Suspecting Immunodeficiency - Health Education England
Suspecting Immunodeficiency David Lowe Royal Free Hospital / University College London Or Suspecting primary immunodeficiency AND
Suspecting secondary hypogammaglobulinemia Primary immunodeficiency (in adults) Antibodies / B cells (XLA) CVID IgA deficiency
(+/- Specific Ab def, IgG subclass def etc) T cells Mild phenotype SCID Idiopathic CD4 lymphopenia Phagocytes CGD Complement
A few other rare ones Dissecting Ab deficiency Hyper IgM syndrome
-Reg -Reg X CTLA4 def
IPEX Connecting Ab deficiency with autoimmunity Antibodies (not auto-!) Opsonise micro-organisms Improve phagocytosis
Improve recognition by other immune cells Activate immune responses & complement Infectious consequences of Antibody deficiency Sino-pulmonary infection URTI LRTI bacteria and viruses
Sinusitis Ear infections Can end up with bronchiectasis / complications thereof GI infections, including Giardia Sepsis, meningitis, osteomyelitis Not especially skin & urine infections Prolonged / severe infection with normal pathogens, eg norovirus
Arthritis Lupus-like features Cunningham-Rundles. Hematology Am Soc Hematol Educ Program, 2012:301-305 28.6% of cohort had autoimmunity Autoimmunity in IgA deficiency
20 30% have autoimmune disease SLE, Graves disease, T1DM, (juvenile) RA ?Myaesthenia, ?IBD Coeliac disease approx. 8% Autoantibodies very common (up to 90%) May be more clinically important than sino-pulmonary infections (bronchiectasis rare) & Giardia
A few pictures. So check the immunoglobulins! A couple of specific disorders
T cell Mr T cell Secondary hypogammaglobulinemia Medication
B-CLL (+ other lymphoproliferative diseases) Protein loss Table 2. Likely cause of secondary antibody deficiency in each subject. Duraisingham SS, Buckland M, Dempster J, Lorenzo L, Grigoriadou S, et al. (2014) Primary vs. Secondary Antibody Deficiency: Clinical Features and Infection Outcomes of Immunoglobulin Replacement. PLoS ONE 9(6): e100324.
doi:10.1371/journal.pone.0100324 http://127.0.0.1:8081/plosone/article?id=info:doi/10.1371/journal.pone.0100324 Hypogammaglobulinaemia after rituximab treatment-incidence and outcomes. Makatsori M et al. QJM 2014; 107:821-8. So check the
immunoglobulins! (before and after Rx) A couple of pitfalls with IVIG N = 80 N = 17
Are these results for: A: Troponin B: Hepatitis B core Antibody C: Attitude to treating Immunologist Serology is useless
on IVIG! Galactomannan 3 2 1 0
0.6 GM EIA INDEX GM EIA INDEX
4 0.4 0.2 0.0
CGD Consequences of CGD Bacterial infections, esp with catalase-positive organisms (eg Staph aureus, Burkholderia cepacia, Serratia marcescens)
A few more pictures. Chronic granulomatous disease carrier geno-dermatosis (CGDCGD), (Finlay et al, Clinical Genetics 1983; 23:276280.)
Key messages Have a low threshold for checking immunoglobulins Remember the secondary causes of hypogammaglobulinemia Dont do serology if people are on IVIG! Look for the clues of immunodeficiency THANKS!
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