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  1. Systemic Lupus Erythematous (SLE) 20 22 20 22 Lupus clinical case Systemic lupus erythematosus (SLE) Lupus clinical case Systemic lupus erythematosus (SLE)
  2. Introduction 20 22 20 22
  3. ● Systemic Lupus Erythematosus (SLE), or lupus, is an autoimmune disorder where the immune system produces antibodies that attack the body’s own tissues, causing inflammation in many different parts of the body such as the skin, joints, brain, lungs, heart and kidneys. ● SLE predominantly affects women between 12 and 45 (frequently starting at childbearing age) INTRODUCTION
  4. Epidemiology
  5. ● Female:Male = 9:1 ● About 90% of SLE sufferers are women while about 10% are men and children. ● About 90% of women with SLE are in their childbearing years, within the range of 15 to 50 years old. ● In Malaysia, it is estimated that more than 10,000 people have been diagnosed with SLE over the past 30 years. However, the Malaysian SLE Association believes that there are many more SLE sufferers in Malaysia who have not been diagnosed. ● In Asians: 30–50/100,000 individuals ● A prevalence of 43/100,000 individuals in Malaysia (2012) ○ Chinese (57/100,000) ○ Malays (33/100,000) ○ Indians (14/100,000) ● Ratio of SLE sufferers : In the West, among Afro-Carribeans 1 in 250-500 people ○ USA – 1 in 2,000 people ○ China – 1 in 1,000 people EPIDEMIOLOGY
  6. Aetiology Nuratun ‘Aufa Binti Bahrul Kamil 20 22 20 22
  7. AETIOLOGY The exact etiology is unknown, but several predisposing factors have been identified. ● Genetic predisposition ○ HLA-DR2 and HLA-DR3 are commonly present in individuals with SLE. ○ Genetic deficiency of classical pathway complement proteins (C1q, C2, C4) in approx. 10% of affected individuals ● Hormonal factors: Hyperestrogenic states (e.g., due to oral contraceptive use, postmenopausal hormonal therapy, endometriosis) are associated with an increased risk of SLE. 20 22 20 22
  8. AETIOLOGY ● Environmental factors ○ Cigarette smoking and silica exposure increase the risk of developing SLE. ○ UV light and EBV infection may trigger disease flares, but there is insufficient evidence on whether they cause SLE. ○ Drugs such as procainamide or hydralazine 20 22 20 22
  9. Pathophysiology 20 22 20 22
  10. Pathophysiology ● Autoantibody development: deficiency of classical complement proteins (C1q, C4, C2) → failure of macrophages to phagocytose immune complexes and apoptotic cell material (i.e., plasma and nuclear antigens) → dysregulated, intolerant lymphocytes targeting normally hidden intracellular antigens → autoantibody production (e.g., ANA, anti-dsDNA) ● Autoimmune reactions ○ Type III hypersensitivity (most common in SLE) → antibody-antigen complex formation in microvasculature → complement activation and inflammation → damage to skin, kidneys, joints, small vessels ○ Type II hypersensitivity → IgG and IgM antibodies directed against antigens on cells (e.g., red blood cells) → cytopenia 20 22 20 22
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  13. Clinical Features 20 22 20 22
  14. Clinical Features SLE is a systemic disease characterized by phases of remission and relapse. Some individuals only experience mild symptoms, while others experience severe symptoms and rapid disease progression. SLE can affect any organ. Common ● Constitutional: fatigue, fever, weight loss ● Joints (> 90% of cases) ○ Arthritis and arthralgia ○ Distal symmetrical polyarthritis 20 22 20 22
  15. Clinical Features ● Skin (85% of cases) ○ Malar rash (butterfly rash) ○ Raynaud phenomenon ○ Photosensitivity → maculopapular rash ○ Discoid rash ○ Oral ulcers (usually painless) ○ Nonscarring alopecia ○ Periungual telangiectasia 20 22 20 22
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  17. COMPLICATION Inflammation caused by lupus can affect many areas of the body, including: ● Kidneys. Lupus can cause serious kidney damage, and kidney failure is one of the leading causes of death among people with lupus. ● Brain and central nervous system. If the brain is affected by lupus, the patient may experience headaches, dizziness, behavior changes, vision problems, and even strokes or seizures. Many people with lupus experience memory problems and may have difficulty expressing their thoughts. ● Blood and blood vessels. Lupus may lead to blood problems, including a reduced number of healthy red blood cells (anemia) and an increased risk of bleeding or blood clotting. It can also cause inflammation of the blood vessels. ● Lungs. Having lupus increases the chances of developing an inflammation of the chest cavity lining, which can make breathing painful. Bleeding into lungs and pneumonia also are possible. ● Heart. Lupus can cause inflammation of the heart muscle, the arteries or heart membrane. The risk of cardiovascular disease and heart attacks will increases.
  18. ► Having lupus also increases the risk of: ► Infection. People with lupus are more vulnerable to infection because both the disease and its treatments can weaken the immune system. ► Cancer. Having lupus appears to increase your risk of cancer ► Bone tissue death. This occurs when the blood supply to a bone declines, often leading to tiny breaks in the bone and eventually to the bone's collapse. ► Pregnancy complications. Women with lupus have an increased risk of miscarriage. Lupus increases the risk of high blood pressure during pregnancy and preterm birth.
  19. Investigation
  20. Lab studies Finding 1) Complete blood count - Leukopenia - thrombocytopenia - Normochromic normocytic anemia - increase in ESR - +/- autoimmune hemolytic anemia - normal CRP - Rh factor positive (30-50%) - False positive syphilis serology - Complement level low (C3 and C4) 2) Autoantibody test - Antinuclear antibody (ANA) is positive - Anti ds-DNA -> double stranded DNA is positive (50%) - Antiphospholipid antibodies (APLs) is positive - Anti-Sm is positive of people with lupus (30%) - Anti Ro (SSA) and anti-La (SSB) -> more common in Sjogren syndrome patient. - Raised igG and IgM -
  21. 3) Urine test Microalbuminuria Creatinine clearance increase Urine microscopic : >5RBCs, >5 WBCs, cellular casts, spot proteins or spot creatinine (proteinuria +) 4) Liver function test Mildly elevated in acute SLE in response to NSAIDs Creatine kinase levels may be elevated
  22. 5) Radiologic studies Joint radiography -> radiographic anomalies in SLE are periarticular osteopenia and soft tissue swelling without erosions Chest X-ray CT scan -> Fluid or inflammation in the lung -> Vasculitis, antiphospholipid, renal failure -> right sided pleural effusion (yellow) -> Risk of developing pulmonary emboli -> atelectasis with scarring in the left -> CT angiogram demonstrates filling defect in lung base (blue) left anterior segmental arrow CXR: monitor interstitial lung disease & assess pneumonitis, PE, alveolar hemorrhage
  23. 5) Radiologic studies • Brain MRI -> used to evaluate for CNS lupus white matter changes, vasculitis, stroke -> Axial, T2-weighted brain MRI demonstrate area of ischemic in the right periventricular white matter 6) Joint effusion & CSF studies • Arthrocentesis -> cell count range <25% PMNs in noninflammatory effusions to >50% in inflammatory effusions -> gross appearance : straw colored or clear, viscosity (noninflammatory cases) of fluids : yellow, viscosity (inflammatory cases) • Lumbar puncture -> Elevated in nonspecific cell count found in CSF fluid -> Proteins level increase with CNS lupus -> Glucose decrease
  24. 7) Kidney biopsies • Help in distinguishing renal lupus from renal vein thrombosis which may be a complication of antiphospholipid antibody syndrome -> serum creatinine increased (absence of sepsis, hypovolemia, medication) -> spot protein: proteinuria >1.0g/24 hour -> proteinuria (0.5g/>24 hours) with; 1) hematuria (>5 RBC/hpf) 2) cellular casts
  26. ● Maintaining an active lifestyle to keep joints flexible and may prevent cardiovascular complications. . ● Patients with lupus should avoid excessive sun exposure because the ultraviolet rays in sunlight can cause a skin rash to flare, and may even trigger a more serious flare in the disease itself. Wearing protective clothing (long sleeves, a big-brimmed hat) and using sunscreen liberally when outdoors on a sunny day should protect against such complications. ● Develop strategies for maintaining wellness. Wellness involves close attention to the body, mind, and spirit. One of the primary goals of wellness for people with SLE is coping with the stress of having a chronic disorder. Effective stress management varies from person to person. Some approaches that may help include exercise, relaxation techniques, and setting priorities for spending time and energy. ● Develop and maintain a good support system.A support system may include family, friends, medical professionals, and support groups such as Persatuan SLE Malaysia. ● Learning more about SLE also helps. Studies have shown that patients who are well-informed and participate actively in their own care tend to experience less pain, make fewer visits to the doctor, and remain more active. Non pharmalogical
  27. 1) NSAIDs - Naproxen sodium - Ibuprofen -> treat pain, swelling and fever associated with lupus -> stomach bleeding, kidney disease and increased risk of heart disease 2) Antimalarial drugs - Hydroxychloroquine -> used to treat malaria -> affect the immune system and help decrease the risk of lupus flares. -> stomach upset -> damage to retina eyes (rarely)
  28. 3) Corticosteroid - Prednisone - Methylprednisolone* - Triamcinolone (IM) -> can counter the inflammation of lupus -> high dose steroids often used to control serious disease that involves kidneys and brain* -> weight gain -> HTN -> easy bruising -> diabetes -> thinning bones -> risk of infection 4) Immunosuppressants - Azathioprine - Cyclosporine - Leflunomide - Mycophenolate - Methotrexate -> Drugs that suppress the immune system -> increased risk of infection -> liver damage -> decreased fertility -> increased risk of cancer
  29. 5) Biologics - Belimumab (IV) - Rituximab -> Reduces lupus symptoms in people -> useful in other medications that haven’t helped -> Nausea, diarrhea, infections, depression worsening -> allergic reactions to the intravenous and infections
  30. ● ● atous/5712406 ● ● References
  31. THANK YOU 20 22 20 22 Lupus clinical case Awesome words Lupus clinical case Awesome words