Vasculitis and kidney involvement in pregnancy: evidence-based medicine and ethics bear upon clinical choices.

Wegener’s granulomatosis is a rare, protean disease, with even sex distribution and higher incidence in Caucasians. Its yearly incidence is 5–12 per 1 000 000 [1] and it displays a wide range of clinical presentations from a mild, localised lesion to widespread severe disease [2]. The age range is wide, with the peak in the 40s. Women in the childbearing age group are not spared, and pregnancy presents a critical condition both for the onset of the disease and for its flare-ups [2,3]. The decision of a patient with Wegener’s granulomatosis to become pregnant presents a clinical dilemma [3]. To face it in the era of evidence-based medicine (EBM) and of patients’ right of self-determination, two tools, systematic literature search and physician– patient therapeutic alliance, were used together (as recently suggested), integrating narrative and EBM problem solving approaches [4]. To address a patient’s questions, the search strategy shown in Table 1 was performed on Medline and Embase, combining terms (Mesh–Emtree and free terms) referring to Wegener’s granulomatosis and those related to pregnancy. In all, reports on 28 pregnancies were retrieved (Table 1); outcome data were available for 27 of those. The diagnosis of Wegener’s granulomatosis occurred in pregnancy in eight out of 28 patients; 19 out of 27 pregnancies ended in live births, seven in abortions and two in maternal deaths (Table 1). In the reviewed literature, the most common advice was to not start a pregnancy in the presence of active disease: with a clear note of caution expressed in all cases. The quality of the evidence available, however, was poor, consisting of case series and case reports, presumed to be tainted by publication biases; however, such biases affect the study and discussion of all rare diseases, especially if, as in the case of pregnancy, the condition cannot feasibly be randomized for study. The present updating, performed after the patients decision, added only one paper to the ones retrieved by the search strategy performed at patients referral [5].