Systemic Lupus Erythematosus (SLE) is a chronic autoimmune disease that can affect various organ systems in the body. It is characterized by the production of autoantibodies against self-antigens, leading to chronic inflammation and tissue damage including the joints, skin, kidneys, blood cells, brain, heart, and lungs. SLE is a heterogeneous disease with a wide range of clinical manifestations, making it difficult to diagnose and manage.

PATHOPHYSIOLOGY

The pathophysiology of SLE involves a complex interplay between genetic, environmental, hormonal, and immunologic factors. Multiple genetic loci have been associated with SLE, including genes involved in immune system function and regulation. Environmental factors such as infections, medications, and ultraviolet light exposure have also been implicated in the development of SLE.

In SLE, immune dysregulation leads to the production of autoantibodies against nuclear components such as DNA, RNA, and histones. These autoantibodies form immune complexes that deposit in various tissues, leading to chronic inflammation and tissue damage. Additionally, immune dysregulation can lead to aberrant T-cell activation, cytokine production, and complement activation, further contributing to the pathogenesis of SLE.

CAUSES

The exact causes of SLE are not fully understood, but a combination of genetic, environmental, hormonal, and immunologic factors are thought to contribute to its development. Women are more commonly affected than men, and the disease often presents during the childbearing years. Genetic factors are estimated to account for up to 66% of the risk for developing SLE. Environmental factors such as infections, medications, and ultraviolet light exposure have also been implicated in the development of SLE.

CLINICAL MANIFESTATIONS

The clinical manifestations of SLE are diverse and can affect multiple organ systems in the body. Common symptoms include fatigue, fever, joint pain and swelling, skin rashes, and photosensitivity. SLE can also cause more serious complications such as lupus nephritis, which is a major cause of morbidity and mortality in patients with SLE.

ASSESSMENT AND DIAGNOSTIC FINDINGS

The diagnosis of SLE is based on a combination of clinical and laboratory findings. The American College of Rheumatology has developed diagnostic criteria for SLE, which require the presence of at least four of the following: malar rash, discoid rash, photosensitivity, oral ulcers, arthritis, serositis, renal disorder, neurologic disorder, hematologic disorder, immunologic disorder, and antinuclear antibody positivity. Laboratory tests that may be helpful in diagnosing SLE include antinuclear antibody (ANA) testing, anti-double-stranded DNA (anti-dsDNA) antibody testing, and complement-level testing.

MEDICAL MANAGEMENT

The management of SLE involves a multidisciplinary approach, including rheumatologists, nephrologists, dermatologists, and other specialists as needed. Treatment goals include controlling disease activity, preventing flares, and minimizing organ damage. Treatment options may include nonsteroidal anti-inflammatory drugs (NSAIDs), antimalarial drugs, glucocorticoids, immunosuppressants, and biological agents.

PHARMACOLOGIC MANAGEMENT

Pharmacologic management of SLE involves a range of medications targeting different aspects of the disease’s pathophysiology. NSAIDs can be used to manage mild to moderate pain and inflammation, while antimalarial drugs such as hydroxychloroquine can be used to prevent disease flares and reduce disease activity. Glucocorticoids such as prednisone can be used to manage severe disease activity and organ involvement, but their long-term use is associated with significant adverse effects. Immunosuppressive agents such as azathioprine, mycophenolate mofetil, and cyclophosphamide can be used to control disease activity and prevent organ damage. Biologic agents such as belimumab, a monoclonal antibody targeting B-cell activating factor, have also been approved for the treatment of SLE.

Systemic lupus erythematosus diagnosis and management, https://academic.oup.com/rheumatology/article/56/suppl_1/i3/2738661.

C. (2023, January 31). Systemic lupuserythematosus (SLE). Centers for Disease Control and Prevention. https://www.cdc.gov/lupus/facts/detailed.html

Systemic lupus erythematosus pathophysiology – wikidoc. (n.d.). Systemic Lupus Erythematosus Pathophysiology – Wikidoc. https://www.wikidoc.org/index.php/Systemic_lupus_erythematosus_pathophysiology