Complement Receptor Deficiency Treatment & Management

Updated: Apr 13, 2021
  • Author: Robert A Schwartz, MD, MPH; Chief Editor: Dirk M Elston, MD  more...
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Treatment

Medical Care

No specific treatment is required for complement receptor deficiencies, other than symptomatic treatment for associated autoimmune disorders or recurrent infections.

Systemic steroids and steroid-sparing agents may be required for the treatment of deficiencies associated with immune disorders. Prophylactic and therapeutic antibiotics may be prescribed as needed. The value of avacopan, an oral C5a receptor inhibitor, in patients with ANCA-associated vasculitis is being accessed. [21]

Bone marrow transplantation may be of help in severe cases of leukocyte adhesion deficiency syndrome. Hematopoietic stem-cell transplantation is of long-term benefit and an early therapeutic option with a suitable HLA-matched stem cell. [22] Another approach is the use of ustekinumab, an antibody that binds the p40 subunit of interleukin 23 and interleukin 12. [23]

Hospitalization may be required in cases associated with severe autoimmune disorders and systemic involvement, as well as in cases with severe infections.

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Consultations

In patients with severe SLE or other autoimmune disorders, referral to a rheumatologist or an internist may be advised.

In patients with leukocyte adhesion deficiency syndrome, a pediatrician and an infectious diseases specialist is needed in the neonatal period.

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Long-Term Monitoring

Patients need to be monitored on a regular basis.

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