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The article describes twp cases of anti-neutrophil cytoplasm antibodies (ANCA)-negative eosinophilic granulomatosis with polyangiitis (EGPA) complicated by eosinophilic fasciitis (EF). A 35-year-old patient developed painful indurations of the lower legs ten years after EGPA onset while in a 22-year-old patient, confirmed EF preceded the diagnosis of EGPA by nine years. Also mentioned are the frequent EGPA manifestations shared by both patients.

Background. Shiga toxin-producing, enteroaggregative Escherichia coli was responsible for the 2011 outbreak of haemolytic uraemic syndrome (HUS). The present single-centre, observational study describes the 1-year course of the disease with an emphasis on kidney function. Outcome data after 1 year are associated with treatment and patient characteristics at onset of HUS. Methods. Patients were treated according to a standardized approach of supportive care, including a limited number of plasmapheresis. On top of this treatment, patients with severe HUS (n = 35) received eculizumab, a humanized anti-C5 monoclonal antibody inhibiting terminal complement activation. The per-protocol decision--to start or omit an extended therapy with eculizumab accompanied by azithromycin--separated the patients into two groups and marked Day 0 of the prospective study. Standardized visits assessed the patients' well-being, kidney function, neurological symptoms, haematological changes and blood pressure. Results. Fifty-six patients were regularly seen during the followup. All patients had survived without end-stage renal disease. Young(er) age alleviated restoring kidney function after acute kidney injury even in severe HUS. After 1 year, kidney function was affected with proteinuria [26.7%; 95% confidence interval (CI) 13.8-39.6], increased serum creatinine (4.4%, CI 0.0-10.4), increased cystatin C (46.7%, CI 32.1-61.3) and reduced (<90 mL/ min) estimated glomerular filtration rate (46.7%, CI 32.1-61.3). Nine of the 36 patients without previous hypertension developed de novo hypertension (25%, CI 10.9-39.1). All these patients had severe HUS. Conclusions. Although shiga toxin-producing Escherichia coli (STEC)-HUS induced by O104:H4 was a life-threatening acute disease, follow-up showed a good recovery of organ function in all patients. Whereas kidney function recovered even after longer duration of dialysis, chronic hypertension developed after severe HUS with neurological symptoms and could not be prevented by the extended therapy. [ABSTRACT FROM AUTHOR]

History and Clinical Findings: We report the case of a 30 years old man presenting with cough and a sore throat. The physical examination showed a painful neck, a scleral icterus, inflammation of the posterior pharyngeal wall and tonsils., Investigations: Initial laboratory studies revealed an increase in white blood cells, a pronounced reduction of platelets, an elevated CRP and renal failure. Ultrasound examination of his neck showed a thrombosis of the jugular vein. Blood cultures were drawn and led to the identification of fusobacterium necrophorum., Diagnosis: Lemierre's Syndrom accompanied by purulent abscess-forming thrombophlebitis of the right external jugular vein., Treatment and Course: Antibiotic therapy was started with ceftazidime plus levofloxacin and was adjusted to ampicillin plus clindamycin. As resistance to ampicillin was detected, therapy was readjusted to meropenem. Inflammation, renal parameters, transaminases and bilirubin decreased. The patient improved clinically and was discharged after 19 days in hospital., Conclusion: The Lemierre's Syndrome is a rare and often underdiagnosed septic disease followed on pharyngeal infections leading to purulent thromboplebitis of small veins. An appropriate antibiotic therapy is mandatory for a successful treatment of this disease., Competing Interests: Die Autoren geben an, dass kein Interessenkonflikt besteht., (© Georg Thieme Verlag KG Stuttgart · New York.)

ANG II interacts with the sympathetic nervous system at central nervous blood pressure-regulating structures, including the baroreflex. It is unknown whether prolonged BP elevation mediated by high ANG II plasma levels could induce a persistent shift of the central nervous baroreflex setpoint, lasting beyond the short ANG II plasmatic half time of a few seconds, thereby consolidating elevated BP and/or increased SNA in healthy humans. In a blinded crossover design, ANG II or placebo (saline) was infused for a 6-h period in 12 resting normotensive students (6 males, 6 females) raising BP to borderline hypertensive levels. Between 60 and 120 min after the infusion period, muscle sympathetic nerve activity (MSNA) was assessed microneurographically and correlated with oscillometric BP measurements and heart rate at supine rest (baseline) and during pharmacologic baroreceptor challenge. Infusion of ANG II increased BP to borderline-hypertensive levels, as intended, whereas heart rate remained unaltered. At baroreflex assessment (i.e., 60-120 min after end of infusion period), systolic BP was significantly higher compared with placebo (Δ8.4 ± 3.1 mmHg; P < 0.05), whereas diastolic values were nearly equal between conditions. Baseline MSNA was neither decreased nor increased, and baroreflex sensitivity to vasoactive drug challenge was not altered. Our results show that elevation of ANG II plasma levels over 6 h was able to increase systolic, but not diastolic, BP far beyond blood-mediated ANG II effects. MSNA or heart rate did not counter-regulate this BP elevation, indicating that ANG II had sustainably reset the central nervous BP threshold of sympathetic baroreflex function to accept elevated BP input signals without counter-regulatory response., (Copyright © 2015 the American Physiological Society.)