Systemic lupus erythematosus (disseminated lupus erythematosus, lupus) is a chronic inflammatory connective tissue disorder that can involve joints, kidneys, mucous membranes, blood, heart, lungs, CNS and blood vessel walls. Systemic lupus erythematosus (SLE) is an autoimmune disease characterized by antinuclear antibody (ANA) production with widespread immune dysregulation, often resulting in multiorgan system inflammation. Fifteen percent of all lupus cases have onset in childhood
It is both type ll and type lll Hypersensitivity reaction in which bound antibody antigen pairs(immune complexes)precipitate and cause a further immune response
may develop gradually over months or years with episodes (called flare-ups) of fever, feeling unwell, or any of the symptoms discussed below alternating with periods when symptoms are absent or minimal. Joint Problems: Joint symptoms, ranging from intermittent joint pains (arthralgias) to sudden inflammation of multiple joints (acute polyarthritis), occur in about 90% of people and may exist for years before other symptoms appear. In long-standing disease, marked joint deformity may occur (Jaccoud's arthropathy) but is rare. However, joint inflammation is generally intermittent and usually does not damage the joints. Symptoms:
SKin Skin rashes include a butterfly-like redness across the nose and cheeks (malar butterfly rash); raised bumps or patches of thin skin; and red, flat or raised areas on the face and sun- exposed areas of the neck, upper chest, and elbows. alopecia photosensitivity
LUNG It is common for people with lupus to feel pain when breathing deeply. The pain is due to recurring inflammation of the sac around the lungs (pleurisy), with or without fluid (effusion) inside this sac. Inflammation of the lungs (lupus pneumonitis), resulting in breathlessness, is rare, although minor abnormalities in lung function are common. Life-threatening bleeding into the lungs may rarely occur. Blockage of arteries in the lung caused by the formation of blood clots (thrombosis) can also occur.
HEART People with lupus may have chest pain due to (pericarditis). More serious but rare effects is coronary artery vasculitis, and inflammation of the heart muscle with scarring (fibrosing myocarditis), which can lead to heart failure The valves of the heart can rarely be involved People are at increased risk of coronary artery disease.
LYMPH NODE AND SPLEEN Wide-spread enlargement of the lymph nodes is common, particularly among children, young adults, and blacks of all ages. Enlargement of the spleen (splenomegaly) occurs in about 10% of people. People may experience nausea, diarrhea, and vague abdominal discomfort
NERVOUS SYSTEM headaches, mild impairment of thinking, personality changes, stroke, epilepsy, severe mental disorders (psychoses), or a condition in which a number of physical changes may occur in the brain, resulting in disorders such as dementia
BLOOD The numbers of red blood cells, white blood cells, and platelets may decrease. ( anemia hemolytic /aplastic, leukopenia, trombocytopenia) Anemic syndrome, recurrent infections, bleeding may occur. Also, and for other reasons, the blood may clot too easily, affecting other organs (such as strokes and blood clots to the lungs.
Gastrointestinal Tract abdominal pain, damage to the liver or pancreas (pancreatitis), or perforation of the gastrointestinal tract.
LUPUS NEPHRITIS Renal involvement in SLE is common A strong predictor of poor outcome Survival rates improved over the last few decades Current treatments are better than before but more toxic Still 10 to 15% of patients progressed to ESRD
Systemic Lupus Erythematosus Head and Neck Manifestations – Malar rash first sign in 50% – Erythematous maculopapular eruption after sun exposure – Oral ulceration – 3-5% nasal septum perforation – Acute parotid enlargement 10% – Xerostomia 15% – Larynx and trachea involvement uncommon -TVC thickening and paralysis, cricoarytenoid arthritis, subglottic stenosis
Clinical Diagnosis Urinalysis: abnormal sediments; hematuria; incidental proteinuria to nephrotic Hematology: anemia Renal Functions: normal to progressive renal failure Serology
LABORATORY antinuclear antibodies. if antinuclear antibodies are detected, antibodies to double-stranded DNA, other autoimmune antibodies (autoantibodies, anti-smith antibodies. antibodies to phospholipids, can help identify people at risk of recurrent blood clots. blood tests can also indicate anemia, a low white blood cell count, or a low platelet count.
Laboratory Diagnosis Anti-ds DNA – Most specific – Farr, ELIZA (Crithidia luciliae) – High levels correlates with renal flares Anti-SM – Specific – Only presence at most in 30% Anti-C1q – Increase in anti-C1q correlates with proliferative GN Others: Complement levels, ESR, Hgb, renal function
Management of Systemic Lupus Erythematosus General Use high-SPF sunscreen throughout the year. Encourage good sleep and nutritional patterns. Address psychological aspects of disease/treatment. Prescribe calcium and vitamin D supplements (especially if on corticosteroids). Immunize against pneumococcus. Treat with anticoagulant if evidence of antiphospholipid antibody is present (agent depends on if the child has had a clot or not). Perform annual ophthalmologic evaluations (especially if on hydroxychloroquine). Treat dyslipoproteinemia when present. Maintain good blood pressure control in those with hypertension
Noncytotoxic Nonsteroidal anti-inflammatory medications: – Prescribe for constitutional symptoms (arthralgia, fatigue, malaise). – Avoid ibuprofen (associated with aseptic meningitis in SLE).ibuprofen Use hydroxychloroquine Arthritis. – Cutaneous disease. Low-dose methotrexate may be useful with mild disease and when arthritis is a prominent feature.
Glucocorticoids Use topical steroids for cutaneous disease. Use low-dose oral (< 0.5 mg/kg/day) for: – Arthritis. – Serositis. – Mild cytopenia. Use moderate-dose oral for mild nephritis (mesangial, focal proliferative, membranous). Use high-dose oral for: – Diffuse proliferative lupus nephritis and membranous disease with nephrotic syndrome. – CNS disease. – Acute hemolytic anemia. Use pulse intravenous for: – Severe, life-threatening or organ-threatening disease. – Severe, hematologic abnormalities. – Catastrophic antiphospholipid syndrome.
Cytotoxic Azathioprine can be used as a steroid-sparing drug for:Azathioprine – Arthritis. – Serositis. – Following induction therapy with cyclophosphamide to maintain remission.cyclophosphamide Mycophenolate mofetil can be used as a steroid-sparing drug, especially if patient does not tolerate azathioprine, for: Mycophenolate mofetilazathioprine – Following induction therapy with cyclophosphamide to maintain remission.cyclophosphamide – Induction therapy in nephritis for patients who do not want the toxicity associated with cyclophosphamide. cyclophosphamide Cyclophosphamide is used for: Cyclophosphamide – Major renal involvement, particularly diffuse proliferative glomerulonephritis. – CNS lupus. – Catastrophic antiphospholipid syndrome. – Other Use plasmapheresis for catastrophic antiphospholipid syndrome. – Use IVIG for refractory thrombocytopenia. – Experimental Therapies* Anti-CD20 B-cell depletion therapy. – Autologous stem cell transplantation. – CTLA4Ig.
Thank you Ahmad Abed elhady, group 3 Hossen Husein, group 2 5 th year genreal medicine(2013-2014)