title: Comparison of diagnostic criteria for common variable immunodeficiency disorder.
abstract: Common variable immunodeficiency disorders (CVIDs) are the most frequent symptomatic primary immune deficiency condition in adults. The genetic basis for the condition is not known and no single clinical feature or laboratory test can establish the diagnosis; it has been a diagnosis of exclusion. In areas of uncertainty, diagnostic criteria can provide valuable clinical information. Here, we compare the revised European society of immune deficiencies (ESID) registry (2014) criteria with the diagnostic criteria of Ameratunga et al. (2013) and the original ESID/pan American group for immune deficiency (ESID/PAGID 1999) criteria. The ESID/PAGID (1999) criteria either require absent isohemagglutinins or impaired vaccine responses to establish the diagnosis in patients with primary hypogammaglobulinemia. Although commonly encountered, infective and autoimmune sequelae of CVID were not part of the original ESID/PAGID (1999) criteria. Also excluded were a series of characteristic laboratory and histological abnormalities, which are useful when making the diagnosis. The diagnostic criteria of Ameratunga et al. (2013) for CVID are based on these markers. The revised ESID registry (2014) criteria for CVID require the presence of symptoms as well as laboratory abnormalities to establish the diagnosis. Once validated, criteria for CVID will improve diagnostic precision and will result in more equitable and judicious use of intravenous or subcutaneous immunoglobulin therapy.
authors.affiliation: Department of Virology and Immunology, Auckland Hospital , Auckland , New Zealand ; Department of Clinical Immunology, Auckland Hospital , Auckland , New Zealand.
authors.forename: Rohan
authors.initials: R
authors.lastname: Ameratunga
authors.affiliation: Department of Clinical Immunology, Royal Melbourne Hospital , Melbourne, VIC , Australia.
authors.forename: Maia
authors.initials: M
authors.lastname: Brewerton
authors.affiliation: Department of Clinical Immunology, Royal Melbourne Hospital , Melbourne, VIC , Australia.
authors.forename: Charlotte
authors.initials: C
authors.lastname: Slade
authors.affiliation: Department of Clinical Immunology, Auckland Hospital , Auckland , New Zealand.
authors.forename: Anthony
authors.initials: A
authors.lastname: Jordan
authors.affiliation: Department of Clinical Immunology, Royal Brisbane Hospital , Brisbane, QLD , Australia.
authors.forename: David
authors.initials: D
authors.lastname: Gillis
authors.affiliation: Department of Virology and Immunology, Auckland Hospital , Auckland , New Zealand.
authors.forename: Richard
authors.initials: R
authors.lastname: Steele
authors.affiliation: Department of Virology and Immunology, Auckland Hospital , Auckland , New Zealand.
authors.forename: Wikke
authors.initials: W
authors.lastname: Koopmans
authors.affiliation: Department of Virology and Immunology, Auckland Hospital , Auckland , New Zealand.
authors.forename: See-Tarn
authors.initials: ST
authors.lastname: Woon
keywords: HGUS; common variable immunodeficiency; diagnostic criteria