Systemic%20lupus%20erythematosus Signs and Symptoms
Definition
- Systemic lupus erythematosus (SLE) is a chronic, multisystem, inflammatory, autoimmune disorder characterized by formation of autoantibodies directed against self-antigens and immune-complex formation
- Has a relapsing and remitting disease course with organ damage resulting from persistent disease activity and therapy
- SLE can be diagnosed with a single organ involvement such as lupus nephritis
- Clinical presentation varies in different patients and the disease activity varies over time in a single patient with frequent flares resulting in damage from inflammation
- Majority of patients have arthralgia of the hand
Epidemiology
- Predominantly diagnosed in females of childbearing age with a female-to-male ratio of 9:1
- The incidence and prevalence rates differ worldwide with SLE more frequently occurring in Asian, African, Caribbean and Hispanic individuals
- In the Asia-Pacific region, the incidence ranged from 0.9 to 8.4 and the prevalence ranged from 3.7 to 127 per 100,000 population
- Asian patients with SLE tend to have a higher disease activity, more severe disease, more organ damage and higher incidence of renal involvement
- Asian patients less commonly develop SLE-related antiphospholipid syndrome and venous thrombosis
Pathophysiology
- The interplay between genetic and environmental factors activate the patient's innate and adaptive immunity resulting in immunologic alterations
- Genetically susceptible individuals lose their immune tolerance upon exposure to environmental factors [eg cigarette smoke, silica, Epstein-Barr virus infection, ultraviolet B (UVB) radiation, oral contraceptives, drugs (eg Procainamide, Hydralazine, Isoniazid, Minocycline, tumor necrosis factor-α inhibitors)] resulting to the irregular activation of autoimmunity
- This leads to the production of autoantibodies, formation of immune complexes, production of autoreactive T cells and B cells, activation of complement and release of cytokines causing tissue damage which may be limited to an organ or can cause systemic involvement leading to significant morbidity and mortality
Signs and Symptoms
Clinical Features of SLE
- Cardiac
- Endocarditis
- Myocarditis
- Pericarditis
- Constitutional
- Fatigue
- Fever
- Weight loss
- Dermatological
- Alopecia
- Butterfly rash
- Livedo reticularis
- Mucous membrane lesion
- Photosensitivity
- Purpura
- Raynaud’s phenomenon
- Urticaria
- Vasculitis
- Gastrointestinal
- Abdominal pain
- Nausea, vomiting
- Oral ulcers
- Hematologic
- Anemia
- Leukopenia
- Thrombocytopenia
- Musculoskeletal
- Arthralgia
- Arthritis
- Myositis
- Neuropsychiatric
- Cognitive dysfunction
- Cranial neuropathies
- Peripheral neuropathies
- Psychosis
- Seizures
- Strokes
- Transverse myelitis
- Pulmonary
- Pleurisy
- Pulmonary hypertension
- Pneumonitis
- Serositis
- Renal
- Casts
- Hematuria
- Nephrotic syndrome
- Proteinuria
- Reticuloendothelial
- Hepatomegaly
- Lymphadenopathy
- Splenomegaly