Systemic Lupus Erythematosus Overview

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Questions and Answers

What is the most specific laboratory test for diagnosing lupus?

C-Reactive Protein (CRP)

Anti-histone

Anti-dsDNA (correct)

Antinuclear Antibody (ANA)

Which demographic is statistically significant for higher prevalence of lupus nephritis according to the US ESRD Registry?

Asian females

Hispanic males

Caucasian males

African-American females (correct)

What environmental factor is NOT commonly associated with the etiology of lupus?

Sunlight

UV light

Tobacco

Physical Activity (correct)

What is the most common presenting feature of systemic lupus erythematosus?

Joint pain Signup and view all the answers

In a severe presentation of lupus with dyspnea and hemoptysis, what would be the best initial treatment?

High-dose corticosteroids Signup and view all the answers

What type of disorder is Systemic Lupus Erythematosus classified as?

Inflammatory heterogeneous autoimmune disorder Signup and view all the answers

At what age range is the peak incidence of Systemic Lupus Erythematosus observed?

14-45 years Signup and view all the answers

Which ethnic group has the highest prevalence of Systemic Lupus Erythematosus?

Native American Signup and view all the answers

What is the female-to-male ratio for Systemic Lupus Erythematosus?

9 to 1 Signup and view all the answers

Which genetic marker is associated with an increased risk of developing Systemic Lupus Erythematosus?

PTPN22 Signup and view all the answers

At what mean age is Systemic Lupus Erythematosus typically diagnosed?

31 years Signup and view all the answers

What is the primary characteristic of Systemic Lupus Erythematosus in terms of auto-antibody production?

Auto-antibodies directed against cell nuclei Signup and view all the answers

Which demographic has the lowest prevalence of Systemic Lupus Erythematosus?

White Men Signup and view all the answers

What is a common clinical feature of serositis in SLE?

Pulmonary hemorrhage Signup and view all the answers

Which of the following is NOT classified as a hematologic disorder in SLE?

Hyperglycemia Signup and view all the answers

What class of lupus nephritis indicates minimal mesangial changes?

Class I Signup and view all the answers

Which of the following cardiac features is not associated with SLE?

Coronary artery blockage Signup and view all the answers

What renal condition is characterized by proteinuria greater than 0.5 gms daily in lupus nephritis?

Nephrotic syndrome Signup and view all the answers

Which of the following neurologic symptoms can be a feature of SLE?

Both B and C Signup and view all the answers

Which of the following factors is NOT considered a high rate of flare in SLE?

Immediate surgical recovery Signup and view all the answers

What phenomenon involves dilated nailfold capillary loops in SLE?

Raynaud’s phenomenon Signup and view all the answers

Study Notes

Systemic Lupus Erythematosus (SLE)

Definition: An inflammatory, heterogeneous autoimmune disorder affecting multiple organ systems, characterized by the production of auto-antibodies directed against cell nuclei.
History: The condition was initially identified and described using various terms like "erythema centrifugum" and "lupus erythematosus", with early descriptions by figures like Laurent-Théodore Biett and Pierre Louis Cazenave.
Epidemiology:
- Peak incidence: 14-45 years old
- Predominantly affects females (85% are women) with a 9:1 female-to-male ratio.
- Ethnic predisposition: Native Americans, African Americans, Hispanics show higher prevalence compared to other ethnicities.
- Genetic factors: Genes like PTPN22, HLA DRB1, ITGAM, or ITGAX implicated.
Pathophysiology:
- Autoantibodies attack cell nuclei.
- Multi-system disease.
- Inflammatory response.
Costs:
- Represents 2% of all dialysis patients in the US.
- Approximately 4000 patients are receiving dialysis at any given time, with 1000 new patients starting per year.
- High rates of lupus nephritis are often observed.
- Statistically significant findings: Frequent occurrence within African-American and Medicaid patients.
Etiology:
- Environmental factors: UV light exposure.
- Infectious agents (viruses).
- Hormones (estrogen).
- Tobacco use.
Clinical Features: General Symptoms
- Fatigue
- Fevers
- Weight loss
- Anorexia
- Alopecia
- Lymphadenopathy
- Raynaud's phenomenon.
Clinical Features: Dermatological
- Malar rash: Fixed erythema affecting the malar eminences (cheeks), sparing the nasolabial folds
- Discoid lupus erythematosus (DLE): Erythematous patches with central clearing, keratotic scaling, follicular plugging.
Clinical Features: Oral Ulcers
- Oral/nasopharyngeal ulceration
- Typically painless.
Clinical Features: Musculoskeletal
- Transient, small joint involvement, symmetrical.
- "Jaccoud's" arthritis (a characteristic change).
- Very frequent symptom (90% of SLE cases).
Clinical Features: Cardiovascular
- Nonserosal: Cardiac arrhythmias, accelerated atherosclerosis.
- Immunologic: Libman-Sacks endocarditis, valvular heart disease.
Clinical Features: Renal
- Hallmark: Proteinuria (greater than 0.5g daily), casts "foamy" urine
- Nephrotic syndrome: Hypoalbuminemia, hyperlipidemia, and thrombophilia.
Clinical Features: Hematologic Disorder
- Hemolytic anemia.
- Leukopenia.
- Lymphopenia.
- Thrombocytopenia.
Clinical Features: Neurological
- Psychosis
- Seizures
- Behavioral/personality changes, migraines, depression, cognitive impairment, stroke, chorea, catatonia, pseudotumor cerebri, longitudinal myelitis, peripheral neuropathy, aseptic meningitis
Case Studies:
- (Case 2): 23-year-old Cambodian female presenting with worsening white, painful fingers (turning blue and red), dyspnea, hemoptysis. Possible diagnosis of SLE, CT chest imaging required
Immunologic findings:
- ANA (Antinuclear Antibody): Non-specific for SLE.
- Anti-dsDNA (double-stranded DNA): Highly specific for SLE.
- Anti-Smith (Sm) antibodies.
- Anti-Ro/SSA, Anti-La/SSB, and Anti-RNP antibodies.
- Antiphospholipid antibodies (aPLs), lupus anticoagulant, anti-cardiolipin, anti-beta2 glycoprotein.
- Depressed serum complement (C3, C4).
- Coombs test positivity.
2012 SLICC (Systemic Lupus International Collaborating Clinics) Classification Criteria for SLE (at least four criteria)
2019 European League Against Rheumatism (EULAR)/ACR Classification Criteria for SLE (at least one clinical criterion and 10 points)
Treatment: (Type I and II)
- Mild: NSAIDs, low-dose glucocorticoids, hydroxychloroquine
- Intermediate: Glucocorticoids, azathioprine, methotrexate, leflunomide, mycophenolate mofetil.
- Severe: Glucocorticoids (high doses), cyclophosphamide, plasmapheresis, IV immunoglobulins, mycophenolate mofetil, rituximab, belimumab, voclosporin, anifrolumab and other therapies.
Treatment Principles:
- 6 FDA approved therapies
- Recognize complications like infection, medication side effects, lifestyle factors
- Address teratogenicity, adherence to treatment, and rare drug side effects.
Pregnancy:
- Similar rates of flares, preeclampsia, fetal loss, preterm delivery.
- Low birth weight infants.
- DVT/PE, neonatal lupus, complete heart block possible.
Summary:
- Quintessential autoimmune condition with autoantibodies.
- Clinical domains: skin, musculoskeletal, renal, pulmonary, hematologic.
- Diagnosis requires CBC, urine protein/creatinine, complement, and anti-dsDNA profiles.
- Treatment often includes hydroxychloroquine, steroids, immunomodulators, and biologics.

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Description

This quiz covers the key aspects of Systemic Lupus Erythematosus (SLE), an autoimmune disorder affecting various organ systems. It delves into the definition, history, epidemiology, and pathophysiology of SLE, highlighting its impact on different populations. Explore the intricacies of this condition and enhance your understanding of its complexities.