Creation of a model to predict survival in patients with refractory coeliac disease using a multinational registry.

BACKGROUND: Refractory coeliac disease is a severe complication of coeliac disease with heterogeneous outcome. AIM: To create a prognostic model to estimate survival of patients with refractory coeliac disease. METHODS: We evaluated predictors of 5-year mortality using Cox proportional hazards regression on subjects from a multinational registry. Bootstrap resampling was used to internally validate the individual factors and overall model performance. The mean of the estimated regression coefficients from 400 bootstrap models was used to derive a risk score for 5-year mortality. RESULTS: The multinational cohort was composed of 232 patients diagnosed with refractory coeliac disease across seven centres (range of 11-63 cases per centre). The median age was 53 years and 150 (64%) were women. A total of 51 subjects died during a 5-year follow-up (cumulative 5-year all-cause mortality = 30%). From a multiple variable Cox proportional hazards model, the following variables were significantly associated with 5-year mortality: age at refractory coeliac disease diagnosis (per 20 year increase, hazard ratio = 2.21; 95% confidence interval, CI: 1.38-3.55), abnormal intraepithelial lymphocytes (hazard ratio = 2.85; 95% CI: 1.22-6.62), and albumin (per 0.5 unit increase, hazard ratio = 0.72; 95% CI: 0.61-0.85). A simple weighted three-factor risk score was created to estimate 5-year survival. CONCLUSIONS: Using data from a multinational registry and previously reported risk factors, we create a prognostic model to predict 5-year mortality among patients with refractory coeliac disease. This new model may help clinicians to guide treatment and follow-up.

Measurement of serum levels of eosinophil cationic protein in the diagnosis of acute gastrointestinal anisakiasis.

Thirty-two patients with abdominal pain and/or intestinal pseudo-obstruction who had consumed raw or undercooked fish in the previous 72 h, were included in a study of anisakiasis, a parasitation of the human gastrointestinal tract by third stage Anisakis simplex larvae. Skin prick test (SPT) against A. simplex were positive in all the patients. High median eosinophil cationic protein (ECP) serum concentrations (> 15 mg/L) at day 0 with normal serum levels at day 30 and a rise of median total and specific IgE against A. simplex at day 30, were observed. We conclude that a raised serum level of ECP in the first 72 h from the onset of symptoms coinciding with a positive SPT against A. simplex and high total and specific immunoglobulin (IgE) in the first month after the parasitation, could be a useful tool in the diagnosis of gastrointestinal anisakiasis, even if the parasite cannot be isolated.

Steroidal management and serum cytokine profile of a case of alcoholic hepatitis with leukemoid reaction.

Leukemoid reactions (LRs) are rare in alcoholic hepatitis (AH), but they are a sign of poor prognosis. The treatment of AH with corticosteroids is controversial, though several reports suggest that these should be used in severe cases of AH. We report a case of AH-associated LRs that presented with an increase of the serum concentrations of the proinflammatory cytokines interleukin (IL)-18 (an initiator of inflammation) and IL-1beta (likely responsible for the neutrophilia of the LRs). These findings provided a pathogenic indication for the use of corticosteroids (that block the transcription of IL-1beta), and this approach achieved a clinical and analytical recovery in our patient. This pathogenic mechanism might also underlie other cases of LRs and other complications of AH, thus providing a rationale for the benefits of corticotherapy in these rare but severe conditions.