Systemic lupus erythematosus and immunodeficiency

Immunol Med. 2019 Mar;42(1):1-9. doi: 10.1080/25785826.2019.1628466. Epub 2019 Jun 17.

Abstract

Systemic lupus erythematosus (SLE) is a prototypic systemic autoimmune disease that develops in genetically susceptible individuals in response to environmental factors. SLE and primary immunodeficiency disease (PID) share some clinical manifestations in that certain PIDs present with autoimmune phenomena. Patients with SLE become susceptible to infection via three pathways. First, SLE and PID share some genetic factors, such as complement and mannose-binding lectin genes, which predispose patients to infection. Second, patients with SLE have an inherently high risk of infection because of their intrinsic immunological abnormalities induced by SLE. Third, patients with SLE receiving immunosuppressive treatment are at high risk of infection. Further studies delineating the abnormalities related to both autoimmunity and immunodeficiency would be warranted to identify a new potential drug target for SLE.

Keywords: Systemic lupus erythematosus; immunodeficiency; infection.

Publication types

  • Review

MeSH terms

  • Autoimmunity
  • Complement System Proteins / immunology
  • Disease Susceptibility
  • Genetic Predisposition to Disease
  • Humans
  • Immunologic Deficiency Syndromes / genetics
  • Immunologic Deficiency Syndromes / immunology*
  • Immunosuppressive Agents / adverse effects
  • Infections / etiology*
  • Lupus Erythematosus, Systemic / drug therapy
  • Lupus Erythematosus, Systemic / genetics
  • Lupus Erythematosus, Systemic / immunology*
  • Mannose-Binding Lectin / genetics
  • Molecular Targeted Therapy
  • Risk

Substances

  • Immunosuppressive Agents
  • Mannose-Binding Lectin
  • Complement System Proteins