Complicating matters is that qualitative deficits may be the primary drivers of many immunodeficiencies. In specific antibody deficiency, for example, the levels of immunoglobulins are within normal limits, but antibody titers demonstrate poor response to antigens. Conversely, patients may have low CD4 helper T cell counts but no appreciable qualitative defects causing clinical symptoms. I manage these patients conservatively, with the understanding that lab values should not dictate the quality of care.
Although I welcome all the business that comes to my clinic with abnormal lab values, this is a decidedly unwelcome hassle for patients and expenditure for the healthcare system at large. I would strongly encourage rheumatologists and others to focus on taking detailed histories and documenting infections, rather than ordering panels of screening labs beyond the widely available, cheap and meaningful IgG, IgA and IgM levels, as well as the complete blood count with differential.
Misconception #5
Treatment for immunodeficiencies are limited and ineffective.
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.
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.
Conclusion
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.
