Autoimmune and inflammatory manifestations occur frequently in patients with primary immunodeficiencies.

BACKGROUND: Primary immunodeficiencies (PIDs) are inherited diseases associated with a considerable increase in susceptibility to infections. It is known that PIDs can also predispose to cancer and immune diseases, including allergy, autoimmunity, and inflammation. OBJECTIVE: We aimed at determining the incidence of autoimmunity and inflammation in patients with PIDs. METHODS: We have retrospectively screened 2183 consecutive cases of PID in the Centre de Référence Déficits Immunitaires Héréditaires registry (CEREDIH; the French national PID registry) for the occurrence of autoimmunity and inflammation. RESULTS: One or more autoimmune and inflammatory complications were noted in 26.2% of patients, with a risk of onset throughout the patient's lifetime. The risk of autoimmune cytopenia was at least 120 times higher than in the general population, the risk of inflammatory bowel disease in children was 80 times higher, and the risk of other autoimmune manifestations was approximately 10 times higher. Remarkably, all types of PIDs were associated with a risk of autoimmune and inflammatory complications, although the greatest risk was associated with T-cell PIDs and common variable immunodeficiency. The occurrence of autoimmune disease is a negative prognostic factor for survival. CONCLUSIONS: Our results provide the basis for a detailed prospective evaluation of autoimmunity and inflammation in the context of PIDs, with a view to accurately assessing these risks and describing the possible effect of medical intervention.

A Framework Proposal for Quality and Safety Measurement in Gynecologic Emergency Care.

OBJECTIVE: To define and assess the prevalence of potentially life-threatening gynecologic emergencies among women presenting for acute pelvic pain for the purpose of developing measures to audit quality of care in emergency departments. METHODS: We conducted a mixed-methods multicenter study at gynecologic emergency departments in France and Belgium. A modified Delphi procedure was first conducted in 2014 among health care professionals to define relevant combinations of potentially life-threatening conditions and near misses in the field of gynecologic emergency care. A prospective case-cohort study in the spring of 2015 then assessed the prevalence of these potentially life-threatening emergencies and near misses among women of reproductive age presenting for acute pelvic pain. Women in the case group were identified at 21 participating centers. The control group consisted of a sample of women hospitalized for acute pelvic pain not caused by a potentially life-threatening condition and a 10% random sample of outpatients. RESULTS: Eight gynecologic emergencies and 17 criteria for near misses were identified using the Delphi procedure. Among the 3,825 women who presented for acute pelvic pain, 130 (3%) were considered to have a potentially life-threatening condition. The most common diagnoses were ectopic pregnancies with severe bleeding (n=54; 42%), complex pelvic inflammatory disease (n=30; 23%), adnexal torsion (n=20; 15%), hemorrhagic miscarriage (n=15; 12%), and severe appendicitis (n=6; 5%). The control group comprised 225 hospitalized women and 381 outpatients. Diagnostic errors occurred more frequently among women with potentially life-threatening emergencies than among either hospitalized (odds ratio [OR] 1.7, 95% CI 1.1-2.7) or outpatient (OR 14.7, 95% CI 8.1-26.8) women in the control group. Of the women with potentially life-threatening conditions, 26 met near-miss criteria compared with six with not potentially life-threatening conditions (OR 25.6, 95% CI 10.9-70.7). CONCLUSIONS: Potentially life-threatening gynecologic emergencies are high-risk conditions that may serve as a useful framework to improve quality and safety in emergency care.