Systemic Diseases and Skin
Systemic lupus erythematosus (SLE) is a chronic autoimmune disease with a largely unknown etiology that affects organs such as skin, joints, kidneys, brain and lungs. It is seen nine times more frequently in women of 20-40 years of old compared to men, with a total incidence of 4-7/100.000 and a prevalence of 15-50/100.000. Pathogenesis thereof is not clearly known. Intracellular antigens, and apoptosis are contemplated. As a result of insufficient clearance of autoantigens secreted by necrotic or apoptotic cells from the circulation, various antinuclear autoantibodies against such antigens are formed. Both genetic and environmental factors take part in this process. The concordance in monozygotic twins is 14-57%. It is seen more frequently in tissue groups of HLA, DR2 and DR3. It has been speculated that estrogen plays an active role in SLE, since it is more frequently seen in women, and SLE is observed in some patients with Klinefelter’s syndrome. However, the effect of exogenous estrogen administration on exacerbation of SLE is not clearly understood due to contradictory study results. Ultraviolet rays and certain viral infections, particularly EBV, are also blamed. Symptoms such as fever, weight loss, fatigue and muscle pains are seen in SLE, as well as 90% of all SLE patients suffer from arthralgia, and 10% of all SLE patients suffer from Jaccoud arthritis causing deformity.
In again 90% of the patients, linear immunoglobulin and complement depositions are detected at the dermoepidermal junction. This detection is performed with lupus band test. The frequency of Raynaud’s phenomenon is 15-40%. SCEL (subacute cutaneous lupus erythematosus) lesions show anti-Ro antibody positivity. Also, splinter hemorrhage, angioneurotic edema, livedo reticularis, painless oral ulcers, or more rarely, genital ulcers may be seen. In 50% of all cases, an apparent kidney involvement occurs within the first years of disease. An interstitial pneumonitis, interstitial fibrosis, pulmonary vasculitis, pulmonary hypertension, pulmonary hemorrhage and pleural fluid in the lungs are observed in 50% of all cases.
The likelihood of pulmonary thromboembolism is higher in cases with antiphospholipid syndrome compared to others. Pericarditis and endocarditis verrucosa (Libman-Sacks) may show a quiet progression, but also may result in an embolism or a deteriorated valve structure. SLE patients are at higher risk for coronary heart diseases compared to general population.
Neurological Symptoms (Common)
- Organic brain syndrome,
- Delirium,
- Headaches,
- Peripheral neuropathy,
- Personality change,
- Convulsions.
Movement Disorders (More Rarely)
- Myelitis,
- Meningitis,
- Cranial nerve involvement .
Arterial thrombotic events are more often seen in patients with antiphospholipid syndrome. Patients with such condition present to the hospital with stroke and focal neurological problems. Scleritis, episcleritis, uveitis and retinal vasculitis in the eyes may be seen. Hematologically, anemia, leukopenia, thrombocytopenia, lymphadenopathy and splenomegaly may be detected. A thrombophilic abnormality may be observed as related to the presence of antiphospholipid antibodies, or as associated with nephrotic syndrome.
Treatment
In heart, brain or kidney involvement, as well as in severe hematological cases, high doses of steroids and immunosuppressive medicines are used. In such cases, cyclophosphamide maintains its status of being the main medicine despite of some side effects thereof. There are studies show that mycophenolate mofetil can be as effective as cyclophosphamide in lupus nephritis. In addition to its effectiveness, the less risky side effect profile of mycophenolate mofetil compared to that of cyclophosphamide enables us to use mycophenolate mofetil for remission-oriented interventions or maintenance treatment in patients with lupus nephritis. In skin or joint involvement, as well as mild hematological involvement and serositis cases, mid doses of steroids and antimalarial medicines are employed. In resistant cases, medicines such as azathioprine and methotrexate are used. For resistant skin problems, medicines such as dapsone and thalidomide are used.
