1. Lupus
By: Brittni McClellan

2. Lupus - description Description: Genetics: Whites: HLA-DR2 and DR3
Autoimmune disease characterized by production of antibodies to various components of cell nucleus
Variety of clinical manifestations (Multisystem)
20% Begins in Childhood, F:M = 3-5:1
Genetics:
Whites: HLA-DR2 and DR3
Blacks: DR2 and DR7

3. Lupus – H&P Patient History: Photosensitivity/Malar Rash
Systemic Complaints:
Fevers, Fatigue, and Malaise
Raynaud’s Phenomenon
Joint Pain
Alopecia
Chest Pain:
Pericarditis/Pleural Effusions

4. Lupus – H&P Serositis: Neurologic: Constitutional Symptoms: Renal:
Signs and Symptoms:
Immune Complex– Mediated Vasculitis
Leukopenia
Renal Failure – Serositis: Usually seen as pericarditis or pleuritis,
Lymphopenia
Cutaneous lesions:
Thrombocytopenia
Serositis:
Neurologic:
Erythematous Malar or “Butterfly” Rash
Pericarditis
Depression
Pleuritis
Maculopapular Rashes
Seizures
Peritonitis
Periungual Erythema
Organic Brain Syndromes
Constitutional Symptoms:
Mucosal Membrane Vasculitis
Peripheral Neuropathies
Arthritis:
Renal:
Fatigue
Symmetric and Non-Erosive
Mesangial Changes
Weight Loss
Hematologic:
Glomerulonephritis
Fever
Hemolytic Anemia
Hypertension
Anemia of Chronic Disease
Nephrotic Syndrome

5. Lupus – signs and symptoms

6. Lupus - Diagnosis Immunoserology: Diagnosis: Diagnostic Criteria: 4/11
Malar (Butterfly) Rash
Discoid Rash
Photosensitivity
Oral/Nasal Ulcers
Arthritis
Cytopenia:
Anemia
Leukopenia
Lymphopenia
Thrombocytopenia
Neurologic Disease:
Seizures or Psychosis
Nephritis:
>0.5 g/d Proteinuria or Cellular Casts
Serositis:
Pleuritis or Pericarditis
Positive Immunoserology:
Antibodies to double-stranded DNA or Smith nuclear antigen, false-positive serologic test for syphilis, lupus r anticoagulant, or
Immunoserology:
Antinuclear Antibody (ANA):
Best Initial Test
High Sensitivity, Low Specificity
Anti-Double Stranded DNA (dsDNA):
Rises During Flares
Low Sensitivity, High Specificity
Indicates Renal Disease
Anti-Smith:
Antibody to snRNPs
Highest Specificity
Anti-Ro/Anti-La:
Seen in Sjogren Syndrome
Higher Risk of Neonatal Lupus in Pregnant Women
Anti-Histone Antibody:
Drug-Induced Lupus

7. Lupus - Diagnosis Diagnosis: CBC: Pancytopenia
Anemia
Leukopenia
Lymphopenia
Thrombocytopenia
Other laboratory abnormalities:
Decreased Complement Levels (C3/C4/CH50)
Increased ESR
Increased PTT:
Lupus Anticoagulant increases the risk for thrombi and miscarriages 
Associated with Antiphospholipid Syndrome
Urinalysis:
>0.5 g/d Proteinuria
Cellular Casts

8. Lupus – treatment Medication NSAIDS:
Musculoskeletal/Mild Systemic Complaints
Can Exacerbate Renal Disease
Ibuprofen: Causes Aseptic Meningitis in Some Patients
Hydroxychloroquine:
Controls Cutaneous Manifestations
Minimizes Lupus Flares
Steroids:
Systemic and Renal Manifestations
Immunosuppressants:
Examples: Cyclophosphamide, Mycophenolate Mofetil
Renal Manifestations
Methotrexate:
Arthritic Symptoms
Daily Baby Aspirin
Antiphospholipid Syndrome
ACE-Is:
Prevent Renal Damage and Proteinuria

9. Question 1
A 29-year-old woman with a 3-year history of systemic lupus erythematosus (SLE) presents to her rheumatologist for routine follow up. Her course has been complicated by lupus nephritis (baseline creatinine 1.1 mg/dL) and she has been on stable doses of methotrexate and hydroxychloroquine for the last 2 months following a flare. Which of the following lab values can be used to best monitor this patient's disease?
Anti-nuclear antibody
Anti-dsDNA antibody
Anti-Smith antibody
Anti-RNP antibody
CH50 level
Antinuclear Antibody (ANA):
Best Initial Test
High Sensitivity, Low Specificity
Anti-Double Stranded DNA (dsDNA):
Rises During Flares
Low Sensitivity, High Specificity
Indicates Renal Disease
Anti-Smith:
Antibody to snRNPs
Highest Specificity
Anti-Ro/Anti-La:
Seen in Sjogren Syndrome
Higher Risk of Neonatal Lupus in Pregnant Women
Anti-Histone Antibody:
Drug-Induced Lupus
b) Anti-dsDNA antibody

10. Question 2
A 27-year-old woman presents to her primary care physician for evaluation of malaise, joint pains, and rash. Over the last 1-2 months, she has felt generally unwell with fatigue and low-grade fever. More recently, she has started to experience joint pains in her hands and noticed a rash over her face after sun exposure. She is otherwise healthy and takes no medications. Examination reveals an erythematous rash with a small amount of underlying edema (Figure A). Which of the following is the most common cardiac manifestation of this patient's underlying condition?
Pericarditis
Myocarditis
Conduction arrhythmias
Valvular disease
Coronary artery disease
This patient presents with a clinical history consistent with systemic lupus erythematosus (SLE). The most common cardiac manifestation of SLE is pericarditis.  SLE can affect the heart in numerous ways. Most commonly it causes pericarditis, with a pericardial effusion present in as many as half of patients. Patients are not typically symptomatic from pericarditis, but suggestive history includes substernal, pleuritic chest pain with an audible rub on examination. SLE can also cause valvular disease, most often mitral regurgitation. Large verrucous vegetations (typically mitral, aortic, or tricuspid) in the context of SLE is referred to as Libman-Sacks endocarditis.  Snyder et al. discuss the diagnosis and management of acute pericarditis. ECG findings in acute pericarditis include widespread concave ST-segment elevations and depression of the PR-segment. They highlight predictors of severe illness, including high fever, evidence of tamponade or a large effusion, or subacute onset; these factors warrant strong consideration of inpatient admission rather than outpatient treatment.  Farzaneh-Far et al. present a series of 200 patients with SLE who underwent extensive cardiac evaluation. They sought to assess the relationship between the presence of antiphospholipid antibodies (APL) and cardiac disease. They found a strong correlation between the presence of high levels of APL and mitral valve disease, including nodules and valvular regurgitation. APL levels did not correlate with altered LV size or function.  Figure A shows a typical "butterfly" (or malar) rash in a patient with SLE. This rash is photosensitive, spares the nasolabial folds, and occurs in up to half of patients with SLE.  Incorrect Answers:  Answer 2: Myocarditis can occur with SLE, and similarly is often asymptomatic, but is less common than pericarditis.  Answer 3: Conduction abnormalities typically represent scarring damage from prior myocarditis in SLE. When present, it is often a transient first-degree AV block.  Answer 4: Mitral valve disease (especially with resultant regurgitation) is a common cardiac manifestation of SLE, but pericarditis is more common. Mitral valve disease is especially associated with Libman-Sacks endocarditis.  Answer 5: Patients with SLE are at markedly increased risk for coronary artery disease, but it is still less common than pericarditis as a cardiac manifestation of SLE.
a) Pericarditis