Clinical Manifestations
Most prevalent in child cases, the presence of visible blood in the urine, causing pink discoloration
Macroscopic hematuria
Failure to regulate the alternative pathway of the complement system. This causes the system to attack the body's own cells (glomeruli)
Alternative Pathway Dysregulation
Tests for nephritic factors that drive the disease by keeping the immune system turned "ON"
Autoantibody Screening
Medicines that target the complement system
Complement Inhibitors
This age group is the most commonly affected by this disorder
Young adults
A rare manifestation that involves the uneven fat loss, most prevalent in the face or upper body
Acquired Partial Lipodystrophy (APL)
Hallmark manifestation involves prominent deposition of complement component C3, with absent or low immunoglobulin, on a renal biopsy
C3 Deposition
Used to establish between the two subtypes of C3 (complement protein), more dense and less dense, via structure differences
Electron Microscopy
Treatment that filters waste, toxins, and excess fluid from the blood
Dialysis
This process refers to the filtration of blood in the kidneys
Glomerular Filtration
Build-up of complement proteins in the eyes, which may cause impaired vision
Ocular Drusen
A rare change in fat distribution, particularly in the face and upper body
Lipodystrophy
Testing for low C3 in the urine via a urine or blood test
Hypocomplementura
A process that filters blood to remove harmful antibodies by separating plasma from blood
Plasma Exchange
This is the functional unit of the kidney that is responsible for filtrations
Nephron
Increased serum creatinine, reduced estimated glomerular filtration rate (eGFR), and decreased urine
Accumulation of C3 in the glomerular basement membrane triggers damage
Glomerular Inflammation and Scarring
Testing for functional abnormalities in complement system proteins, and test for underlying causative genetic mutations.
Complement System evaluation
Medications that help control blood pressure and reduce protein loss
ACE Inhibitors
This organ is responsible for filtering blood to remove waste and excess
Kidney
Presence of excess protein in the urine, giving it a foamy or bubbly appearance
Proteinuria
Disruption of the alternative pathway of activation due to the presence of autoantibodies - antibodies that recognize the host's own tissues
Complement Dysfunction
Dominant C3 staining is visible in immunofluorescence microscopy, giving a definitive diagnosis
Kidney Biopsy
These drugs suppress the immune system and stop it from attacking the kidney
Immunosuppressants
This is the primary organ system that is affected by this disorder
Urinary System