Bacterial infections in a pediatric cohort of primary and acquired complement deficiencies
Abstract Background 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 r...
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| Format: | Article |
| Language: | English |
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BMC
2020-09-01
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| Series: | Pediatric Rheumatology Online Journal |
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| Online Access: | http://link.springer.com/article/10.1186/s12969-020-00467-0 |
| _version_ | 1819060703465570304 |
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| author | Taha Al-Shaikhly Kristen Hayward Matthew L. Basiaga Eric J. Allenspach |
| author_facet | Taha Al-Shaikhly Kristen Hayward Matthew L. Basiaga Eric J. Allenspach |
| author_sort | Taha Al-Shaikhly |
| collection | DOAJ |
| description | Abstract Background 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. Methods We 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. Results Acquired 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. Conclusion Pediatric 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. |
| first_indexed | 2024-12-21T14:31:12Z |
| format | Article |
| id | doaj.art-68fcafb0c5ae4393bd489af2f002bf0d |
| institution | Directory Open Access Journal |
| issn | 1546-0096 |
| language | English |
| last_indexed | 2024-12-21T14:31:12Z |
| publishDate | 2020-09-01 |
| publisher | BMC |
| record_format | Article |
| series | Pediatric Rheumatology Online Journal |
| spelling | doaj.art-68fcafb0c5ae4393bd489af2f002bf0d2022-12-21T19:00:30ZengBMCPediatric Rheumatology Online Journal1546-00962020-09-011811610.1186/s12969-020-00467-0Bacterial infections in a pediatric cohort of primary and acquired complement deficienciesTaha Al-Shaikhly0Kristen Hayward1Matthew L. Basiaga2Eric J. Allenspach3Division of Pulmonary, Allergy & Critical Care Medicine, Department of Medicine, Penn State College of MedicineDivision of Rheumatology, Department of Pediatrics, University of WashingtonDivision of Pediatric Rheumatology, Department of Pediatric and Adolescent Medicine, Mayo ClinicDivision of Rheumatology, Department of Pediatrics, University of WashingtonAbstract Background 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. Methods We 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. Results Acquired 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. Conclusion Pediatric 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.http://link.springer.com/article/10.1186/s12969-020-00467-0Systemic lupus ErythematosusSLEConnective tissue diseaseComplement |
| spellingShingle | Taha Al-Shaikhly Kristen Hayward Matthew L. Basiaga Eric J. Allenspach Bacterial infections in a pediatric cohort of primary and acquired complement deficiencies Pediatric Rheumatology Online Journal Systemic lupus Erythematosus SLE Connective tissue disease Complement |
| title | Bacterial infections in a pediatric cohort of primary and acquired complement deficiencies |
| title_full | Bacterial infections in a pediatric cohort of primary and acquired complement deficiencies |
| title_fullStr | Bacterial infections in a pediatric cohort of primary and acquired complement deficiencies |
| title_full_unstemmed | Bacterial infections in a pediatric cohort of primary and acquired complement deficiencies |
| title_short | Bacterial infections in a pediatric cohort of primary and acquired complement deficiencies |
| title_sort | bacterial infections in a pediatric cohort of primary and acquired complement deficiencies |
| topic | Systemic lupus Erythematosus SLE Connective tissue disease Complement |
| url | http://link.springer.com/article/10.1186/s12969-020-00467-0 |
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