Systemic Lupus Erythematoses

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SLE - explained

Transcript of Systemic Lupus Erythematoses

  • 1. SYSTEMIC LUPUS ERYTHEMATOSUS (SLE) Dr. Angelo Smith M.D WHPL

2. Autoimmune disease that affects multisystems 1.5 million cases of lupus Prevalence of 17 to 48 per 100,000 population Women > Men - 9:1 ratio 90% cases are women African Americans >Whites Onset usually between ages of 15 and 45 years, but Can occur in childhood or later in life 3. Clinical Manifestations Ranges from a relatively mild disorder to rapidly progressing, affecting many body systems. Chronic with relapsing and remitting course. Most commonly affects the skin / muscles, lining of lungs, heart, nervous tissue, and kidneys 4. Etiology Etiology is unknown Most probable causes Genetic influence Hormones Environmental factors Certain medications 5. Pathogenesis of SLE 6. Pathophysiology Autoimmune reactions directed against constituents of cell nucleus, DNA Antibody response related to B and T cell hyperactivity 7. General symptoms The most common symptoms listed as initial complaints are fatigue, fever, and weight loss. Fever: fever secondary to active disease was recorded from 50% to 86%. No fever curve or pattern is characteristic. It can be difficult, but very important to distinguish the fever of SLE from that caused by complicating infections. 8. Clinical Manifestations Infection Increased susceptibility to infections Fever should be considered serious Infections such as pneumonia are a common cause of death 9. Fatigue is common in patients with SLE, especially during periods of disease activity. It is also often the only symptom that remains after treatment of acute flares. Low grade fever, anemia, or any source of inflammation can result in fatigue. 10. Clinical Manifestations Dermatologic Cutaneous vascular lesions Photosensitivity Butterfly rash (Malar Rash) Oral/nasopharyngeal ulcers Alopecia 11. Malar Rash 12. Discoid Rash 13. Maculopapular eruption 14. Oral Ulcers 15. Raynauds phenomenon is commonly found in lupus. It lack specificity. (a triphasic reaction of distal digits to cold or emotion, in which the skin colour changes from white to blue to red) 16. Vasculitic skin lesion 17. Clinical Manifestations Musculoskeletal (jaccoud arthropathy) Polyarthralgia with morning stiffness A vascular necrosis Arthritis bilateral hands / wrists / knees Swan neck fingers Ulnar deviation Subluxation with hyperlaxity of joints 18. Avacular necrosis of bone 19. Clinical Manifestations Cardiopulmonary Tachypnea Pleurisy Dysrhythmias Accelerated CAD Pericarditis 20. Pulmonary manifestations Pleurisy it is the most common manifestation of pulmonary involvement of SLE. The volume of pleural effusions usually is small to moderate and maybe unilateral or bilateral. Large pleural effusion are uncommon. It usually exudative in character. Pleural effusions may also occur in SLE patients with nephrotic syndrome, infection, cardiac failure. 21. Lung 1) acute lupus pneumonitis: fever, dyspnea, cough with scanty sputum, hemoptysis, tachypnea and pleuritic chest pain. 2) pulmonary hemorrhage 3) chronic diffuse interstitial lung disease. the diagnosis should not be made until infectious processes such as viral pneumonia, tuberculosis, and other bacterial, fungal and pneumocystis carinii infection have been completely excluded. 22. Cardiovascular manifestations Pericarditis is the most common cardiac manifestation of SLE. Myocarditis (the clinical features of lupus myocarditis resembles that of viral myocarditis) Libman-Sacks endocarditis and valvular disease Hypertension, cardiac failure 23. SLE can be associated with endocarditis. Shown here is Libman-Sacks endocarditis in which there are many flat, reddish-tan vegetations spreading over the mitral valve and chordae. 24. Clinical Manifestations Renal Lupus nephritis Ranging from mild proteinuria to glomerulonephritis Primary goal in treatment is slowing the progression 25. Haematuria Proteinure (>0.5g protein/d or 3+ ) Cast 26. Lupus nephritis Class I Minimal mesangial Normal light microscopy; abnormal electron microscopy Class II Mesangial proliferative Hypercellular on light microscopy Class III Focal proliferative 50% glomeruli involved; segmental/global Class V Membranous Predominantly nephrotic disease Class VI Advanced sclerosing Chronic lesions and sclerosis 27. Clinical Manifestations Nervous system Generalized/focal seizures Peripheral neuropathy Cognitive dysfunction Disorientation Memory and reasoning deficits Psychiatric symptoms severe depression / psychosis 28. Clinical Manifestations Red blood cells a normochromic, normocytic anemia is frequently found in SLE. They appears to be related to chronic inflammation, drug-related haemorrhage. haemolytic anemia as detected by the Coombs test is the feature of SLE. on rare occasion, a serum antibody may be produced which impairs red cell production. 29. Platelets thrombocytopenia (