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Systemic lupus erythematosus

            Systemic lupus erythematosus (SLE) is a multisystem autoimmune disorder, which is characterized by production of autoantibodies against nuclear antigens and against factors of coagulation system and manifests with a wide spectrum of clinical manifestations. The prevalence varies between 12 and 50 cases per 100 000, as it is higher in USA compared with Europe and differs in different ethnic groups. There is a female predominance with female to male ratio 9-10:1 and peak age of onset among young women between late teens and the age of 40. These age-related differences are associated with hormonal changes. No single cause of SLE is identified, but a number of etiologic factors has been noticed to cause SLE exacerbations such as sun light and drugs.


            Patient with SLE may report fever, hair loss, lymphadenopathy, which are non-specific symptoms that may occur at the disease presentation or in the periods of disease flares. Raynaud’s phenomenon occurs in over 1/3 of the patients and usually is mild without tissue necrosis and represents a clinical manifestations of vasospasm of  the peripheral arteries and arterioles mainly of fingers and toes provoked by cold exposure or emotional stress with well-demarcated blanching, cyanosis and redness of the affected areas. The disease may mimick pathologic conditions with different localization. Thus, according to the organ involvement, the patient may report for oedema, reduced diuresis and hypertension in cases of nephritis; for headache or seizures in patients with central nervous system involvement.            Arthralgias and arthritis are frequent findings in SLE patients as joint inflammation is classically polyarticular and symmetrical. Cutaneous lesions affect over 80% of SLE patients. The butterfly rash is erythematous, located over the malar bones and the nose, sparing the naso-labial folds. Although diagnostic, it is only one of the numerous ways, in which the disease may involve the skin. Another diagnostic skin lesion is the discoid lupus, which may be an isolated skin disease as well as a feature of SLE. Maculopapular rash with different localization, livedo reticularis, vasculitic lesions are examples of other skin manifestations of the disease. Renal involvement occurs in more than 50% of SLE patients. The lupus nephritis is classified histologically on the basis of the findings of renal biopsy. In SLE, the three layers of the heart may be involved – pericardium, myocardium and endocardium. Pericarditis is the most common form of heart involvement and manifests clinically with dyspnea and chest pain. Lung involvement manifests with interstitial fibrosis, pulmonary vasculitis and pneumonitis. Neuropsychiatric involvement in SLE may affect central or peripheral nervous system. Central nervous system manifestations include headache, seizure disorders, cerebrovascular diseases, etc. Examples of peripheral nervous system involvement are mononeuropathy, polyneuropathy, etc.


            Laboratory and instrumental methods

            Laboratory investigations include obligatory check-up of complete blood count and differential count. The anemia may be from haemolytic type or in the context of iron deficiency. Low platelet count (<100 000mm3), leucopenia (<4000mm3) and low lymphocyte count (<1500mm3) are characteristic features and a markers of disease activity in SLE.


            Urine analysis and quantitative measurement of protein and cells (leucocytes and erythrocytes in urine) should be performed when nephritis is suspected. Normal protein level in the urine for 24 hours is 150mg while in cases of lupus nephritis it is above 500mg for 24 hours. 


            Specific immunological markers for SLE are antibodies against native DNA (anti-dsDNA), against Sm antigen, antiphospholipid antibodies (anticardiolipin antibodies, anti-β2-glycoprotein, lupus anticoagulant). Antiphospholipid antibodies are associated with secondary antiphospholipid syndrome in a proportion of SLE patients that is characterized with recurrent arterial and venous thrombosis and/or miscarriages. Positive total antinuclear antibodies (ANA), measured by immunofluorescence at titer >1:160 in the absence of drugs known to be associated with "drug-induced lupus" syndrome is diagnostic for SLE in clinical context. Low serum concentration C3 and C4 complement components indicate disease activity.


            Chest X-ray and chest computed tomography are indicated in cases of pulmonary involvement. Echocardiogrpahy is performed, when pericardial effusion is suspected. Electromyography and head magnetic resonance imaging are methods used for demonstration of peripheral and central nervous system involvement. Invasive procedures such as arthrocentesis, lumbar puncture and renal biopsy with histological examination are performed if indicated.


            The therapy includes corticosteroids (used at varying therapeutic protocols – from pulse and high dose to medium and low dose), antimalarial agents, cytotoxic drugs (cyclophosphamide incl. pulse thrapy, azathioprine, mycophenolate mofetil, etc.), intravenous immunoglobulin, belimumab, etc. according to the form of the disease and the disease activity. Patient education and appropriate hygienic-dietary regimen is of crucial importance  for disease control. 


                                               Dr Sevdalina Lambova, MD, PhD