Systemic Lupus Erythematosus Lecture 3 PDF

Systemic Lupus Erythematosus By Samar Tharwat Radwan Assistant Professor of Rheumatology and Immunology (Internal Medicine Department ) Musculoskeletal Ultrasound –EULAR Mansoura University –Horus University  At the end of this lecture ,the students should be able to : Identify th...

Systemic Lupus Erythematosus By Samar Tharwat Radwan Assistant Professor of Rheumatology and Immunology (Internal Medicine Department ) Musculoskeletal Ultrasound –EULAR Mansoura University –Horus University  At the end of this lecture ,the students should be able to : Identify the clinical manifestations of systemic lupus erythematosus Choose the relevant investigations in assessment of a case of systemic lupus erythematosus Identify the therapeutic options in systemic lupus erythematosus and their side effects. Identify antiphospholipid syndrome and drug induced lupus General Considerations Autoimmune disease Multisystem involvement Autoantibodies Variable presentation Female to male ratio is 9:1 Child bearing period Etiology of SLE remains unclear Genetic: twins Hormonal: sex hormones, menarche, oral contraceptive use, postmenopausal hormones, Klinefelter syndrome Environmental: smoking, UV light, viruses (EBV) Clinical Findings 1.Constitutional manifestations Fever Fatigue Weight changes  2. Mucocutaneous manifestations ) 80–90%( Photosensitivity Butterfly” rash Diffuse Patchy alopecia alopecia Discoid lesions Oral ulcers Livedo reticularis 3. Lymphadenopathy: multiple lymph node enlargement may be present.  4. Musculoskeletal manifestations Arthralgia or arthritis, usually affects small joints. However, large joints may also be affected. - Lupus arthritis is not deforming and not erosive. - Myositis: with proximal muscles weakness. - Avascular hip necrosis: due to prolonged use of steroids. Arthritis Arthralgia - In some cases, deformities may occur mimicking rheumatoid arthritis - However, these deformities are correctable. 5. Lupus nephritis 90% Hematuria, pyuria, dysmorphic red blood cells, and red blood cell casts Renal biopsy is done for diagnosis and classification of lupus nephritis. International Society of Nephrology/Renal Pathology Society classification of lupus nephritis 6. Cardiovascular Pericarditis Myocarditis which may lead to heart failure Valvular heart disease : libman-sacks endocarditis (non-infective endocarditis). Accelerated atherosclerosis 7. Pulmonary Pleurisy or Pleural effusions Lupus pneumonitis Interstitial lung disease Diffuse alveolar hemorrhage: hemoptysis, sudden decrease of hemoglobin level and new lung infiltration. Pulmonary arterial hypertension Vanishing lung syndrome 8. Gastrointestinal Abdominal pain Peritonitis Pancreatitis Mesenteric vasculitis:may lead to intestinal obstruction Intestinal pseudoobstruction Lupus hepatitis Vascular disorders : Budd-Chiarisyndrome Hepatic veno-occlusive disease, Hepatic infarction  9. Neuropsychiatric Investigations Laboratory Biopsy Radiology Hematology Anemia: Autoimmune, Fe deficiency ,anemia of chronic disease Leukopenia Lymphopenia Thrombocytopenia Chemistry Hyperkalemia Increase serum creatinine in renal deterioration Liver function tests Liver function tests: SGOT and SGPT may be elevated in lupus myositis or hepatitis. Muscle enzymes Muscle enzymes: CPK, LDH, aldolase may be elevated in lupus myositis. Acute phase reactants CRP is usually normal or even low in lupus ESR usually increases activity. in lupus activity. Autoantibodies - ANA: 95% of patients (highly sensitive) but not specific (present in normal individuals and in other connective tissue diseases. - Anti ds-DNA: Antibodies to double-stranded DNA. High specificity for SLE. Predict and assess disease activity in particularly those with renal disease. - Anti-Sm antibodies: High diagnostic specificity. - Anti SSA/Ro and SSB/La: Occur in patients with photosensitivity, dry eyes and dry mouth (secondary Sjogren’s syndrome). - Complement (C3, C4): Low in active disease. - Anti histone antibodies: drug induced lupus Biopsy Renal biopsy: in lupus nephritis. Skin biopsy: in undiagnosed skin lesions. Imaging Studies X-ray, CT chest : Pleural effusion, pericardial effusion…etc. X-ray joints, musculoskeletal US : To assess arthritis. Echocardiography: in heart affection. MRI brain: in lupus cerebritis. X ray joints: no deformity ,no erosion  X ray chest Percardial effusion Pleural effusion  CT Chest 1. Interstitial lung disease 2. Alveolar hemorrhge MRI brain: Lupus cerbritis Diagnostic Criteria >/= 4/11 criteria, serially or simultaneously= SLE After exclusion of other causes. Alternative criteria (SLICC 2012)  A. Accelerated Atherosclerosis B. End-stage Renal Disease Complications C. Infection D. Osteoporosis and Avascular Necrosis E. Malignancy Treatment Patient education Avoid sun Sun protective clothing Exercise Healthy diet Smoking cessation Avoidance of stress Avoidance of estrogen containing drugs Glucocoticoids Antimalarials (hydroxychroquine ) Methotrexate Azathioprine Mycophenolate mofetil Cyclophosphamide Plasmapharesis, rituximab and intravenous immunoglobulin (IVIG): in sever and resistant cases. Drug induced lupus It is an autoimmune disorder similar to SLE Caused by chronic use of certain drugs as hydralazine, procainamide, and isoniazid. Manifested as muscle pain and joint pain. Generally, the symptoms subsides after discontinuing use of the drugs.  Antiphospholipid syndrome It is an autoimmune hypercoagulable state caused by antibodies against cell membrane phospholipids It is characterized by : 1. Thrombosis in both arteries and veins 2. Pregnancy related complications as miscarriage ,stillbirth ,preterm labor ,or sever preeclampsia Investigations : Lupus anticoagulant Anticardiolipin antibody B2 glycoprotein 1 antibodies. Antiphospholipid syndrome may be : Primary :in the absence of any other related disease Secondary :coexists with other diseases such as SLE Systemic lupus