Epidemiology, Signs and Symptoms of Systemic Lupus Erythematosus

There are numerous diseases, and infections that affect humans, while we are conversant with so many common diseases, infections, and deficiencies, some of them are very scarce that we might only hear or see them rarely. Today, I will be discussing Systemic Lupus Erythematosus but for the purpose of this post, I will be referring to it as Lupus, since it is the most common type of Lupus.

Systemic Lupus Erythematosus is a chronic autoimmune condition involving multi-system inflammation as a result of the immune system attacking its own tissues and organs, damaging them. Systemic Lupus Erythematosus can affect the blood vessel, joints, brain, skin, and lungs. The Etiology of Lupus is unknown, so we cannot say if it is caused by a pathogen or an infection. Systemic Lupus Erythematosus involves the production of autoantibodies causing immune complexes which are deposited in different areas of the body. Autoantibodies are antibodies produced by the body to fight against self-antigens or its own tissue. They bind to a certain component of the patient's body creating immune complex formation in those parts of the body. In simple words, Lupus is when the immune system which should protect the body from infection and diseases starts to attack its own tissues.

Image Credit

Epidemiologically, Lupus is more likely to affect females than males, at a ratio of 9 to 1. It is common among females of childbearing age (between the age of 14 - 40), and while is it rare in men, it is very severe in men. Risk factors for Systemic Lupus Erythematosus include family history, genetics (including monozygotic twins, Epigenetics), environmental factors (UV light)and hormonal factors (oestrogen), Ebstein Barr virus, and smoking. Systemic Lupus Erythematosus damages the cell protein and DNA in the cell, which could lead to organ injury. when cells are damaged, they repair themselves, but when they cannot repair themselves, they undergo apoptosis (programmed death) where the internal proteins of the cell kill the cell. This is important in preventing cancer. When the cells are destroyed, macrophages phagocytose the dead cells. When these cells are not properly engulfed, they could trigger the immune system to attack the protein and nuclei material.

Patients suffering from Systemic Lupus Erythematosus could have a deficiency in component proteins C1, C2, C3, and C4 which are proteins that help macrophages engulf apoptotic cells. When the immune system becomes sensitized to these materials, they start to mount an immune response to the materials, and antibodies start to target histones, chromatin, and dsDNA of the apoptotic cells at different parts of the body. The antigens are displayed to T-helper cells in nearby lymph nodes where they produce T-Helper 2 cells which then produce auto-antibodies to target nuclear proteins from the dead cell, but since they are found in every other cell, they also attack healthy cells assuming them as an antigen, causing inflammation to the tissues and cells affected thereby damaging the cells and tissues more and on a continuous process. This is made possible because the body creates memory B-cells which helps the cells to identify antigens and act on them next time.

Signs and symptoms of Systemic Lupus Erythematosus include Malar Rash (butterfly rash) is a rash that affects the face, sparing the Nasolabial folds of the face. Discoid Rash (a disk-shaped rash as the name implies), Alopecia (patchy hair loss which is often caused by discoid lesions), Bittle hair in regions where alopecia isn't functional, *Photosensitivity (sun sensitivity), Ulcerations (basically in the mouth, nose or gum), ulcerative keratitis, Polyarthritis (multiple joint inflammations) causing pains in multiple joints basically symmetrical in distribution, also, with lupus both joints are affected. Cognitive impairment, headaches, seizures, intestinal fibrosis, pulmonary vasculitis, hypertension (associated with lupus nephritis), hepatomegaly, abdominal serositis, anaemia, leukopenia, polyarthralgia, arthritis, Patients could experience Raynaud's phenomenon (cynosis of the fingers), and osteoporosis.

Patients with Lupus could suffer from serositis including pleurisy or pericarditis (inflammation of the pericardium), Endocarditis (inflammation of the endocardium of the heart) and Endocarditis common in lupus is the Libman-Snack Endocarditis, Pleuritis (the inflammation of the lung pleura) and about 50% of patients suffering from lung diseases, Cerebritis (Inflammation of the cerebrum). Patients with Systemic Lupus Erythematosus would also suffer from alveolitis, alveolar wall injury, oedema, haemorrhage, and intestinal pneumonitis. Systemic Lupus Erythematosus patients could suffer from Lymphadenopathy. Tests will reveal hyperplasia with giant cells, and plasma cells. They could also suffer from Lupus pneumonitis. about 10% of patients with Systemic Lupus Erythematosus suffer from Lupus Pneumonitis.

Diagnosis and Investigation for Systemic Lupus Erythematosus will be a a full blood count, Urine tests (Urinalysis, Urine Microscopy, and Urinary Albumin Creatinine ratio), antinuclear antibodies (ana) test (Anti ds DNA, ANti Histone, etc), extractable nuclear antigens (ENA) test, and Anti cardiolipin test.

Systemic Lupus Erythematosus currently has no treatment, as a definite cause cannot be identified, but there have been numerous ways to manage it. Management involves avoiding UV light exposure, Reduce Cardiovascular disease, use anti-inflammatory medications against joint pain, steroid creams for rashes, and other treatments to fight diseases arising from immunocompromisation.

Conclusion
Lupus currently has no cure, and while it is quite a rare disease, it is common in women, and patients who have Lupus have to manage the disease as it currently has no cure.