Format

Send to

Choose Destination
Clin Exp Immunol. 2016 Apr;184(1):73-82. doi: 10.1111/cei.12748. Epub 2016 Jan 27.

Clinical and laboratory correlates of lung disease and cancer in adults with idiopathic hypogammaglobulinaemia.

Author information

1
Paediatric Allergy and Immunology, University of Manchester, Manchester.
2
UK-PIN UKPID Registry Team, London and Manchester.
3
Immunology, Royal Free Hospital, London.
4
Immunology, St Bartholomew's Hospital, London.
5
West Midlands Immunodeficiency Centre, Birmingham Heartlands Hospital, Birmingham.
6
Immunology, Derriford Hospital, Plymouth.
7
Immunology, NHS Greater Glasgow and Clyde, Glasgow.
8
Immunology, John Radcliffe Hospital, Oxford.
9
Department of Immunology, University Hospital of Wales, Cardiff.
10
Immunology, Salford Royal Foundation Trust, Manchester.
11
Immunology, Addenbrookes Hospital, Cambridge.
12
Immunology, Papworth Hospital, Cambridge.
13
Regional Immunology Service, the Royal Hospitals, Belfast.
14
Department of Immunology, Manchester Royal Infirmary, Manchester.
15
Primary Immunodeficiency Group, Newcastle University, Newcastle upon Tyne, UK.

Abstract

Idiopathic hypogammaglobulinaemia, including common variable immune deficiency (CVID), has a heterogeneous clinical phenotype. This study used data from the national UK Primary Immune Deficiency (UKPID) registry to examine factors associated with adverse outcomes, particularly lung damage and malignancy. A total of 801 adults labelled with idiopathic hypogammaglobulinaemia and CVID aged 18-96 years from 10 UK cities were recruited using the UKPID registry database. Clinical and laboratory data (leucocyte numbers and serum immunoglobulin concentrations) were collated and analysed using uni- and multivariate statistics. Low serum immunoglobulin (Ig)G pre-immunoglobulin replacement therapy was the key factor associated with lower respiratory tract infections (LRTI) and history of LRTI was the main factor associated with bronchiectasis. History of overt LRTI was also associated with a significantly shorter delay in diagnosis and commencing immunoglobulin replacement therapy [5 (range 1-13 years) versus 9 (range 2-24) years]. Patients with bronchiectasis started immunoglobulin replacement therapy significantly later than those without this complication [7 (range 2-22) years versus 5 (range 1-13) years]. Patients with a history of LRTI had higher serum IgG concentrations on therapy and were twice as likely to be on prophylactic antibiotics. Ensuring prompt commencement of immunoglobulin therapy in patients with idiopathic hypogammaglobulinaemia is likely to help prevent LRTI and subsequent bronchiectasis. Cancer was the only factor associated with mortality. Overt cancer, both haematological and non-haematological, was associated with significantly lower absolute CD8(+) T cell but not natural killer (NK) cell numbers, raising the question as to what extent immune senescence, particularly of CD8(+) T cells, might contribute to the increased risk of cancers as individuals age.

KEYWORDS:

bronchiectasis; cancer; common variable immunodeficiency; idiopathic hypogammagloblinaemia

PMID:
26646609
PMCID:
PMC4778100
DOI:
10.1111/cei.12748
[Indexed for MEDLINE]
Free PMC Article

Supplemental Content

Full text links

Icon for Wiley Icon for PubMed Central
Loading ...
Support Center