Back to Search
Start Over
Systemic complement activation levels in Stargardt disease
- Source :
- PLoS ONE, PLoS ONE, Vol 16, Iss 6, p e0253716 (2021)
- Publication Year :
- 2021
-
Abstract
- Purpose Preclinical research provides evidence for the complement system as a potential common pathway in Stargardt disease (STGD1) and age-related macular degeneration (AMD) leading to retinal pigment epithelium (RPE) loss. However, systemic complement activation has not yet been assessed in STGD1 patients. We conducted a cross-sectional case-control study to assess systemic complement activation in STGD1 patients and its association with disease severity. Methods Systemic concentrations of complement component C3 and its degradation product C3d were compared between 80 STGD1 patients and 80 controls that were frequency matched for age and sex. The C3d/C3 ratio was used as parameter of systemic complement activation. Within the STGD1 cohort, we additionally examined the association between the C3d/C3 ratio, demographic and behavioural factors (age, sex, smoking and BMI), and measures of disease severity (age at onset, visual acuity, and area of atrophy). Results The C3d/C3 ratio did not significantly differ between patients (mean C3d/C3 ratio 3.5±1.4) and controls (mean C3d/C3 ratio 3.6±1.0), mean difference -0.156 (p = 0.804, independent samples t-test). The overall effect size was 8% (95% confidence interval, 3–15%). Elevated C3d/C3 ratios (>8.1) were found in three patients who all had a concomitant inflammatory condition at the time of blood draw. Within the patient cohort, C3 levels were associated with sex (mean difference -134, p = 0.001, independent samples t-test) and BMI (correlation coefficient 0.463, p Conclusions Systemic complement levels were not elevated in STGD1 patients compared to age and sex matched controls and was not associated with STGD1 severity. Considering the continued absent proof of a systemic contribution of the complement system to RPE loss in STGD1 patients, we hypothesize that complement activation in STGD1 is more likely a local process. In light of upcoming complement-targeted therapies, further studies are needed that measure complement levels in the eye of STGD1 patients.
- Subjects :
- 0301 basic medicine
Male
Visual acuity
Complement Inhibitors
Eye Diseases
Physiology
Complement System
Retinal Pigment Epithelium
Gastroenterology
Biochemistry
Macular Degeneration
0302 clinical medicine
Medical Conditions
Immune Physiology
Medicine and Health Sciences
Stargardt Disease
Geriatric Ophthalmology
Complement Activation
Aged, 80 and over
Sex Characteristics
Multidisciplinary
Immune System Proteins
Retinal Degeneration
Middle Aged
Complement C3d
Cohort
Medicine
Retinal Disorders
Female
medicine.symptom
Anatomy
Research Article
Adult
medicine.medical_specialty
Adolescent
Science
Inflammatory Diseases
Immunology
03 medical and health sciences
Atrophy
Ocular System
Internal medicine
medicine
Genetics
Humans
Aged
business.industry
Biology and Life Sciences
Proteins
Human Genetics
Macular degeneration
medicine.disease
Confidence interval
Complement system
Stargardt disease
Ophthalmology
030104 developmental biology
Cross-Sectional Studies
Geriatrics
Concomitant
Immune System
Macular Disorders
030221 ophthalmology & optometry
Eyes
business
Head
Subjects
Details
- ISSN :
- 19326203
- Volume :
- 16
- Issue :
- 6
- Database :
- OpenAIRE
- Journal :
- PloS one
- Accession number :
- edsair.doi.dedup.....30a9e429092819f7336f7423352f97aa