Immunogenicity of live phoP gene deletion strain of Riemerella anatipestifer serotype 1.

Duck infectious serositis is an acute and infectious disease caused by Riemerella anatipestifer (R. anatipestifer) that leads to perihepatitis, pericarditis, meningitis, and airbag inflammation in ducks, which causes serious economic losses to the global duck industry. The phoP/phoR is a novel 2-component signal transduction system first reported in gram-negative bacteria, of which phoP acts as a global regulator and virulence factor. In this study, the phoP gene from the R. anatipestifer YM strain was knocked out using homologous recombination technology and replaced with the spectinomycin resistance gene (Spec). The virulence of the R. anatipestifer YMΔphoP strain was reduced by approximately 47,000 times compared to that of the wild-type R. anatipestifer YM strain. Ducks were immunized with live R. anatipestifer YMΔphoP strain by subcutaneous inoculation at a dose of 106 to 107 CFU (0.2 mL per duck) and challenged with the wild-type R. anatipestifer YM strain 14 days later. The protection rate in the immunized group was 100%. The growth characteristics of ducks in the immunized and negative control groups were normal, and the research demonstrated R. anatipestifer YMΔphoP strain have suitable immunogenicity and protective effects. Thus, the study findings suggest that the novel R. anatipestifer YMΔphoP strain may provide a candidate for the development of a gene deletion activated vaccine against duck infectious serositis.

Genetic and epigenetic alterations of cyclic AMP response element modulator in rheumatoid arthritis.

BACKGROUND: Genetic and epigenetic factors are strongly associated with the autoimmune disease rheumatoid arthritis (RA). Cyclic AMP response element modulator (CREM), a gene related to immune system regulation, has been implicated in various immune-mediated inflammatory processes, although it remains unknown whether CREM is involved in RA. METHODS: This study enrolled 278 RA patients and 262 controls. Three variants [rs12765063, rs17499247, rs1213386] were identified through linkage disequilibrium and expression quantitative trait locus analysis, and CREM transcript abundance was determined by quantitative real-time polymerase chain reaction. The identified variants were genotyped using the TaqMan Allelic Discrimination assay, and CREM promoter methylation was assessed by bisulphite sequencing. Differences between groups and correlations between variables were assessed with Student's t-tests and Pearson's correlation coefficients. Associations between phenotypes and genotypes were evaluated with logistic regression. RESULTS: Rheumatoid arthritis patients exhibited increased CREM expression (p < .0001), which was decreased by methotrexate (p = .0223) and biologics (p = .0001), but could not be attributed to CREM variants. Interestingly, rs17499247 displayed a significant association with serositis (p = .0377), and rs1213386 increased the risk of lymphadenopathy (p = .0398). Furthermore, seven CpG sites showed decreased methylation in RA (p = .0477~ p < .0001). CONCLUSIONS: Collectively, our results indicate that CREM hypomethylation and CREM upregulation occur in RA and that CREM variants are involved in the development of serositis and lymphadenopathy in RA. This study highlights the novel roles of CREM in RA pathophysiology.

A familial Mediterranean fever patient with double homozygous mutations treated with anakinra after kidney transplantation / Un paciente con poliserositis familiar recurrente con mutaciones homocigóticas dobles tratado con anakinra tras un trasplante renal

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Polirradiculoneuritis, síndromes periódicos asociados a criopirina y fiebre mediterránea familiar / Polyradiculoneuritis, cryopyrin-associated periodic syndromes, and familial Mediterranean fever

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[Polyserositis in a female patient with hypothyroidism]. / Poliserozyt u khvoroï na hipotyreoz.

A case is described of hypothyrosis, with an exudate coming into the serous cavities (pericardium, pleura, peritoneum) being a predominant symptom in the clinical picture of the condition. It took three months for the events related to polyserositis to dispel completely as a result of the substitution hormonotherapy. There were no sings of decomposition of hypothyrosis (recurrence of exudation in the serous cavities) during a 6-year prophylactic management. The authors maintain that polyserositis is suggestive of hypothyrosis, so can be of help in the diagnosis of this medical condition.

A syndrome of fibrosing pleuritis, pericarditis, and synovitis with infantile contractures of fingers and toes in 2 sisters: "familial fibrosing serositis".

We describe a family in which 2 sisters born to consanguineous parents developed childhood onset fibrosing pleuritis in association with constrictive pericarditis and bilateral deforming arthropathy of large and small joints of upper and lower extremities including flexion contractures of several fingers (camptodactyly) and toes. One patient also had mitral value prolapse. Histopathological examination of the synovium and pericardium revealed fibrosis, and ultrastructural study of synovium showed abundant inter and intracellular mesh of 9 nm microfibrils. We describe this distinct clinicopathological entity with pleiotropic manifestations, the common features of which appears to be the fibrosis of serous membranes. Therefore, the term "familial fibrosing serositis" is proposed for this entity.

Serositis: comparative analysis of histological findings and pathogenetic mechanisms in nonbacterial serosal inflammation.

Peritonitis is the established term for infective inflammation of the peritoneum, while serositis generally refers to nonorganismal inflammation in any serous cavity, including the peritoneum. In continuous ambulatory peritoneal dialysis (CAPD) literature, however, culture-negative peritoneal inflammation is referred to as "sterile" or "chemical" peritonitis. These terms not only imply unwarranted etiologic assumptions, but may also deflect attention from the existence of medical conditions to which the peritoneum is subject. This is evident in CAPD literature where there is little recognition that the peritoneum, as a member of the serosa and a secretor of lamellar bodies, is prey to a wide range of disorders. Thus before, during, and after CAPD, the membrane is liable to fall victim to disease states unconnected with the process of dialysis. Significant peritoneal pathology occurs as part of a pan-serositis, which may be metabolic (uremia, cholesterolosis), autoimmune (systemic lupus erythematosus, rheumatoid disease, acute rheumatism, endocrinopathies), genetic (recurrent hereditary polyserositis), allergic (eosinophilic serositis), and granulomatous in nature. This paper presents a comparative analysis of histopathological presentation and pathogenetic mechanisms involved in all forms of peritoneal serositis. It incorporates recent advances in molecular biology of the membrane into a holistic reappraisal of peritoneal pathology, revealing hitherto unrecognized homologies in peritoneal reaction to diverse disorders.

Serositis with autoimmune endocrinopathy: clinical and immunogenetic features.

Twenty patients with autoimmune endocrinopathies experienced 45 episodes of pleural and/or pericardial serositis. Seventeen of these patients were women and 15 had clinical or serologic evidence of 2 or more endocrinopathies. Idiopathic primary hypoadrenalism (10 cases), Graves' disease (8 cases), Hashimoto's disease (4 cases), atrophic thyroiditis with hypothyroidism (3 cases), idiopathic primary hypogonadism (3 cases), transient thyroiditides (2 cases), and type I diabetes mellitus (1 case) were diagnosed at a mean age of 24 years. Serositis recurred after asymptomatic intervals of months to years even in patients treated for endocrine dysfunction. Fourteen of 16 Caucasians had circulating immune complexes, including all 9 patients with a C4AQ0 (C4A null) phenotype and including all 12 patients with HLA antigens B8 and DR3, antigens associated with systemic lupus and with autoimmune endocrinopathies. Serositides associated with autoimmune endocrinopathies can occur with chest pain, fever, and exudative effusions in young Caucasian women with the HLA B8 DR3 C4AQ0 phenotype. These serositides may have a common pathophysiologic mechanism.

Enrollment bias and variation in clinical manifestations: a review of consecutive cases of familial paroxysmal polyserositis.

The effect of systematic enrollment bias was studied in a series of 158 patients with familial paroxysmal polyserositis (F.P.P.) from a special clinic in a university hospital. The characteristics of 79 patients seen for the first time at this special clinic in its first 16 months of operation, were compared with the characteristics of 79 patients presented during the last 6 yr of its operation. As a group the patients studied first at the clinic had a more severe form of the disease. This group had a significantly larger number of patients with multiple clinical manifestations, with proteinuria and amyloidosis. In addition, there were more males and more patients with positive family history of the disease in this earlier group. It is probable that much of the temporal variation in these clinical characteristics between patients studied in the earlier and later periods can be explained by case selection originating from differentials in referral patterns. Thus, a larger proportion of the earlier patients were referred following an episode of hospitalization. Differences in referral pattern, in case selection and in the sources of data, can account for the significant variation in the frequency of the various clinical manifestations in the different published F.P.P. case series from several countries. The effect of enrollment bias on the results of clinical studies and the importance of a population base for such studies is emphasized.