Lupus Erythematosus
Lupus erythematosus is a chronic, multisystem, autoimmune disease characterized by the production of autoantibodies and immune complexes that result in widespread inflammation and tissue damage. The pathogenesis involves a complex interplay between genetic predisposition, environmental triggers, hormonal influences, and immune dysregulation, leading to loss of self-tolerance and the generation of pathogenic autoantibodies, particularly against nuclear antigens. These autoantibodies promote immune complex deposition in various organs, including the skin, joints, kidneys, heart, and central nervous system, resulting in diverse clinical manifestations. The health impacts of lupus erythematosus are profound and can range from mild cutaneous involvement to severe, life-threatening organ dysfunction. The disease course is variable, often marked by periods of remission and exacerbation, with significant morbidity associated with organ damage, treatment side effects, and increased risk of infections and cardiovascular complications.
Systemic lupus erythematosus is the most prevalent and clinically significant form of lupus, characterized by the involvement of multiple organ systems. SLE presents with a broad spectrum of manifestations, including constitutional symptoms such as fatigue and fever, cutaneous findings like malar rash and photosensitivity, musculoskeletal complaints such as arthritis and arthralgia, and involvement of vital organs including the kidneys (lupus nephritis), heart, lungs, and central nervous system. The disease course is marked by relapses and remissions, and the severity can range from mild to life-threatening. Immunologically, SLE is distinguished by the presence of a variety of autoantibodies, including anti-dsDNA, anti-Sm, and antiphospholipid antibodies.
Cutaneous lupus erythematosus encompasses a group of lupus variants that primarily affect the skin. CLE is further subdivided into acute, subacute, and chronic forms. Acute CLE often presents as a malar or 'butterfly' rash, while subacute CLE is characterized by annular or psoriasiform lesions with minimal scarring. Chronic CLE, the most common of which is discoid lupus erythematosus (DLE), features well-demarcated, scaly plaques that may lead to scarring and pigmentary changes. Although cutaneous manifestations may occur in isolation, they can also be a harbinger of systemic involvement.
Drug-induced lupus erythematosus is a lupus-like syndrome triggered by prolonged exposure to certain medications. DILE typically presents with symptoms similar to SLE, such as arthralgia, myalgia, fever, and serositis, but usually lacks major organ involvement like nephritis or central nervous system disease. The syndrome is associated with the presence of antihistone antibodies and generally resolves upon discontinuation of the offending drug.
Neonatal lupus erythematosus is a rare condition affecting neonates born to mothers with specific autoantibodies, most commonly anti-Ro/SSA and anti-La/SSB. Clinical manifestations include transient cutaneous lesions, congenital heart block, hepatobiliary disease, and hematologic abnormalities. While most symptoms resolve spontaneously as maternal antibodies are cleared, congenital heart block may be permanent and carries significant morbidity.
Lupus erythematosus, particularly systemic lupus erythematosus, exhibits marked epidemiological variability based on age, gender, ethnicity, and geographic location. The global prevalence of SLE ranges from approximately 20 to 150 cases per 100,000 individuals, with higher rates reported in certain populations, such as African Americans, Hispanics, and Asians. The disease predominantly affects women, with a female-to-male ratio of approximately 9:1, especially during the reproductive years, highlighting the role of hormonal influences in disease susceptibility. The peak age of onset is typically between 15 and 45 years. Although the incidence of SLE is lower in children and the elderly, these age groups may experience more severe disease courses. Mortality rates have improved over the past decades due to advances in diagnosis and management, but patients with lupus erythematosus still face increased risks of premature death, particularly from infections, cardiovascular disease, and end-stage renal disease.
The diagnosis of lupus erythematosus is based on a combination of clinical evaluation, laboratory investigations, and the application of established classification criteria. The most widely used criteria are the 2019 European League Against Rheumatism/American College of Rheumatology (EULAR/ACR) classification criteria for systemic lupus erythematosus, which require a positive antinuclear antibody (ANA) test as an entry criterion, followed by the accumulation of weighted clinical and immunological features across multiple organ systems. Key laboratory findings include elevated ANA titers, the presence of specific autoantibodies such as anti-dsDNA, anti-Sm, anti-Ro/SSA, and antiphospholipid antibodies, as well as evidence of complement consumption (low C3 and C4). Additional investigations may include complete blood counts, renal and liver function tests, urinalysis, and imaging studies as indicated by organ involvement. Skin or renal biopsies may be performed to confirm the diagnosis and assess disease activity or damage. Differential diagnosis is essential to exclude other autoimmune, infectious, or drug-induced conditions that can mimic lupus erythematosus. Early and accurate diagnosis is crucial for optimal management and prevention of irreversible organ damage.
Hydroxychloroquine sulfate is an antimalarial agent widely utilized in the management of lupus erythematosus for its immunomodulatory effects, particularly in controlling cutaneous and musculoskeletal symptoms and reducing disease flares. Belimumab, a monoclonal antibody targeting B-lymphocyte stimulator (BLyS), is indicated for patients with active, autoantibody-positive systemic lupus erythematosus and functions by inhibiting B cell survival and differentiation. Anifrolumab, a monoclonal antibody directed against the type I interferon receptor, is used for the treatment of moderate to severe systemic lupus erythematosus by attenuating interferon-mediated inflammatory pathways. Telitacicept is a recombinant fusion protein that inhibits both BLyS and APRIL, thereby modulating B cell activity and is employed in the management of lupus erythematosus to reduce autoantibody production. Adrenocorticotropic hormone, also known as corticotropin, is used for its capacity to stimulate endogenous corticosteroid production, providing anti-inflammatory and immunosuppressive effects in patients with lupus erythematosus, particularly in acute or refractory cases.
| Structure | Generic Name | CAS Registry Number | Molecular Formula | Molecular Weight |
|---|---|---|---|---|
| telitacicept (Rec INN) | 2136630-26-5 | |||
| anifrolumab (Prop INN; USAN); anifrolumab-fnia | 1326232-46-5 | |||
| belimumab (Rec INN; USAN) | 356547-88-1 | |||
 | hydroxychloroquine hydrosulfate; hydroxychloroquine sulfate | 118-42-3 (free base); 747-36-4 | C18 H26 Cl N3 O . H2 O4 S | 433.95 |
 | adrenocorticotropic hormone; corticotropin | C210 H314 N56 O57 S | 4567.146 |
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