Systemic Lupus Erythematosus and Deficiencies of Early Components of the Complement Classical Pathway

Systemic Lupus Erythematosus and Deficiencies of Early Components of the Complement Classical Pathway

The complement system plays an important role in the innate and acquired immune response against pathogens. It consists of more than 30 proteins found in soluble form or attached to cell membranes. Most complement proteins circulate in inactive forms and can be sequentially activated by the classical, alternative, or lectin pathways. Biological functions, such as opsonization, removal of apopto...

Complete complement components C4A and C4B deficiencies in human kidney diseases and systemic lupus erythematosus.

Although a heterozygous deficiency of either complement component C4A or C4B is common, and each has a frequency of approximately 20% in a Caucasian population, complete deficiencies of both C4A and C4B proteins are extremely rare. In this paper the clinical courses for seven complete C4 deficiency patients are described in detail, and the molecular defects for complete C4 deficiencies are eluc...

Complement and systemic lupus erythematosus

Complement is implicated in the pathogenesis of systemic lupus erythematosus (SLE) in several ways and may act as both friend and foe. Homozygous deficiency of any of the proteins of the classical pathway is causally associated with susceptibility to the development of SLE, especially deficiency of the earliest proteins of the activation pathway. However, complement is also implicated in the ef...

Inherited Classical and Alternative Pathway Complement Deficiencies in Children: A Single Center Experience

Neurobrucellosis in systemic lupus erythematosus

Background: Brucellosis is a zoonotic infection which is endemic in many countries. It is a multisystem disease which may present with a broad spectrum of clinical manifestations and complications. Neurobrucellosis is an uncommon complication of brucellosis. Case presentation: A 25-year-old woman with a history of lupus for 5 months referred to the emergency ward of Shahid Beheshti Hosp...