DESTIN, Fla. — Nephritis is less prevalent among patients with both systemic lupus erythematosus and common variable immunodeficiency disease than in other lupus cohorts, while neuropsychiatric and other autoimmune complications may be more common, according to a presenter at the 2019 North American Young Rheumatology Investigator Forum.
“We know that common variable immune deficiency is a disease impacting B cells, in which there is a defect in producing immunoglobin isotypes that are necessary for effective primary and secondary antibody response to microbial pathogens,” Hamdy Ahmed, MD, a clinical rheumatology fellow at the University of Alabama Birmingham, told attendees. “We also know that, in SLE, B cells producing self-reactive autoantibodies are persistently activated.”
To analyze patients with both SLE and common variable immunodeficiency, as well as their effects on clinical and treatment implications, Ahmed and colleagues conducted a search of electronic medical records to identify individuals seen in adult lupus and immunology clinics with both diseases from 20142018. Of the 82 patient charts reviewed using i2b2 software with an identifying algorithm, 43 met the inclusion criteria of both SLE and confirmed common variable immunodeficiency, defined as hypogammaglobulinemia with failure to respond appropriately to pneumococcal immunization.
All 43 included patients were women, with 42 who were Caucasian, including one Hispanic patient, and one who was African American.
According to the researchers, 40% of the included patients reported connective tissue conditions including SLE, rheumatoid arthritis, inflammatory arthritis not specified and antiphospholipid syndrome. Other autoimmune conditions reported in 13 patients included autoimmune thyroiditis, one case of Crohn’s disease and eight cases of unspecified diseases. In addition, family history of immunodeficiency syndromes was reported in four patients but were not specified. During follow up, antinuclear antibodies were rechecked in 34 patients, with the antibodies disappearing in 62% of included patients and persisting in 35%.
Predominant SLE clinical features included mucocutaneous, arthritis and immune cytopenias. Notably, lupus nephritis was uncommon in this cohort. Malar rash, serositis and renal involvement were also less common in the in patients with SLE and common variable immunodeficiency, compared with another Caucasian SLE cohort from the same regional area. Belimumab and rituximab were used more frequently among patients with both SLE and common variable immunodeficiency, compared with other immunosuppressant drugs, with overall favorable outcomes.
“What we took from this study is that SLE patients with common variable immunodeficiency disease may behave differently than lupus patients, and that if may be due to the common variable immunodeficiency disease,” Ahmed said. “Rheumatologists should be aware of this coexistence, because it could change the way that you treat these patients.” – by Jason Laday
Ahmed H. Systematic lupus erythematosus patients with common variable immunodeficiency disease. Presented at: North American Young Rheumatology Investigator Forum; May 1, 2019; Destin, Fla.
Disclosure: Ahmed reports no relevant financial disclosures.