Bogaert, Anne-Marie

De Scheerder, Marie-Angélique

De Vriendt, Ciel

English

Ghent University Academic Bibliography

 Case history      Pathogenesis A  62 year old female was referred because of persistent elevated inflammatory parameters.  She had been treated for chronic lumbar pain with  a morphine pump, leading to recurrent infections and finally removal of  the pump. Furthermore her clinical history reveals peripheral vascular insufficiency treated with endovascular surgery and stent placement but complicated by a chronic wound on the right foot and recurrent Staphylococci infections (MSSA and MRSA), requiring long courses of antibiotics. She is also known with several allergies towards disinfectants and atopic dermatitis.   Naturally antibodies = without any antigen exposure.  Recognize "oxidation-specific" epitopes, as found in oxidative processes involved in metabolism, aging, and inflammation.  Localised on both pathogens (viral and bacterial) and aging/apoptotic  host cells .  Role in:  •early defense: before more specific antibodies can be produced •housekeeping: removal of cellular debris, homeostasis of inflammation: anti-atherosclerosis, less auto-immunity and allergy  Forgotten immunodeficiencies: a case of  Primary Selective Immunoglobuline M deficiency De Scheerder M-A, M.D., De Vriendt Ciel, M.D.., Bogaert A-M, M.D. General Internal Medicine, Department of Haematology, UZ Gent, Gent ,  Department of Nephrology, Sint-Elisabethziekenhuis, Zottegem; AZ Glorieux, Ronse   Diagnosis A broad laboratory screening revealed increased inflammation parameters (sedimentation 44 mm/u, CRP 24.8mg/l), normal leucocytes count and formula, normal liver and kidney function but a remarkable dysglobulinemia characterized by an IgM of 0.179g/l (normal range 0.46-3), confirmed after repeating the test. IgG and IgA  showed normal values and as expected the IgE values were also elevated (119kU/l) confirming the associated allergic diathesis. Further screening for associated auto-immune disease, acquired  immunodeficiency syndrome (HIV), hematological disorders and lymphocyte analysis revealed no associated abnormalities.    Deficiency in serum IgM may explain a  paradox of diminished responsiveness to   foreign antigens (therefore susceptibility   to infections) and increased inflammation   (because of lack of homeostasis) resulting in   auto-immunity (and possibly malignancy) in   patients with Selective IgM deficiency.   Follow-up and management   Screening for auto-immuune disease, lymphoproliferative disease and allergic diathesis 1x/y  DD between primary and secondary IgM deficiencies   Treatment as suggested after splenectomy (because of the lack of direct  opsonisation).   Low threshold to start antibiotics but no prophylaxis: on demand treatment is recommended in Belgium   No indication for IVIG because of low concentration of IgM and their short half life. References Conclusion - Rothstein TL et al. Annals of the New York Academy of Sciences Volume 1285, Issue 1, pages 97-114, 21 MAY 2013  - L. Temmerman, and E.A.L. Biessen. Cardiovasc Res. 2015;cvr.cvv133 - Grönwall C et ak, Protective rols of natural IgM antibodies. Front.Immun. 2012;3:66 - Louis AG et al. Primary selective IgM deficiency: An ignored immunodifeciency. Clinical reviews in Allergy and Immunology. 2014 Apr;46(2):104-11  Antigen mediated germinal center B2-cell expansion and isotype switching (IgA, IgE, IgG)  Polyreactive, non germinal center B1-cell secretion  of “natural” IgM antibodies   

Recurrent skin and soft tissue infections as a presentation of Primary Selective Immunoglobuline M Deficiency

https://biblio.ugent.be/publication/7055107/file/7055108

Recurrent skin and soft tissue infections as a presentation of Primary Selective Immunoglobuline M Deficiency

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