Fertility, pregnancies and outcomes reported by females with common variable immune deficiency and hypogammaglobulinemia: results from an internet-based survey.

Adi V Gundlapalli,Christopher Scalchunes,Marcia Boyle,Harry R Hill

doi:10.1007/s10875-014-0123-3

Abstract

BackgroundIssues of fertility and pregnancy place an extra burden on females with primary immunodeficiencies. Patients lack reliable information and providers lack guidelines to counsel patients on these anxiety-provoking matters.ObjectiveTo collate concerns and experiences related to fertility and pregnancy from females with humoral immune deficiencies.MethodsWe conducted an internet-based survey of female patients who self-identified as having a diagnosis of primary humoral immune deficiency.ResultsResponses from 490 women with common variable immune deficiency and 100 with hypogammaglobulinemia were evaluated. The reported fertility measure (% of women who had had a birth) was statistically significantly lower as compared to the general US population (70 % vs. 85 %, p < 0.0001) whereas the rates of spontaneous pregnancy loss were comparable. This group reported a total of 966 pregnancies; 72 % resulted in a live birth. A majority of the pregnancies progressed with no incident and with continuation of their IgG replacement therapy; 23 % reported an increase in IgG dosing during pregnancy. Only 15 % of those reporting a first pregnancy indicated that they had been diagnosed with immune deficiency prior to their first pregnancy; these women expressed concern regarding the effect of immune deficiency on their fertility, pregnancy and decision to have children.ConclusionWith inherent limitations of self-reported responses to surveys, females with humoral immune deficiencies reported relatively good rates of fertility and pregnancies ending in live births. Results of the survey will serve as peer support for patients and inform counseling guidelines for providers.Electronic supplementary materialThe online version of this article (doi:10.1007/s10875-014-0123-3) contains supplementary material, which is available to authorized users.

Highlights

Primary immunodeficiency disorders (PI), such as common variable immune deficiency (CVID) and hypogammaglobulinemia, affect a small but significant proportion of the population
A majority of the pregnancies progressed with no incident and with continuation of their immunoglobulin G (IgG) replacement therapy; 23 % reported an increase in IgG dosing during pregnancy
490 reported a diagnosis of CVID and 100 reported hypogammaglobulinemia, resulting in a cohort of 590 females whose survey responses were analyzed for this study

Summary

Introduction

Primary immunodeficiency disorders (PI), such as common variable immune deficiency (CVID) and hypogammaglobulinemia, affect a small but significant proportion of the population. The exact prevalence of CVID is not known, it is estimated that 1 in 25,000 to 1 in 50, 000 are affected in the US with equal numbers of males and females [2, 3]. The etiology of these conditions is likely multifaceted and polygenic with complex inheritance patterns [4]. Besides low IgG levels, patients have decreased levels of immunoglobulins such as IgA and/or IgM The diagnosis of these conditions is often delayed [3, 5] and is made with a compatible history and immunological testing. Patients lack reliable information and providers lack guidelines to counsel patients on these anxiety-provoking matters

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Results

Discussion

Conclusion