Abstract
BackgroundAcquired complement deficiency can occur in the setting of autoimmune syndromes, such as systemic lupus erythematosus (SLE), with very low or, occasionally, undetectable C3 levels. Based on inherited complement defects, patients with transiently low complement may be at similar risk for serious bacterial infection, but the degree of risk related to C3 level and temporal association is unknown.MethodsWe performed a retrospective study including pediatric patients with undetectable total complement activity or absent individual complement components measured at our institution from 2002 to 2018. We assessed annual rate of serious bacterial infection (SBI) defined as requiring hospitalization and/or parenteral antibiotics by manual chart review. Among included SLE patients, we assessed the 30-day probability of SBI for given C3 measurements using a logistic regression model to determine risk. Primary complement deficiency was analyzed for SBI rate as comparison. Covariates included age, level of immune suppression and history of lupus nephritis.ResultsAcquired complement deficiency secondary to SLE-related disease [n = 44] was the most common underlying diagnosis associated with depressed complement levels and were compared to a cohort of primary complement deficient patients [n = 18]. SBI per 100 person-years and cohort demographics were described in parallel. Our logistic regression analysis of pediatric patients with SLE showed low C3 level was temporally associated with having an SBI event. Given equivalent immunosuppression, patients with an SBI had lower C3 levels at the beginning of the observation period relative to patients without SBI.ConclusionPediatric patients with the diagnosis of SLE can develop very low C3 levels that associate with risk of serious bacterial infection comparable to that of patients with primary complement deficiency. Patients prone to severe complement consumption may particularly be at risk.
Highlights
Acquired complement deficiency can occur in the setting of autoimmune syndromes, such as systemic lupus erythematosus (SLE), with very low or, occasionally, undetectable C3 levels
We report serious bacterial infections (SBI) among patients with SLE/connective tissue disease (CTD)-related disorders with a history of complement consumption, including detailed clinical history to account for kidney disease, immune suppression and types of infection, compared to that of an established cohort of primary complement deficient patients
Study participants We performed an unbiased review of all complement levels measured at our hospital from 2002 to 2018 (n = 5876, from 1643 total patients)
Summary
Acquired complement deficiency can occur in the setting of autoimmune syndromes, such as systemic lupus erythematosus (SLE), with very low or, occasionally, undetectable C3 levels. Based on inherited complement defects, patients with transiently low complement may be at similar risk for serious bacterial infection, but the degree of risk related to C3 level and temporal association is unknown. Secondary complement defects from an acquired disease process, such as systemic lupus erythematosus (SLE) or connective tissue disease (CTD), are usually partial and transient from immune complex formation and deposition. We report serious bacterial infections (SBI) among patients with SLE/CTD-related disorders with a history of complement consumption, including detailed clinical history to account for kidney disease, immune suppression and types of infection, compared to that of an established cohort of primary complement deficient patients
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