BackgroundAn outbreak of haemolytic uraemic syndrome (HUS) due to Shiga toxin-secreting Escherichia coli (STEC) O104:H4 from contaminated fenugreek sprouts occurred in June 2011 near Bordeaux, France. In the context of this outbreak, all patients were treated with the monoclonal anti-C5 antibody, eculizumab.MethodsThe diagnosis of HUS was made based on haemolytic anaemia, low platelet count and acute kidney injury. Data were obtained from initial gastrointestinal symptoms to the end of follow-up 10 weeks after the start of eculizumab.ResultsAmong 24 cases of STEC gastroenteritis, HUS developed in nine patients (eight adults and one child), 6 (median; range 3–12) days after digestive symptoms begun. The median (range) highest or lowest biological values were platelet count 26 (range 14–93) G/L; haemoglobin 6.6 (range 5–10.7) g/dL; LDH 1520 (range 510–2568) IU/L; creatinine 152 (range 48–797) µmol/L. All patients had extra-renal complications (liver 9, pancreas 5, brain 3 and heart 3). Two patients were dialysed, and one was ventilated. After failure of plasma exchange to increase platelets in the first three patients, eculizumab was administered in all nine patients, 0–4 days after HUS diagnosis (median 1 day). One patient with very severe neurological HUS received immunoadsorption. Outcome was favourable in all patients, with rapid normalization of haemoglobin, platelets, LDH levels, renal function and neurological improvement. There were no deaths and no serious adverse events related to eculizumab.ConclusionsEarly treatment of O104:H4 STEC-HUS by eculizumab was associated with a rapid and efficient recovery. Controlled prospective evaluation of eculizumab in STEC-HUS is warranted.

SUMMARY BackgroundMucosal healing has become a new therapeutic goal in Crohn's disease and can be achieved with azathioprine (AZA) or biologics. Methotrexate (MTX) is an effective drug for both the induction and maintenance of remission in Crohn's disease. However, mucosal healing with MTX has been poorly investigated.

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A 73-year-old woman was admitted after the first upper gastric tract haemorrhage due to gastric variceal bleeding. A CREST syndrome associated with Hashimoto's thyroiditis, Gougerot-Sjögren syndrome, cryoglobulinaemia and complicated with severe pulmonary hypertension was diagnosed. Liver histology found precirrhotic lesions of primary biliary cirrhosis (PBC) and nodular regenerative hyperplasia (NRH). Collagen diseases are often associated with liver test abnormalities and liver disease usually associated with CREST syndrome is PBC. NRH has been found in association with collagen diseases but also with haematological diseases or drugs or with autoimmune diseases, such as PBC. This case shows the association of PBC and NRH with porto pulmonary hypertension in CREST syndrome.