With greater knowledge of the immune system, we have newer treatments available for primary and secondary immunodeficiencies. All patients with suspected immunodeficiencies need to be aggressively vaccinated according to the Advisory Committee on Immunization Practices guidelines. Immunodeficient patients should also engage in behaviors to avoid infections, such as washing hands frequently, trimming nails to avoid scratching and skin infections and, if applicable, performing nasal saline irrigation at least twice daily.
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For those with antibody deficiencies, immunoglobulin replacement, either through intravenous or subcutaneous routes, may be a viable option. When dosed appropriately and monitored for side effects by a trained specialist familiar with immunoglobulin replacement, this may lead to improved quality of life.5 Alternatively, prophylactically rotating antibiotics may be helpful in preventing infections, although it may not affect other effects of immunodeficiency.
Lastly, and most intriguingly, for certain primary immunodeficiencies, like LRBA deficiency, novel therapeutic agents, such as abatacept, have shown promise. The greater adoption of genetic testing will potentially open the doors to novel treatment modalities based on the principles of precision medicine.6 Therefore, rheumatologists and their patients will find great value in identifying immunodeficiencies.
As we continue to traverse the oceans of immunology, it becomes increasingly vital for rheumatologists to recognize immunodeficiencies as an underlying cause and/or a consequence of systemic autoimmunity. Once identified, these can be addressed by specialists who have familiarity with immune deficiencies, leading to improvements in the quality of life for patients.
Bharat Kumar, MD, MME, FACP, RhMSUS, is the associate program director of the rheumatology fellowship training program at the University of Iowa in Iowa City. Follow him on Twitter @BharatKumarMD.
- Xiao X, Miao Q, Chang C, et al. Common variable immunodeficiency and autoimmunity—an inconvenient truth. Autoimmun Rev. 2014 Aug;13(8):858–864.
- Peter HH. Adult-onset immunodeficiency—Why is it important in rheumatology? Arthritis Res Ther. 2013 Jan 30;15(1):105.
- Goyal R, Bulua AC, Nikolov NP, et al. Rheumatologic and autoimmune manifestations of primary immunodeficiency disorders. Curr Opin Rheumatol. 2009 Jan;21(1):78–84.
- Subesinghe S, Rutherford AI, Byng-Maddick R, et al. Recurrent serious infections in patients with rheumatoid arthritis-results from the British Society for Rheumatology Biologics Register. Rheumatology (Oxford). 2018 Apr 1;57(4):651–655.
- Rider NL, Kutac C, Hajjar J, et al. Health-related quality of life in adult patients with common variable immunodeficiency disorders and impact of treatment. J Clin Immunol. 2017 Jul;37(5):461–475.
- Leiding JW, Ballow M. Precision medicine in the treatment of primary immunodeficiency diseases. Curr Opin Allergy Clin Immunol. 2018 Apr;18(2):159–166.