Podcast
Questions and Answers
What is the primary goal in the management of Systemic Lupus Erythematosus (SLE)?
What is the primary goal in the management of Systemic Lupus Erythematosus (SLE)?
Hydroxychloroquine is used in the management of non-life-threatening SLE.
Hydroxychloroquine is used in the management of non-life-threatening SLE.
True
What is the first step in managing Systemic Lupus Erythematosus?
What is the first step in managing Systemic Lupus Erythematosus?
Identifying whether SLE is constitutional or life threatening/organ related
In the case of non-life-threatening SLE, the treatment usually involves low-dose ______.
In the case of non-life-threatening SLE, the treatment usually involves low-dose ______.
Signup and view all the answers
Match the following treatment options with their corresponding SLE categories:
Match the following treatment options with their corresponding SLE categories:
Signup and view all the answers
Which antibody is most specific for psychosocial symptoms in CNS lupus?
Which antibody is most specific for psychosocial symptoms in CNS lupus?
Signup and view all the answers
Anti Ro/La antibodies have no significance in asymptomatic patients.
Anti Ro/La antibodies have no significance in asymptomatic patients.
Signup and view all the answers
What is the major central nervous system (CNS) manifestation in lupus?
What is the major central nervous system (CNS) manifestation in lupus?
Signup and view all the answers
The presence of Anti ds DNA antibodies is associated with an increased risk of ______ and _____ in SLE patients.
The presence of Anti ds DNA antibodies is associated with an increased risk of ______ and _____ in SLE patients.
Signup and view all the answers
Match the following antibodies with their associated conditions:
Match the following antibodies with their associated conditions:
Signup and view all the answers
Which neurological symptom is the most common in patients with systemic lupus erythematosus (SLE)?
Which neurological symptom is the most common in patients with systemic lupus erythematosus (SLE)?
Signup and view all the answers
An anti-dsDNA antibody is considered to have low specificity in the classification criteria for systemic lupus erythematosus.
An anti-dsDNA antibody is considered to have low specificity in the classification criteria for systemic lupus erythematosus.
Signup and view all the answers
What is the minimum number of points required for classification as having systemic lupus erythematosus (SLE)?
What is the minimum number of points required for classification as having systemic lupus erythematosus (SLE)?
Signup and view all the answers
The presence of _____ is a required entry criterion for patients to be classified as having systemic lupus erythematosus.
The presence of _____ is a required entry criterion for patients to be classified as having systemic lupus erythematosus.
Signup and view all the answers
Match the following cutaneous symptoms of SLE with their corresponding points:
Match the following cutaneous symptoms of SLE with their corresponding points:
Signup and view all the answers
Which of the following drugs is NOT a treatment option for refractory lupus?
Which of the following drugs is NOT a treatment option for refractory lupus?
Signup and view all the answers
Anti ds DNA antibodies are typically positive in drug-induced SLE.
Anti ds DNA antibodies are typically positive in drug-induced SLE.
Signup and view all the answers
Name one goal of treatment for lupus.
Name one goal of treatment for lupus.
Signup and view all the answers
The drug _______ is an anti type I interferon used for refractory lupus.
The drug _______ is an anti type I interferon used for refractory lupus.
Signup and view all the answers
Match the following causes of drug-induced SLE with their respective letters:
Match the following causes of drug-induced SLE with their respective letters:
Signup and view all the answers
Which of the following statements about Antiphospholipid Syndrome (APS) is correct?
Which of the following statements about Antiphospholipid Syndrome (APS) is correct?
Signup and view all the answers
Half of the cases of Antiphospholipid Syndrome are secondary to diseases other than SLE.
Half of the cases of Antiphospholipid Syndrome are secondary to diseases other than SLE.
Signup and view all the answers
What is the primary mechanism by which antibodies affect platelets in Antiphospholipid Syndrome?
What is the primary mechanism by which antibodies affect platelets in Antiphospholipid Syndrome?
Signup and view all the answers
The presence of ______ may indicate Sjogren's syndrome or Systemic Lupus Erythematosus.
The presence of ______ may indicate Sjogren's syndrome or Systemic Lupus Erythematosus.
Signup and view all the answers
Match the following conditions with their relevance to Antiphospholipid Syndrome:
Match the following conditions with their relevance to Antiphospholipid Syndrome:
Signup and view all the answers
Which of the following is commonly associated with SLE and has a high risk of developing into diffuse large B-cell lymphoma (DLBCL)?
Which of the following is commonly associated with SLE and has a high risk of developing into diffuse large B-cell lymphoma (DLBCL)?
Signup and view all the answers
Pleuritis with effusion is not a common symptom observed in SLE patients.
Pleuritis with effusion is not a common symptom observed in SLE patients.
Signup and view all the answers
What is an indication for renal biopsy in patients with SLE?
What is an indication for renal biopsy in patients with SLE?
Signup and view all the answers
With SLE, the most common valvular heart disease is __________.
With SLE, the most common valvular heart disease is __________.
Signup and view all the answers
Match the following SLE-related complications with their characteristics:
Match the following SLE-related complications with their characteristics:
Signup and view all the answers
What is the most common form of Lupus specific cutaneous manifestation?
What is the most common form of Lupus specific cutaneous manifestation?
Signup and view all the answers
Lupus is often associated with weight gain and increased energy levels.
Lupus is often associated with weight gain and increased energy levels.
Signup and view all the answers
What skin condition is associated with Anti Ro/La positivity?
What skin condition is associated with Anti Ro/La positivity?
Signup and view all the answers
The ______ rash is a hallmark of Acute Cutaneous Lupus Erythematosus.
The ______ rash is a hallmark of Acute Cutaneous Lupus Erythematosus.
Signup and view all the answers
Match the following cutaneous manifestations of lupus to their descriptions:
Match the following cutaneous manifestations of lupus to their descriptions:
Signup and view all the answers
Which characteristic is associated with Discoid Rash (DLE)?
Which characteristic is associated with Discoid Rash (DLE)?
Signup and view all the answers
Patients with Subacute Cutaneous Lupus Erythematosus (SCLE) commonly have non-scarring lesions.
Patients with Subacute Cutaneous Lupus Erythematosus (SCLE) commonly have non-scarring lesions.
Signup and view all the answers
What is the risk associated with Discoid Rash (DLE)?
What is the risk associated with Discoid Rash (DLE)?
Signup and view all the answers
In SCLE, the lesions are typically found in __________ areas.
In SCLE, the lesions are typically found in __________ areas.
Signup and view all the answers
Match the types of lupus-related skin conditions with their characteristics:
Match the types of lupus-related skin conditions with their characteristics:
Signup and view all the answers
What condition is characterized by non-erosive, deforming arthritis due to ligament laxity?
What condition is characterized by non-erosive, deforming arthritis due to ligament laxity?
Signup and view all the answers
Acute coronary syndrome is a common cause of death in patients with SLE after 10 years of disease.
Acute coronary syndrome is a common cause of death in patients with SLE after 10 years of disease.
Signup and view all the answers
What percentage of SLE patients progress to chronic kidney disease (CKD)?
What percentage of SLE patients progress to chronic kidney disease (CKD)?
Signup and view all the answers
In antiphospholipid antibody syndrome (APLA), patients with SLE often experience _______ as a complication.
In antiphospholipid antibody syndrome (APLA), patients with SLE often experience _______ as a complication.
Signup and view all the answers
Match the following complications with their descriptions:
Match the following complications with their descriptions:
Signup and view all the answers
Which class of lupus nephritis is characterized by over 90% glomerular sclerosis?
Which class of lupus nephritis is characterized by over 90% glomerular sclerosis?
Signup and view all the answers
Class 2 Mesangial proliferative lupus nephritis usually presents with over 50% glomerular involvement.
Class 2 Mesangial proliferative lupus nephritis usually presents with over 50% glomerular involvement.
Signup and view all the answers
What is a common feature seen in Class III and IV lupus nephritis?
What is a common feature seen in Class III and IV lupus nephritis?
Signup and view all the answers
The _____ pattern in lupus nephritis indicates the presence of IgG, IgA, IgM, Clq, and C3 deposits.
The _____ pattern in lupus nephritis indicates the presence of IgG, IgA, IgM, Clq, and C3 deposits.
Signup and view all the answers
Match the following classes of lupus nephritis with their corresponding features:
Match the following classes of lupus nephritis with their corresponding features:
Signup and view all the answers
Study Notes
Systemic Lupus Erythematosus (SLE) Management
- Treatment targets reduction of SLEDAI to zero
- Initial treatment depends on disease severity:
- Constitutional symptoms: Hydroxychloroquine (HCQ) and close observation
- Non-life-threatening SLE: Low-dose steroid
- Progression through treatment stages based on response
- Anti-dsDNA antibody: Present in 75% of SLE patients, preferred for ELISA testing
- Anti-Smith antibody: Present in 25% of SLE patients, more specific, absence indicates lower risk of nephritis and vasculitis
Antiphospholipid Syndrome (APS)
- Occurs in 1/3 of SLE cases
- Can be primary or secondary:
- Primary: Associated with HLA DR4, HLA DRWS3
- Secondary: Most common cause is SLE
- Complications:
- Accelerated atherosclerosis
- Glomerular disease (Class III/IV Lupus nephritis)
- Complications of immunosuppression
- Vasculitis (medium vessel: GIT/CNS)
- Increased susceptibility to thrombus formation
- Associated with Thrombocytopenia (low platelet count: 50,000-100,000/µL)
- Resistance to activated protein C due to antibody competition for phospholipid binding
SLE - Clinical Profile & Management
- CNS:
- Cognitive decline (most common)
- Increased risk of stroke due to antiphospholipid antibody
- Small fiber neuropathy
- CNS vasculitis
- ACR Criteria for SLE classification (low sensitivity, high specificity)
- EULAR/ACR classification criteria for SLE:
- Requires 10 points to classify as SLE
- All patients must have ANA ≥ 1:80
- Points are awarded based on clinical and immunologic domains
- Maintenance Rx:
- Steroid (tapered slowly)
- Cyclophosphamide if no response to steroid
- Refractory Lupus (no response to standard treatment):
- Rituximab
- Voclosporin (Calcineurin inhibitor) + MMF
- Belimumab (Anti BLYS)
- Anifrolumab (Anti type I interferon)
- Plasma exchange for DAH/CNS vasculitis
- Goals of Rx:
- Asymptomatic
- Normal urine
- Drug-Induced SLE:
- Causes:
- CHIMP (Carbamazepine, Chlorpromazine, Hydralazine, Isoniazid, Procainimide, Interferon, Infliximab)
- Clinical features:
- Mostly in females
- Negative anti-dsDNA, positive anti-histone
- 100% ANA positive
- Skin and joint involvement
- No CNS or renal involvement
- Good prognosis on withdrawal of offending drug
- Causes:
Hematological Manifestations
- RBC:
- Anemia of chronic disease
- Rapidly progressive anemia: Warm antibody (IgG) autoimmune hemolytic anemia (AIHA)
- WBC:
- Leukopenia/Lymphopenia
- Increased risk of diffuse large B-cell lymphoma (DLBCL)
- Platelet:
- Secondary immune thrombocytopenic purpura (2° ITP)
Lung Manifestations
- Lung parenchyma spared (no ILD)
- Pleuritis (most common) with or without effusion (small, bilateral, exudative)
- Cough and hemoptysis
- Bilateral airspace opacification on X-ray
- Viral/TB infection prompts discontinuation of immunosuppressants
- Diffuse alveolar hemorrhage (DAH) requires continued immunosuppressant therapy
- Poor prognosis for DAH
- SLE lung disease activity is the most important marker for prognosis
Cardiac Manifestations
- Pericarditis (without tamponade - most common)
- Myocarditis (associated with anti-Ro antibodies)
- Libman-Sacks endocarditis (vegetations on the valve surface)
- Neonatal lupus with congenital heart block (if anti-Ro/La antibodies are present during pregnancy)
- Mitral regurgitation (MR) is the most common valvular heart disease
GIT Manifestations
- Generally spared
Renal Manifestations
- Indications for renal biopsy in SLE:
- Proteinuria > 1 g/day
- Proteinuria > 2500 mg/day with 23+ RBCs in urine (microhematuria)
Hepatobiliary Manifestations
- Type 1 autoimmune hepatitis (lupoid hepatitis)
Assessment of Disease Activity
- Clinically active disease (new onset rashes)
- Increased anti-dsDNA titers
- Decreased C3 and C4 titers
- Increased erythrocyte sedimentation rate (ESR)
- Decreased C-reactive protein (CRP)
Clinical Features
- Constitutional symptoms:
- Fatigue and weight loss
- Pyrexia of unknown origin (PUO)
- Leucopenia
- Cutaneous manifestations:
- Classified into lupus-specific and non-specific
- Lupus-specific:
- ACLE (most common)
- Localized: Malar rash
- Generalized: Spare Knuckles
- Toxic epidermal necrolysis (TEN)
- CCLE: Discoid lupus
- SCLE:
- Associated with HLA DR 3
- Photosensitive, anti-Ro/La antibodies present
- ACLE (most common)
- Localized ACLE (Malar Rash, Butterfly Rash):
- Associated with younger age of onset
- Located on malar eminences, spares nasolabial folds
- Symmetrical, erythematous, scaly, non-scarring rash ± edema, highly photosensitive
- Features associated with ACLE:
- Painless oral/nasopharyngeal ulcers
- Oral ulcer
- Non-scarring alopecia
- Discoid Rash (DLE):
- 5% of patients with DLE have SLE
- 20% of patients with SLE have DLE
- Location: Face, scalp, neck
- Characteristics: Circular, raised erythematous patches, scarring, follicular plugging, keratin scaling, dermal atrophy, risk of progression to squamous cell carcinoma, associated with cicatricial (scarring) alopecia
- Subacute Cutaneous Lupus Erythematosus (SCLE):
- 20% of patients with SCLE have SLE
- Prevalent in white population
- Associated with: HLA DR3, Anti Ro (decreased renal involvement)
- Location: Sun-exposed areas, spares mid-facial region
- Characteristics: Non-scarring, highly photosensitive, patterns: annular, psoriasiform (increased conversion to SLE)
Musculoskeletal Manifestations
- Chronic inflammatory synovitis (similar to RA):
- Site: Upper limb -> Lower limb
- Small joint involvement (wrist)
- Features: Bilateral symmetrical arthralgia and arthritis
- Non-erosive, deforming arthritis (due to ligament laxity): Jaccoud's arthropathy (also seen in Sjogren's)
- SLE with erosive arthritis: Rhupus (RA/SLE overlap), anti-CCP(+)
- Complications:
- Septic arthritis: Patient on Rx for SLE, single joint involved out of proportion
- Avascular Necrosis: Acute joint pain after ~3 months of disease, IOC: MRI
- Crystal arthropathy
Organ System Involvement
- Bad prognosis for SLE with organ system involvement
- Kidney involvement (50-60%): 10% progress to CKD, immunosuppressive Rx leads to infection (most common cause of death in the first 10 years of disease)
- Accelerated atherosclerosis: MI equivalent, acute coronary syndrome (most common cause of death after 10 years of disease)
- Lung parenchyma spared
Vascular Manifestations
- Antiphospholipid antibody syndrome (APLA): In 1/3 patients with SLE, thrombosis
- Accelerated atherosclerosis
- Vasculitis:
- Immune complex mediated:
- Small vessel: Palpable purpura (most common)
- Dangerous medium vessel: Mesenteric, CNS
- Immune complex mediated:
Classification of Lupus Nephritis
- Class I: Minimal mesangial (asymptomatic, excellent prognosis)
- Class II: Mesangial proliferative (requires active immunosuppression, poor prognosis)
- Class III: Focal lupus nephritis (adult onset nephrotic syndrome, poor prognosis)
- Class IV: Diffuse (resistant to steroid, better prognosis than Class III)
- Class V: Membranous (membranous nephropathy, worst prognosis)
- Class VI: Advanced sclerotic lupus ( >90% glomerular sclerosis, CKD, worst prognosis)
- Class III & IV Lupus Nephritis:
- HP: Endocapillary + mesangial proliferation
- IF: IgG, IgA, IgM, Clq, C3 deposits
- Full house pattern : Subendothelial (wire loop lesion) + mesangial deposit, Hematoxylin bodies of Gross (most specific), Tubuloreticular inclusion
- Gross bodies on e/m: Microscopic image showing gross bodies
- Crescents (RPGN): Microscopic image highlighting crescents
- Full House Pattern: Set of microscopic images displaying different proteins (IgG, IgM, C1, C3, Kappa, Lambda)
- Capillary thickening: Microscopic image highlighting thick capillaries
- Arrow Spikes: Microscopic image with arrows and spikes
- Diffuse Proliferative GN: Microscopic image illustrating widespread cellular proliferation
Other Notes
- Lupus pernio: Sarcoidosis
- Lupus vulgaris: TB
- Monoarthritis, Crystal arthropathy, Septic arthritis are complications of SLE.
- Rash involving nasolabial fold: Acne rosacea
- CCLE = Discoid Rash: 5/20 rule.
- Drugs causing SLE are safe for lupus patients.
Studying That Suits You
Use AI to generate personalized quizzes and flashcards to suit your learning preferences.
Description
This quiz covers the management strategies for Systemic Lupus Erythematosus (SLE) and Antiphospholipid Syndrome (APS). It includes treatment options based on disease severity, antibody testing specifics, and complications associated with SLE. Understand the important clinical distinctions and management approaches involved in SLE treatment.
