Clinical Characteristics and Outcomes of Primary Immunodeficiency Diseases Complicated with Autoimmune disorders: Experience from a Single Tertiary Center

Abstract Purpose To investigate the clinical characteristics and outcomes of primary immunodeficiency diseases (PID) patients complicated with autoimmune disorders (AD). Methods Patients with PID who had been admitted to Peking Union Medical College Hospital (PUMCH) from April 1999 to July 2021 were all retrospectively reviewed. We further analyzed the clinical characteristics and long-term outcomes of common variable immunodeficiency (CVID) patients complicated with AD (CVID-AD), and further compared them with CVID patients without AD (CVID-nAD). Results A total of 241 PID patients were identified with common variable immunodeficiency (CVID) accounting for the majority (99, 41.1%). AD was observed in 31.1% (n = 75) of all PID patients. Among 99 CVID patients, 25 (25.3%) were complicated with AD (CVID-AD), while 74 (74.7%) without AD (CVID-nAD). The Comparison between these two groups indicated that bronchiectasis, skin infection, splenomegaly and lymphadenectasis were more common in CVID-AD group than in CVID-nAD group (all p < 0.05). Intravenous immunoglobulin was adopted in 80.0% of CVID-AD patients and 74.3% CVID-nAD patients. Glucocorticoids and immunosuppressant were prescribed to 28.0% and 12.0% CVID-AD patients, respectively. The survival of the CVID-AD group was slightly poorer than that of the CVID-nAD group with the median estimated survival time being 42 months for CVID-AD group and 70 months for CVID-nAD group. Conclusion AD is a common complication in PID patients and can have various manifestations. CVID-AD patients had a slightly poorer prognosis than CVID-nAD patients but with no significant statistical difference.