Screening protocols to monitor respiratory status in primary immunodeficiency disease: findings from a European survey and subclinical infection working group

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Screening protocols to monitor respiratory status in primary immunodeficiency disease : findings from a European survey and subclinical infection working group. / Jolles, S.; Sánchez-Ramón, S.; Quinti, Isabella; Soler-Palacín, P.; Agostini, C.; Florkin, B.; Couderc, L. J.; Brodszki, N.; Jones, A.; Longhurst, Hilary; Warnatz, Klaus; Haerynck, F.; Matucci, A.; de Vries, Dick E.

In: Clinical and Experimental Immunology, Vol. 190, No. 2, 01.11.2017, p. 226-234.

Research output: Contribution to journalArticle

Harvard

Jolles, S, Sánchez-Ramón, S, Quinti, I, Soler-Palacín, P, Agostini, C, Florkin, B, Couderc, LJ, Brodszki, N, Jones, A, Longhurst, H, Warnatz, K, Haerynck, F, Matucci, A & de Vries, DE 2017, 'Screening protocols to monitor respiratory status in primary immunodeficiency disease: findings from a European survey and subclinical infection working group', Clinical and Experimental Immunology, vol. 190, no. 2, pp. 226-234. https://doi.org/10.1111/cei.13012

APA

CBE

Jolles S, Sánchez-Ramón S, Quinti I, Soler-Palacín P, Agostini C, Florkin B, Couderc LJ, Brodszki N, Jones A, Longhurst H, Warnatz K, Haerynck F, Matucci A, de Vries DE. 2017. Screening protocols to monitor respiratory status in primary immunodeficiency disease: findings from a European survey and subclinical infection working group. Clinical and Experimental Immunology. 190(2):226-234. https://doi.org/10.1111/cei.13012

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Author

Jolles, S. ; Sánchez-Ramón, S. ; Quinti, Isabella ; Soler-Palacín, P. ; Agostini, C. ; Florkin, B. ; Couderc, L. J. ; Brodszki, N. ; Jones, A. ; Longhurst, Hilary ; Warnatz, Klaus ; Haerynck, F. ; Matucci, A. ; de Vries, Dick E. / Screening protocols to monitor respiratory status in primary immunodeficiency disease : findings from a European survey and subclinical infection working group. In: Clinical and Experimental Immunology. 2017 ; Vol. 190, No. 2. pp. 226-234.

RIS

TY - JOUR

T1 - Screening protocols to monitor respiratory status in primary immunodeficiency disease

T2 - findings from a European survey and subclinical infection working group

AU - Jolles, S.

AU - Sánchez-Ramón, S.

AU - Quinti, Isabella

AU - Soler-Palacín, P.

AU - Agostini, C.

AU - Florkin, B.

AU - Couderc, L. J.

AU - Brodszki, N.

AU - Jones, A.

AU - Longhurst, Hilary

AU - Warnatz, Klaus

AU - Haerynck, F.

AU - Matucci, A.

AU - de Vries, Dick E.

PY - 2017/11/1

Y1 - 2017/11/1

N2 - Many patients with primary immunodeficiency (PID) who have antibody deficiency develop progressive lung disease due to underlying subclinical infection and inflammation. To understand how these patients are monitored we conducted a retrospective survey based on patient records of 13 PID centres across Europe, regarding the care of 1061 adult and 178 paediatric patients with PID on immunoglobulin (Ig) G replacement. The most common diagnosis was common variable immunodeficiency in adults (75%) and hypogammaglobulinaemia in children (39%). The frequency of clinic visits varied both within and between centres: every 1–12 months for adult patients and every 3–6 months for paediatric patients. Patients diagnosed with lung diseases were more likely to receive pharmaceutical therapies and received a wider range of therapies than patients without lung disease. Variation existed between centres in the frequency with which some clinical and laboratory monitoring tests are performed, including exercise tests, laboratory testing for IgG subclass levels and specific antibodies, and lung function tests such as spirometry. Some tests were carried out more frequently in adults than in children, probably due to difficulties conducting these tests in younger children. The percentage of patients seen regularly by a chest physician, or who had microbiology tests performed following chest and sinus exacerbations, also varied widely between centres. Our survey revealed a great deal of variation across Europe in how frequently patients with PID visit the clinic and how frequently some monitoring tests are carried out. These results highlight the urgent need for consensus guidelines on how to monitor lung complications in PID patients.

AB - Many patients with primary immunodeficiency (PID) who have antibody deficiency develop progressive lung disease due to underlying subclinical infection and inflammation. To understand how these patients are monitored we conducted a retrospective survey based on patient records of 13 PID centres across Europe, regarding the care of 1061 adult and 178 paediatric patients with PID on immunoglobulin (Ig) G replacement. The most common diagnosis was common variable immunodeficiency in adults (75%) and hypogammaglobulinaemia in children (39%). The frequency of clinic visits varied both within and between centres: every 1–12 months for adult patients and every 3–6 months for paediatric patients. Patients diagnosed with lung diseases were more likely to receive pharmaceutical therapies and received a wider range of therapies than patients without lung disease. Variation existed between centres in the frequency with which some clinical and laboratory monitoring tests are performed, including exercise tests, laboratory testing for IgG subclass levels and specific antibodies, and lung function tests such as spirometry. Some tests were carried out more frequently in adults than in children, probably due to difficulties conducting these tests in younger children. The percentage of patients seen regularly by a chest physician, or who had microbiology tests performed following chest and sinus exacerbations, also varied widely between centres. Our survey revealed a great deal of variation across Europe in how frequently patients with PID visit the clinic and how frequently some monitoring tests are carried out. These results highlight the urgent need for consensus guidelines on how to monitor lung complications in PID patients.

KW - antibody deficiency

KW - lung disease

KW - monitoring

KW - primary immunodeficiency disease

KW - subclinical infection

UR - http://www.scopus.com/inward/record.url?scp=85028303819&partnerID=8YFLogxK

U2 - 10.1111/cei.13012

DO - 10.1111/cei.13012

M3 - Article

VL - 190

SP - 226

EP - 234

JO - Clinical and Experimental Immunology

JF - Clinical and Experimental Immunology

SN - 0009-9104

IS - 2

ER -