Systemic lupus erythematosus

How SLE causes thrombosis

Endothelial cells are tissue cells which form the lining of blood vessels and limit clot formation, providing a non-thrombotic surface under normal physiological conditions. When autoantibodies attack the endothelial cells, a sequence of coagulation reactions occur.

In patients with severe SLE, the reduction of NETs (neutrophil extracellular traps) is impaired, and NETs therefore permanently activate the complement system. The complement system is an enzyme cascade that helps defend against infection.

Neutrophils are immune system cells which circulate in blood vessels and are enrolled to an inflammation site when the body responds to microbial infections. One of the processes of neutrophil action is the formation of NETs. NETs are formed upon contact with various bacteria, fungi, activated platelets, or under the effect of several inflammatory stimuli.

Causes and risk factors

In the majority of cases, the cause is unknown. It may be a combination of genetics and environment. People which have inherited a predisposition for the disease may develop SLE once activated by one of the potential triggers – sunlight, infections, or some medications (for example anti-seizure medications and antibiotics).

Lupus can affect all ages however it is mostly diagnosed between ages 15-55. It is more common in women than men, and among African and Asian Americans, and Hispanics.

Signs and symptoms

Lupus can present with general signs and symptoms, making diagnosis difficult. As this condition is a multisystem disease, it can involve different organs, therefore signs and symptoms will depend on which organ or body system is affected.

  • Facial rash which looks like butterfly wings across both cheeks – this is the most unique sign which occurs in most cases.
  • Rashes can occur on other parts of the body.
  • Fatigue.
  • Fever.
  • Joint pain, stiffness and swelling.
  • Skin lesions that appear or exacerbate with sun exposure.
  • Fingers and toes turn white or blue – due to cold exposure or stress.
  • Shortness of breath.
  • Chest pain.
  • Dry eyes.
  • Headaches, confusion and memory loss.

References