Management of these patients has varied based on clinical manifestations.<h4>Conclusion</h4><i>UNC93B1-</i>mutation<i>-</i>associated disease should be considered in the context of early-onset autoimmune disease, especially childhood-onset SLE, juvenile arthritis, and rheumatoid arthritis. The gene discussed is UNC93B1; the disease is systemic lupus erythematosus.