Systemic lupus erythematosus (SLE) is an autoimmune disease that affects multiple organs. Autoantibodies are produced due to failure to maintain self-tolerance. Antinuclear antibodies (ANAs).
What is the Pathology of Systemic Lupus Erythematosus?
The pathology of systemic lupus erythematosus is:
-Etiology: The cause of systemic lupus erythematosus is unknown, but can be attributed to multiple genetic predispositions and gene-environmental interaction.
-Pathogenesis: The sequence of events that lead to systemic lupus erythematosus is the self-dependent antigen activation of autoreactive B cells and CD4+ T cells in secondary lymphoid organs leading to the release of autoantibodies and inflammatory cytokines that lead to tissue injury.
-Morphologic changes: The morphologic changes involved with systemic lupus erythematosus can be seen on the:
- Skin (malar rash, urticaria, bullae and ulcerations)
- Kidneys (Minimal mesangial lupus nephritis, Mesangial proliferative lupus nephritis, Focal lupus nephritis, Diffuse lupus nephritis, Membranous lupus nephritis, and Advance sclerosing lupus nephritis)
- Heart (myocarditis, valvular abnormalities, and endocarditis)
- Spleen (splenomegaly, follicular hyperplasia, onion-skin lesions)
- Lungs (chronic interstitial fibrosis)
How does Systemic Lupus Erythematosus Present?
Patients with systemic lupus erythematosus typically women of childbearing age or at a range of 14-64 years, and predominates in Blacks, Asians, and Americans. The symptoms, features, and clinical findings associated with systemic lupus erythematosus include a triad of fever, joint pains, and a characteristic butterfly malar rash.
How is Systemic Lupus Erythematosus Diagnosed?
Systemic lupus erythematosus is diagnosed through clinical findings and laboratory evidence. Standard laboratory studies include CBC, serum creatinine, and urinalysis; autoantibody tests are also performed such as ANA and anti-dsDNA.
Criteria of systemic lupus erythematous include four or more of the following:
- Malar rash
- Discoid rash
- Photosensitivity
- Oral ulcers
- Arthritis
- Serositis
- Renal disorder
- Neurologic disorder
- Hematologic disorder
- Immunologic disorder
- Antinuclear antibody
Other laboratory tests that may be helpful in its diagnosis are presence of ESR/ CRP, complement levels, liver function tests, creatinine kinase assay, and spot protein/spot creatinine ratio. Radiologic studies are only used to monitor progression of the disease, and involvement of other organs.
How is Systemic Lupus Erythematosus Treated?
Systemic lupus erythematosus is treated mainly with hydroxychloroquine. Cutaneous manifestations are managed with NSAIDs and SLE without major organ involvement can benefit from glucocorticoids and antimalarial agents. Other immunosuppressive agents (azathioprine, mycophenolate mofetil, methotrexate) may be considered in refractory cases. DMARDS are also utilized to reduce disease activity such as belimumab, rituximab, and anifrolumab.
What is the Prognosis of Systemic Lupus Erythematosus?
The prognosis of systemic lupus erythematosus varies per individual. Systemic lupus erythematous ranges from benign to progressive to fatal manifestations of the disease. A milder disease progression and higher survival rate is associated with isolated skin and musculoskeletal disease manifestation; while patients with kidney and central nervous disease are associated with disease severity and poor survival rate.
