Questions and Answers
What is Systemic Lupus Erythromatosus (SLE)?
Systemic Lupus Erythromatosus (SLE) is more common in men than women.
False
What are the two types of Systemic Lupus Erythromatosus?
Discoid and systemic
Which ethnic groups show predisposition for SLE?
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What is a common symptom of Systemic Lupus Erythematosus?
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Patients with SLE do not experience joint destruction.
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What is the hallmark of Scleroderma?
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Match the manifestations of Scleroderma with their respective definitions:
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What is a common complication associated with SLE involving the heart?
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The ACR Criteria to diagnose SLE generally require _____ out of 11 symptoms.
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What percentage of patients with Scleroderma may die within 5 years of onset?
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Study Notes
Systemic Lupus Erythematosus (SLE)
- Multisystem chronic inflammatory disease of unknown cause, characterized by autoimmune activity.
- Two forms: Discoid (cutaneous) and systemic involvement affecting multiple systems.
- Autoantibodies target RBCs, lymphocytes, platelets; certain drugs can also trigger SLE.
Epidemiology of SLE
- Predominantly affects young adults aged 15-64; also observed in children (10-15% of cases).
- Gender ratio: More common in women at a ratio of 9:1.
- Higher incidence among certain ethnic groups: Native American, African American (earlier onset), Hispanic, Chinese, Filipino.
Pathology of SLE
- Activation of multiclonal B-cells results in excessive antibody production and immune complex formation.
- Immune complexes lead to inflammation and tissue damage, particularly involving vascular and tissue surfaces.
- Nearly all SLE patients have antinuclear antibodies; anti-DNA antibodies are also common but not exclusive to SLE.
Clinical Manifestations of SLE
- Symptoms include fatigue, fever, malaise, and weight loss; no joint destruction is typical.
- 30% of patients may experience deformities; it is often termed "the great imitator" due to symptom overlap with other diseases.
- Symptoms can fluctuate ("waxes and wanes") and may include arthralgias, skin rashes (butterfly rash), and sunlight sensitivity.
Extra-Auricular Manifestations of SLE
- Renal complications include lupus nephritis, often assessed via renal biopsy (staging I-IV).
- Cardiovascular issues: pericarditis, valvular disease, 9 times greater risk of myocardial infarction, rarely leading to cardiac tamponade (1%).
- Pulmonary symptoms: pleural effusions and pleurisy.
- CNS involvement can lead to cognitive difficulties, stroke, or hemorrhage due to vasculitis.
- Hematologic manifestations may include pancytopenia (deficiencies in red/white blood cells and platelets).
Diagnosis of SLE
- Diagnosis requires meeting at least 4 of 11 ACR criteria, including:
- Specific rashes (malar, discoid), photosensitivity, oral ulcers.
- Polyarthritis (involving more than 2 joints), pleuritis, or pericarditis.
- Renal disease, neurologic disorders (e.g., seizures, psychosis).
- Hematological abnormalities (anemia, neutropenia, thrombocytopenia).
- Positive tests for anti-dsDNA, anti-Sm antibodies; antinuclear antibody titer of 1:40 or higher is indicative.
- Laboratory focus on ANA, ESR, and pancytopenia assessment; clinical judgment vital for diagnosis.
Scleroderma
- An uncommon connective tissue disorder known as systemic sclerosis, with unknown etiology.
- More prevalent in women; prognosis is poor, with 50% mortality within 5 years of onset.
- Localized form affects skin and muscles, while systemic involves organs; localized rarely progresses to systemic.
Pathophysiology of Scleroderma
- Characterized by excessive collagen production leading to significant skin and tissue changes.
- Skin undergoes fibrosis, becoming shiny and hardened; facial features may tighten, resembling a mask.
- Raynaud's phenomenon is commonly observed, along with swelling, pain, and potential joint contractures; protecting hands is crucial.
Clinical Manifestations of Scleroderma
- Manifestations summarized by the acronym CREST:
- Calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, telangiectasia.
- Most affected systems include pulmonary, cardiac, gastrointestinal, kidney, and vascular systems.
- Early organ involvement correlates with a poorer prognosis; diagnosis is primarily clinical.
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Test your knowledge on Systemic Lupus Erythromatosus and Scleroderma with these flashcards. Explore key definitions, types, and related autoimmune aspects. Perfect for students and professionals in the medical field.
