema marginatum (41d)) and positive antistreptolysin O titre (44d). Parotid swelling, peripheral oedema, elevated liver enzymes and diffuse lymphadenopathy complicated the diagnosis. Throat swab, chorea and carditis were negative or absent. Atypical ARF is challenging to recognise. There is no diagnostic test and its presentation is similar to that of other diseases. While the 2015 Jones criteria modification increased specificity of ARF diagnosis, atypical cases may still be missed, especially by physicians in developed countries. Suspicion of atypical ARF, especially after travel to high incidence regions, would allow for earlier treatment and prevention of rheumatic heart disease.

Diagnostic Value of Different Noninvasive Criteria of Latent Myocarditis in Comparison with Myocardial Biopsy.

PURPOSE: The aim of this study was to quantify the value of various clinical, laboratory, and instrumental signs in the diagnosis of myocarditis in comparison with morphological studies of the myocardium. METHODS: In 100 patients (65 men, 44.7 ± 12.5 years old) with "idiopathic" arrhythmias (n = 20) and dilated cardiomyopathy (DCM; n = 80), we performed the following: 71 endomyocardial biopsies (EMB), 13 intraoperative biopsies, 5 studies of explanted hearts, and 11 autopsies with virus investigation (real-time PCR) of the blood and myocardium. Antiheart antibodies (AHA) were also measured as well as cardiac CT (n = 45), MRI (n = 25), and coronary angiography (n = 47). The comparison group included 50 patients (25 men, 53.7 ± 11.7 years old) with noninflammatory heart diseases who underwent open heart surgery. RESULTS: Active/borderline myocarditis was diagnosed in 76.0% of the study group and in 21.6% of patients in the comparison group (p < 0.001). The myocardial viral genome was observed more frequently in patients in the comparison group than in the study group (65.0 and 40.2%; p < 0.01). We evaluated the diagnostic value of noninvasive markers of myocarditis. The panel of AHA had the greatest importance in the identification of myocarditis: sensitivity was 81.5%, and the positive and negative predictive values were 75.0 and 60.5%. This defined the diagnostic value of noninvasive markers of myocarditis and established a diagnostic algorithm providing an individual assessment of the likelihood of myocarditis development. CONCLUSION: AHA have the greatest significance in the diagnosis of latent myocarditis in patients with "idiopathic" arrhythmias and DCM. The use of a complex of noninvasive criteria allows the probability of myocarditis to be estimated and the indications for EMB to be determined.

NF-κB-driven miR-34a impairs Treg/Th17 balance via targeting Foxp3.

The subset of regulatory T (Treg) cells, with its specific transcription Foxp3, is a unique cell type for the maintenance of immune homeostasis by controlling effector T (Teff) cell responses. Although it is common that a defect in Treg cells with Treg/Teff disorder causes autoimmune diseases; however, the precise mechanisms are not thoroughly revealed. Here, we report that miR-34a could attenuate human and murine Foxp3 gene expression via targeting their 3' untranslated regions (3' UTR). The human miR-34a, increased in peripheral blood mononuclear cells (PBMCs) and CD4+ T cells from rheumatoid arthritis (RA) or systemic lupus erythematosus (SLE) patients, displayed a positive correlation with some serum markers of inflammation including rheumatoid factor (RF), anti-streptolysin antibody (ASO), erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) as well as Th17 signature gene RORγt, but inversely correlated with the mRNA expression levels of FOXP3. In addition, murine miR-34a levels were downregulated in TGF-ß-induced Treg cells but upregulated in Th17 cells induced in vitro compared to activated CD4+ T cells. It has also been demonstrated that elevated miR-34a disrupting Treg/Th17 balance in vivo contributed to the progress of pathogenesis of collagen induced arthritis (CIA) mice. Furthermore, IL-6 and TNF-α were responsible for the upregulation of miR-34a and downregulation of Foxp3, which was reverted by the addition of NF-κB/p65 inhibitor BAY11-7082, thus indicating that NF-κB/p65 inhibited Foxp3 expression in an miR-34a-dependent manner. Finally, IL-6 or TNF-α-activated p65 could bind to the miR-34a promotor and enhance its activity, resulting in upregulation of its transcription. Taken together, we show that NF-κB activated by inflammatory cytokines, such as IL-6 and TNF-α, ameliorates Foxp3 levels via regulating miR-34a expression, which provides a new mechanistic and therapeutic insight into the ongoing of autoimmune diseases.

Crescentic infection related glomerulonephritis in adult and its outcome.

The epidemiology of infection-related glomerulonephritis (IRGN) is changing in recent times both in developed and developing nations. Although published studies showed renal outcome in adult IRGN was not as benign as in children, literature regarding clinical profile and outcome of crescentic form of adult IRGN is scarce; hence, we aimed to study the clinical profile of crescentic IRGN. We conducted a retrospective observational study in patients with crescentic IRGN in adults at the Department of Nephrology, Madras medical college, Chennai between 2009 and 2014. A total of 47 patients were included with a mean follow-up of 9.9 ± 4.2 months. The mean age was 42 ± 13.5 years. About 19.1% of patients had diabetes. The skin was the most common site of infection (38.3%) with methicillin-resistant Staphylococcus acareas (MRSA) as the most common organism. Hypocomplementemia was present in 100% in our study. Hemodialysis (HD) was required in 53.2% of patients and oral steroids were given in 78.7%. Complete renal recovery was seen only in 25.5%, progression to chronic kidney disease in 40.4%, seven patients reached end-stage renal disease, and nine patients died during follow-up. On univariate analysis, MRSA infection, the unidentified source of infection, nonisolation of organisms presence of interstitial fibrosis and tubular atrophy in renal biopsy and requirement of HD were found to be significant risk factors for poor renal outcome. In our study, crescentic form of IRGN is associated with poor renal outcome.

Henoch-Schonlein purpura, post-streptococcal glomerulonephritis and acute rheumatic carditis after Group A ß-haemolytic streptococcal infection.

Besides association with acute rheumatic fever (ARF) and acute glomerulonephritis (APSGN), in up to 40% of cases, Group A ß-haemolytic streptococcal (GABHS) infections are also implicated as a trigger for Henoch-Schonlein purpura (HSP). A 7-year-old girl with GABHS throat infection who developed HSP, APSGN and rheumatic carditis is reported. She presented with palpable purpura and arthritis in both ankles and later developed carditis characterised by mitral/aortic regurgitation and glomerulonephritis characterised by mixed nephritic/nephrotic syndrome. She had a raised anti-streptolysin titre (ASOT), blood urea nitrogen and creatinine and hypocomplementaemia (C3), and renal biopsy demonstrated endocapillary and extracapillary proliferative glomerulonephritis with crescents. Immunofluorescence microscopy demonstrated a 'full house' of immunoglobulin and complement, viz. IgA + 2, IgG + 3, IgM + 2, C3c + 1, Clq + 2 with predominantly IgG deposition. One week earlier, her 4-year-old sister had presented to another hospital with HSP complicated by microscopic haematuria, nephrotic-range proteinuria and gastro-intestinal involvement, and with raised ASOT and low C3 levels. Although HSP has been associated with either ARF or APSGN, this is the first case of a child with HSP, ARF and APSGN in combination.

[Clinical, laboratory and instrumental criteria for myocarditis, established in comparison with myocardial biopsy: A non-invasive diagnostic algorithm]. / Klinicheskie, laboratornye i instrumental'nye kriterii miokardita, ustanovlennye v sopostavlenii s biopsiinym issledovaniem miokarda (algoritm neinvazivnoi diagnostiki).

AIM: To determine the diagnostic value of different clinical, laboratory, and instrumental signs in the diagnosis of myocarditis in patients with the picture of idiopathic arrhythmias, dilated cardiomyopathy (DCM) and in a comparison group when comparing with myocardial morphological examination. SUBJECTS AND METHODS: A study group included 100 patients (35 women; mean age, 44.7±12.5 years) with idiopathic arrhythmias (n=20) and DCM as a syndrome (n=100). All underwent myocardial morphological examination: endomyocardial biopsy (EMB) (n=71), intraoperative biopsy (n=13), study of the explanted heart (n=6), and autopsy (n=11). A comparison group consisted of 50 patients (25 women; mean age, 53.7±11.7 years) with non-inflammatory diseases of the heart (left ventricular end-diastolic dimension <6.0 cm, ejection fraction >50%) who underwent open-heart surgery (n=47), EMB (n=2), or autopsy (n=1). The investigators also performed polymerase chain reaction for cardiotropic viral DNA in the blood and myocardium, anticardiac antibody (ACA) identification, myocardial scintigraphy (n=26), coronary angiography (n=47), magnetic resonance imaging (MRI) (n=25), and multislice computed tomography of the heart (n=45). The diagnostic value of the extended spectrum of clinical, laboratory, and instrumental markers for myocarditis was estimated. RESULTS: Active/borderline myocarditis was diagnosed in 76% of the patients in the study group (75.5% in the arrhythmia subgroup and 76.3% in the DCM one) and in 24.3% of those in the comparison group (p<0.001). A viral genome in the myocardium was detected statistically significantly less frequently in the study group than that in the comparison one (40.2 and 65%, respectively; p<0.01): in 46.6% in the DCM subgroup and 15.8% in the arrhythmia one. An ACA set (sensitivity, specificity, and predictive value of positive and negative test results (45.7, 80, 80.4, and 45%, respectively)) was of the greatest diagnostic importance in identifying myocarditis; antibodies to cardiomyocyte nuclei in a titer of 1:160-1:320 had the highest specificity (93.3%). A specificity above 70% was seen for a full medical history triad (acute onset, an association between onset and infection, a symptom duration of less than one year), systemic immune manifestations, anginas in the history and elevated anti-O-streptolysin levels, systemic blood changes, Q waves/QS complexes on ECGs, local hypokinesias, pericardial effusion, atriomegalia (in arrhythmias), angina/ischemia with intact coronary arteries, and focal perfusion defects during myocardial scintigraphy. A sensitivity higher than 50% was observed for age over 40 years (differential diagnosis with genetic forms), acute onset, a correlation with infection, and delayed contrast agent accumulation, as evidenced by MSCT/MRI. CONCLUSION: When the incidence of myocarditis is similar in the arrhythmia and DCM subgroups, the viral genome detection rate is statistically significantly higher in DCM. Among the non-invasive markers, an ACA set (high sensitivity and specificity) is of the greatest diagnostic value in the diagnosis of myocarditis. The diagnostic rule based on counting the number of scores has been developed, which makes it possible to individually establish the risk of myocarditis in patients with idiopathic arrhythmias and DCM for both the determination of indications for biopsy and the lack of the possibility of its performance. The risk of myocarditis is high if there are 5-7 scores; that is close to 100% if there are 8 scores or more.

Clinical value of antistreptolysin O levels in adult patients with tonsillitis: report I.

To assess the clinical value of antistreptolysin O (ASO) level in adult patients with acute tonsillitis of group A beta-hemolytic streptococcus (GABHS) etiology and its interaction with the Centor score and throat cultures data. ASO antibody titers and throat cultures were obtained from 260 adult patients with acute tonsillitis of GABHS etiology initially proven by the Centor score. The results were compared with the group of 100 adult patients with recurrent tonsillitis who underwent tonsillectomy and with the group of 100 healthy adults. Throat cultures revealed GABHS-positive results in 69 acute cases (26.5%) and in 24 recurrent cases (24%), i.e., with no significant differences between the groups (p = 0.845). There was no significant difference between cases with GABHS-positive and with GABHS-negative throat culture in ASO titers results (mean 250 and 280, respectively, p = 0.44) but these titers were significantly higher than established normative data (p < 0.01). For the group of recurrent tonsillitis cases, the mean ASO titer was 363 being significantly higher in comparison with acute cases (p = 0.015). The ASO antibody titers are significantly higher than normative ranges in cases of acute tonsillitis in adults. The detection of the elevated titers may lead to early antibiotherapy to tonsillitis. The Centor score is supported by the ASO data and less supported by throat cultures data. Further research should reveal if these titers might have predictive value for possible further recurrence or serve as an indicator for tonsillectomy in cases of recurrent tonsillitis.

A cytokine study of pediatric Tourette's disorder without obsessive compulsive disorder.

It has been suggested that post-infectious inflammation in central nervous system is a cause of tic disorder including Tourette's disorder (TD). Since pro-inflammatory cytokines are important mediators inducing inflammation, the cytokine levels are regarded as one of the important indicators of inflammation. Several studies have investigated the relationship of autoimmunity and the pathogenesis of TD by measuring the inflammatory cytokine levels of blood. However, when using human samples, the experimental results can be affected by the factors like size of sample, comorbidity, medication that patients take and the severity of the diseases. Thus, it is important to exclude the possibility that comorbidity and medication affects the level of inflammatory cytokines in the serum of TD patients. In our experiment, we recruited 29 patients without obsessive compulsive disorder (OCD) comorbidity and the majority of these patients did not take medication. The six pro-inflammatory cytokine levels of blood between patient and healthy groups were compared, considering the factors above, to determine more accurate results. Of the cytokines we investigated, the interleukin 12 p70 (IL-12p70) and tumor necrosis factor α (TNFα) levels increased in patient group compared to healthy controls and the patient group which have anti-streptolysin O (ASO) score under the 200 or YTGSS score from 10 to 19 also showed higher IL-12p70 or TNFα levels. In addition, the patients who did not take medication showed higher TNFα levels compared to healthy controls. In conclusion, we suggest that inflammatory pathways that involve IL-12p70 or TNFα are important to the pathogenesis of TD.

Parameters indicative of persistence of valvular pathology at initial diagnosis in acute rheumatic carditis: the role of albumin and CD19 expression / Parâmetros indicativos de persistência de patologia valvular no diagnóstico inicial de cardite reumática aguda: o papel da albumina e da expressão de CD19

Abstract Objective: The aim of this study is to define the predictors of chronic carditis in patients with acute rheumatic carditis (ARC). Methods: Patients diagnosed with ARC between May 2010 and May 2011 were included in the study. Echocardiography, electrocardiography, lymphocyte subset analysis, acute phase reactants, plasma albumin levels, and antistreptolysin-O (ASO) tests were performed at initial presentation. The echocardiographic assessments were repeated at the sixth month of follow-up. The patients were divided into two groups according to persistence of valvular pathology at 6th month as Group 1 and Group 2, and all clinical and laboratory parameters at admission were compared between two groups of valvular involvement. Results: During the one-year study period, 22 patients had valvular disease. Seventeen (77.2%) patients showed regression in valvular pathology. An initial mild regurgitation disappeared in eight patients (36.3%). Among seven (31.8%) patients with moderate regurgitation initially, the regurgitation disappeared in three, and four patients improved to mild regurgitation. Two patients with a severe regurgitation initially improved to moderate regurgitation (9.1%). In five (22.8%) patients, the grade of regurgitation [moderate regurgitation in one (4.6%), and severe regurgitation in 4 (18.2%)] remained unchanged. The albumin level was significantly lower at diagnosis in Group 2 (2.6 ± 0.48 g/dL). Lymphocyte subset analysis showed a significant decrease in the CD8 percentage and a significant increase in CD19 percentage at diagnosis in Group 2 compared to Group 1. Conclusion: The blood albumin level and the percentage of CD8 and CD19 (+) lymphocytes at diagnosis may help to predict chronic valvular disease risk in patients with acute rheumatic carditis.

Resumo Objetivo: Definir os preditores da cardite crônica em pacientes com cardite reumática aguda (CRA). Métodos: Os pacientes diagnosticados com CRA entre maio de 2010 e maio de 2011 foram incluídos no estudo. Foram feitos os testes de ecocardiografia, eletrocardiograma, uma análise do subgrupo de linfócitos, provas de fase aguda, níveis de albumina plasmática, antiestreptolisina-O (ASO) na manifestação inicial. As avaliações ecocardiográficas foram repetidas no 6º mês de acompanhamento. Os pacientes foram divididos em dois grupos de acordo com a persistência da patologia valvular no 6º mês como Grupo 1 e Grupo 2 e todos os parâmetros clínicos e laboratoriais na internação foram comparados entre dois grupos de comprometimento valvular. Resultados: Durante o período do estudo de um ano, 22 pacientes apresentaram doença valvular; 17 (77,2%) apresentaram regressão da patologia valvular. Houve desaparecimento de regurgitação moderada inicial em oito pacientes (36,3%). Entre sete (31,8%) pacientes com regurgitação moderada inicialmente, a regurgitação desapareceu em três e quatro apresentaram melhoria para regurgitação leve. Dois pacientes com regurgitação grave inicialmente apresentaram melhoria para regurgitação moderada (9,1%). Em cinco (22,8%) pacientes o grau de regurgitação (regurgitação moderada em um [4,6%] e regurgitação grave em quatro [18,2]) continuou inalterado. O nível de albumina foi significativamente menor no diagnóstico no Grupo 2 (2,6 ± 0,48 gr/dL). A análise do subgrupo de linfócitos mostrou uma redução significativa no percentual de CD8 e um aumento significativo no percentual de CD19 no Grupo 2 em comparação com o Grupo 1. Conclusão: O nível de albumina no sangue e o percentual de linfócitos CD8 e CD19 (+) no diagnóstico podem ajudar a prever risco de doença valvular crônica em pacientes com cardite reumática aguda.

Prevalence of rheumatic heart disease in patients with recurrent tonsillitis and elevated anti-streptolysin O titers.

INTRODUCTION: Patients with elevated anti-streptolysin O (ASO) titers (ASOT) and recurrent tonsillitis episodes are known to be at higher risk for rheumatic heart disease (RHD). However, there is no data regarding prevalence of RHD in this high risk population. In this study, we aimed to screen ambulatory patients with elevated ASOT and recurrent tonsillitis episodes using echocardiography for identification of RHD. We hypothesized that prevalence of RHD is higher in this patient group compared to general population. METHODS: 102 patients (10.33 ± 4.01 years, 50.98% female) who were diagnosed with recurrent tonsillitis and had elevated ASOT were included this study. Echocardiographic evaluation was performed by an experienced cardiologist. RESULTS: Echocardiographic examination revealed definite RHD in 2/102 (1.96%) patients and borderline RHD in 3/102 (2.94%) patients. CONCLUSION: Our study demonstrates a high prevalence of RHD in patients with recurrent tonsillitis episodes and high ASOT. Screening with echocardiography is beneficial to improve the detection rates of subclinical RHD in such high-risk populations.

Anterior and Intermediate Uveitis with Retinal Vasculitis: An Unusual Manifestation of Post-Streptococcal Uveitis Syndrome.

PURPOSE: To report a case of bilateral panuveitis with vasculitis, an unusual manifestation of post-streptococcal uveitis syndrome (PSU). METHODS: An 8-year-old patient consulted for bilateral red eye following acute tonsillitis. Exploration revealed bilateral anterior uveitis, vitritis, macular edema, and Frosted Branch Angiitis. Given a clinical suspicion of PSU, blood and serology tests were performed to rule out other causes of vasculitis and retinitis. RESULTS: Serologies came back negative except for highly elevated antistreptolysin-O titers. Topical and oral corticosteroids normalized the patient's visual acuity and clinical findings within a few weeks. A subsequent anterior-only recurrence was successfully resolved with topical treatment. CONCLUSIONS: Although PSU most commonly manifests as anterior uveitis, it may present with involvement of the posterior pole. To achieve a correct diagnosis, clinical suspicion and assessment of antistreptolysin-O titers as well as ruling out other conditions with similar clinical features are the mainstay approaches to diagnosis. Prognosis is generally good.

Valores referenciales de antiestreptolisina O y portadores asintomáticos de estreptococos β-hemolíticos en adolescentes y adultos del Municipio Francisco Linares Alcántara, Venezuela / Reference valúes antistreptolysin O and asymptomatic carriers of β-hemolytic streptococci in adolescents and adults at Municipality Francisco Linares Alcántara, Venezuela

Introduction: β-hemolytic streptococci (Streptococcus pyogenes) groups A, C or G, secretes streptolysin O, toxin which causes in the infected individual an adaptive humoral immune response with production of serum antibodies called anti-streptolysin O (ASO). Objectives: To determine the reference value of ASO in a sample of 159 individuals aged 16-72 years from municipality Francisco Linares Alcántara, Aragua state, applying indirect (passive) agglutination test. By using a throat swab sample which was sown in blood agar 5% the frequency of asymptomatic carriers of β-hemolytic streptococci was also determined. Results and Discussion: As reference value for determining ASO by agglutination method a title of up to 200 IU/mL was obtained, this reference value differs from that recommended by the commercial equipment. Asymptomatic carriers frequency was 21.2% (n = 34). The distribution of β-hemolytic streptococci isolated were: group A (17.6%), group B (32.3%), group C (20.5%), group D (2.9%), group F (8.8%), group G (14.7%) and unclusterable (2.9%). Conclusions: The new ASO reference value for teens and adults of the mentioned municipality is up to 200 IU/mL. β-hemolytic Streptococcus group B was the most frequently isolated.

Introducción: Los estreptococos β-hemolíticos del grupo A (Streptococcus pyogenes), C o G, secretan estreptolisina O, toxina que causa en el individuo infectado una respuesta inmune adaptativa humoral con producción de anticuerpos séricos denominados antiestreptolisina O (ASO). Objetivos: Determinar el valor referencial de ASO en una muestra poblacional de 159 individuos con edades comprendidas entre 16 y 72 años del municipio Francisco Linares Alcántara, estado Aragua mediante aglutinación (pasiva) indirecta. También se determinó la frecuencia de portadores asintomáticos de estreptococos β-hemolíticos utilizando una muestra de exudado faríngeo que se sembró en agar sangre de cordero al 5%. Resultados y Discusión: Como valor referencial para la determinación de ASO por el método de aglutinación se obtuvo un título de hasta 200 UI/mL, valor que difiere del recomendado por el kit comercial. La frecuencia de portadores fue 21,2% (n = 34). La distribución de los estreptococos β-hemolíticos aislados fue: grupo A (17,6%), grupo B (32,3%), grupo C (20,5%), grupo D (2,9%), grupo F (8,8%), grupo G (14,7%) y no agrupable (2,9%). Conclusiones: El nuevo valor referencial de ASO para adolescentes y adultos del municipio mencionado es hasta 200 UI/mL. Streptococcus β-hemolítico del grupo B fue el grupo más frecuentemente aislado.

Increased ß-haemolytic group A streptococcal M6 serotype and streptodornase B-specific cellular immune responses in Swedish narcolepsy cases.

BACKGROUND: Type 1 narcolepsy is a neurological disorder characterized by excessive daytime sleepiness and cataplexy associated with the HLA allele DQB1*06:02. Genetic predisposition along with external triggering factors may drive autoimmune responses, ultimately leading to the selective loss of hypocretin-positive neurons. OBJECTIVE: The aim of this study was to investigate potential aetiological factors in Swedish cases of postvaccination (Pandemrix) narcolepsy defined by interferon-gamma (IFNγ) production from immune cells in response to molecularly defined targets. METHODS: Cellular reactivity defined by IFNγ production was examined in blood from 38 (HLA-DQB1*06:02(+) ) Pandemrix-vaccinated narcolepsy cases and 76 (23 HLA-DQB1*06:02(+) and 53 HLA-DQB1*06:02(-) ) control subjects, matched for age, sex and exposure, using a variety of different antigens: ß-haemolytic group A streptococcal (GAS) antigens (M5, M6 and streptodornase B), influenza (the pandemic A/H1N1/California/7/09 NYMC X-179A and A/H1N1/California/7/09 NYMC X-181 vaccine antigens, previous Flu-A and -B vaccine targets, A/H1N1/Brisbane/59/2007, A/H1N1/Solomon Islands/3/2006, A/H3N2/Uruguay/716/2007, A/H3N2/Wisconsin/67/2005, A/H5N1/Vietnam/1203/2004 and B/Malaysia/2506/2004), noninfluenza viral targets (CMVpp65, EBNA-1 and EBNA-3) and auto-antigens (hypocretin peptide, Tribbles homolog 2 peptide cocktail and extract from rat hypothalamus tissue). RESULTS: IFN-γ production was significantly increased in whole blood from narcolepsy cases in response to streptococcus serotype M6 (P = 0.0065) and streptodornase B protein (P = 0.0050). T-cell recognition of M6 and streptodornase B was confirmed at the single-cell level by intracellular cytokine (IL-2, IFNγ, tumour necrosis factor-alpha and IL-17) production after stimulation with synthetic M6 or streptodornase B peptides. Significantly, higher (P = 0.02) titres of serum antistreptolysin O were observed in narcolepsy cases, compared to vaccinated controls. CONCLUSION: ß-haemolytic GAS may be involved in triggering autoimmune responses in patients who developed narcolepsy symptoms after vaccination with Pandemrix in Sweden, characterized by a Streptococcus pyogenes M-type-specific IFN-γ cellular immune response.

Reference values for serum antistreptolysin O in Chinese adult men from a Chinese male population survey.

BACKGROUND: Numerous diseases come from streptococcal infection. The antistreptolysin O (ASO) test is the most widely used serological test for streptococcal infection applied for the clinical diagnosis. The aim of this study was to establish reference values for serum ASO in Han and Zhuang Chinese from a Chinese male population survey. METHODS: A cross-sectional study in which 1,762 serum samples were collected between September 2009 and December 2009 from Chinese men aged 20 to 69 years. The reference values for serum ASO were measured with the immunonephelometric method on the Hitachi 7600 autoanalyzer. RESULTS: Serum ASO values were not normally distributed but log normally distributed. The reference value was < or = 125.00 IU/mL for serum ASO. A significant decrease with age was found, and age-dependent reference values were calculated for serum ASO levels. The Zhuang and Han groups (p < 10(-3)) exhibited differences in serum ASO. There was no significant difference according to smoking (p = 0.718) and drinking (p = 0.388) in Zhuang. However, there was a significant difference between smoking and non-smoking (p < 10(-3)) in the Han ethnic group, whereas there was no significant difference between the non-drinking and drinking groups (p = 0.245). CONCLUSIONS: The reference values for serum ASO for healthy adult men showed a huge deviation compared to the currently used reference value (200 IU/mL). Thus, the reference values for serum ASO should be established for different ethnic groups and regions.

Raised serum levels of interleukins 6 and 8 and antiphospholipid antibodies in an adult patient with Henoch-Schönlein purpura.

BACKGROUND: The long-term prognosis of Henoch-Schönlein purpura (HSP) is determined by the severity of renal involvement, known as HSP nephritis, which varies considerably from patient to patient. There is now increasing evidence that dysregulated cytokine production plays a crucial role in human autoimmune and inflammatory processes. AIM: To explore the possible contributions of serum antistreptolysin O, C-reactive protein, IgA, interleukin (IL)-6, IL-8, tumour necrosis factor (TNF)-α, anticardiolipin antibody (aCL) and antiphosphatidylserine-prothrombin complex antibody (anti-PSPT) in the pathogenesis of HSP, and to evaluate correlations between those biological parameters and the clinical features. METHODS: Records were reviewed of 58 patients with HSP who presented initially with palpable purpura between 2003 and 2009. Serum IL-6 levels were determined by chemiluminescent enzyme immunoassay, IL-8 levels by ELISA and TNF-α levels by quantitative sandwich enzyme immunoassay. Serum aCL and anti-PSPT levels were measured according to our previously published procedures. RESULTS: There was a significant correlation between the serum IL-6 and IgA anti-PSPT levels, and also between the serum IL-8 and IgA anti-PSPT levels. Serum IL-8 and IgA aCL levels were both significantly higher in patients with renal involvement than in those without. Serum IL-6 and IgM anti-PSPT levels were also significantly higher in patients with gastrointestinal symptoms than in those without. CONCLUSIONS: We suggest that serum IL-6 and IL-8 associated with antiphospholipid antibodies play a pivotal role in the induction of HSP. Based on our results, IL-8 and IgA aCL levels could be useful as markers to monitor the development of HSP nephritis, and IL-6 and IgM anti-PSPT levels could be used as markers to monitor the development of gastrointestinal involvement.

Henoch-Schönlein purpura with hypocomplementemia.

BACKGROUND: Abnormalities of the complement system in Henoch-Schönlein purpura (HSP) have been reported, but how this abnormality in the complement system impacts on the prognosis of HSP remains unknown. METHODS: We retrospectively studied patients hospitalized for HSP in the Children's Hospital Affiliated to Soochow University between October 2010 and May 2011. Patients with HSP and hypocomplementemia were the cases, and those without hypocomplementemia were the HSP controls. Another group of children (n = 50) with upper respiratory tract infections, but without HSP acted as negative controls. RESULTS: A total number of 338 HSP patients were included in this study (n = 53 cases, n = 285 controls). In the cases, C3 and C4 levels decreased in 29 patients, C3 was low in 6, and C4 in 18. Complement levels returned to normal within 3 months in all HSP patients except one. Case group patients had higher levels of serum IgG and arthralgia, as well as positive titers of antistreptolysin-O. Rates of abdominal pain, gastrointestinal bleeding, Henoch-Schönlein purpura nephritis (HSPN), and serum IgA and IgM levels were similar in the two HSP groups. CONCLUSION: Hypocomplementemia associated with HSP is a transient phenomenon. The incidence of significant sequelae such as HSPN between patients with and without hypocomplementemia does not differ.

Use of serum antistreptolysin O titers in the microbial diagnosis of orthopedic infections.

The utility of serologic tests in the microbial diagnosis of orthopedic infections is unknown. Antistreptolysin O titer determination is inexpensive and accurate in the diagnosis of beta-hemolytic group A, C, and G streptococci. In patients with negative culture results and positive titers, antibiotics might be reduced to the narrowest spectrum, penicillin.

[Scarlet fever with multisystem organ failure and hypertrophic gastritis]. / Scarlatine compliquée d'une défaillance multiviscérale avec gastrite hypertrophique.

Scarlet fever is a rare disease in adult patients. We report a patient in whom scarlet fever was associated with hypertrophic gastritis and multiple organ failure. A 62-year-old woman presented with septic shock and multiple organ failure. Bacteriological survey was negative. Abdominal tomodensitometry showed an hypertrophic gastritis. Histological analysis demonstrated a non specific gastritis without any tumoral sign. Cefotaxime and amoxicillin led to improvement and hypertrophic gastritis progressively resolved. A sandpaper rash over the body with finger desquamation, elevation of antistreptolysin O and a recent contact with an infected grandson led to the diagnosis of scarlet fever. Due to antibiotic prescription, scarlet fever is now uncommon. Although classical, ENT or gastroenteritis presentations may be puzzling for the diagnosis of scarlet fever. As 150 years ago, diagnosis of scarlet fever is still a clinical challenge.