Which of the following is not due to complement deficiency?
**Core Concept**
Complement deficiency refers to a condition where there is a lack or malfunction of the complement system, a group of proteins that play a crucial role in the immune system. The complement system helps to eliminate pathogens and damaged cells from the body. Deficiencies in the complement system can lead to increased susceptibility to infections and autoimmune diseases.
**Why the Correct Answer is Right**
Paroxysmal nocturnal hemoglobinuria (PNH) is a rare disease characterized by the destruction of red blood cells, bone marrow failure, and the presence of blood clots. However, PNH is not caused by a deficiency in the complement system. Instead, it is caused by a mutation in the PIGA gene, which leads to the absence of the glycosylphosphatidylinositol (GPI) anchor. This mutation prevents the complement system from binding to the surface of red blood cells, leading to their destruction. PNH is not an autoimmune disease, and it does not result from a deficiency in the complement system.
**Why Each Wrong Option is Incorrect**
**Option A:** Systemic lupus erythematosus (SLE) is an autoimmune disease that can result from a deficiency in the complement system, specifically the C1q and C4 components. These deficiencies can lead to the development of SLE, making this option incorrect.
**Option C:** Glomerulonephritis can result from an immune complex-mediated response, which involves the complement system. Deficiencies in the complement system can lead to impaired clearance of immune complexes, resulting in glomerulonephritis. This option is incorrect because it is related to complement system function.
**Option D:** Neisseria infections, such as meningitis and septicemia, can be more severe and frequent in individuals with deficiencies in the complement system, specifically the C5-C9 components. These deficiencies can lead to impaired opsonization and killing of Neisseria bacteria, making this option incorrect.
**Clinical Pearl / High-Yield Fact**
A deficiency in the complement system can lead to increased susceptibility to Neisseria infections, particularly meningitis and septicemia. This is because the complement system plays a crucial role in opsonizing and killing these bacteria.
**Correct Answer:**
β Correct Answer: B. PNH