While relatively rare, individuals with a homozygous deficiency in complement C1 activity have been identified, and 90% of them developed a lupus-like illness at a very young age. These individuals had significant kidney damage and damage to blood vessels in the central nervous system, both of which were associated with severe inflammation. A reasonable hypothesis to explain the development of the lupus-like disease in these patients is:

Question

While relatively rare, individuals with a homozygous deficiency in complement C1 activity have been identified, and >90% of them developed a lupus-like illness at a very young age. These individuals had significant kidney damage and damage to blood vessels in the central nervous system, both of which were associated with severe inflammation. A reasonable hypothesis to explain the development of the lupus-like disease in these patients is:

Quizplus · Accepted Answer

A deficiency in complement C1 impairs the clearance of apoptotic cells, leading to the accumulation of cellular debris and potential autoantigen exposure, which can trigger autoimmune responses similar to lupus.

While Relatively Rare, Individuals with a Homozygous Deficiency in Complement