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SLE Flashcards

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1
Q

What type of hypersensitivity reaction is SLE classified as?

A

Type 3 hypersensitivity affecting females aged 15-40 years old and Afro-Carribean heritage.

SLE primarily affects females aged 15-40 years old and those of Afro-Caribbean heritage.

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2
Q

Name four environmental risk factors for SLE.

A
  • Smoking
  • Ultraviolet light
  • Silica exposure
  • Epstein-Barr virus

These factors can contribute to the onset of SLE.

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3
Q

What are some triggers for flares of SLE?

A
  • Oestrogen exposure from pregnancy and the combined oral contraceptive
  • Infections
  • Emotional stress
  • Physical stress e.g surery or injury
  • Excessive ultraviolet light exposure

These triggers can exacerbate the condition.

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4
Q

What is the classification of SLE?

A

Classification of SLE is based on the EULAR/ACR criteria for patients requiring positive antinuclear antibodies an a score of 10 or more with at least one crtieria

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5
Q

What are the criteria domains for EULAR-ACR in SLE?

A

Haemotological
Neuropsychiatric
Mucocutaneous

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6
Q

According to the EULAR/ACR criteria, how many points are needed for a positive diagnosis of SLE?

A

10 or more points

At least one criterion must be met, including positive antinuclear antibodies.

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7
Q

What is the neuropsychiatric domain of the EULAR-ACR?

A

-> delirium is 2 points
-> psychosis is 3 points
-> seizure is 5 points

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8
Q

What is the mucocutaneous domain of the EULAR-ACR?

A

-> non-scarring alopecia is 2 points
-> oral ulcers is 2 points

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9
Q

What is the immunological domain of SLE?

A

-> antiphospholippd antibodies present being anti-cardiolipin, glycoprotein I or lupus anticoagulant= 2 pints
-> complement proteins with either low C3 OR low C4= 3 points

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10
Q

What is the renal domain for SLE?

A

Proteinuria over 0.5g in 24 hours = 4 points
-> class II or V lupus nephritis on biopsy = 8 points
-> class III or IV lupus nephritis on biopsy= 10 points

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11
Q

What are the SLE specific antibodies for the EULAR-ACR domain?

A

Low C3 AND low C4= 4 points
-> anti-dsDNA or anti-smith antibodies= 4 points

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12
Q

What is the presentation of a malar rash in SLE?

A

Butterfly appearance across the face, SPARING the nasal folds

The rash may be flat or raised.

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13
Q

What are the dermatological manifestations of SLE?

A

*malar rash
*Discoid rash: disk-shaped rash that is reddened and raised in sun exposed areas. They can become pigmented and hyperkeratotic before atrophy.
*Livedo reticularis: net-like reddish blue discolouration of skin resembling vessels due to disrupted blood flow in dermal arteries.
*Alopecia: hair loss that is patchy, linked to discoid lesions and scarring.
*Cutaneous vasculitis with splinter haemorrhages/purpura
Photosensitivity, causing rash

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14
Q

List two haematological features of SLE and their corresponding points.

A
  • Leukopenia: 3 points
  • Thrombocytopenia: 4 points
  • Autoimmune Hemolysis: 4 points

These features contribute to the scoring system for diagnosis.

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15
Q

What is Raynaud’s phenomenon associated with in SLE?

A

Vasospasm leading to color changes in fingers and toes

It is a common symptom in SLE patients.

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16
Q

What kind of arthritis is seen in SLE?

A

Polyarthritis with symmetrical distribution: must be minmum of 2 joints affected but at least 5 are typically affected. Both large and small joints are affected; it is a non-erosive arthritis with swelling, joint effusion and tenderness worse in the morning and better throughout the day

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17
Q

What is SLE associated with systemically?

A

*Pericarditis and myocarditis
* Endocarditis
* cerebritis, causing encephalopathy, seizures, psychosis and coma and decreased concentration
* lupus nephritis
* pregnancy loss due to hypercoagulability

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18
Q

True or false: Patients with SLE are predisposed to pericarditis and myocarditis.

A

TRUE

These conditions can lead to arrhythmias and heart failure.

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19
Q

What is the most common cardiac manifestation of SLE?

A

Pericarditis

It can lead to significant complications if not managed properly.

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20
Q

What are the cardiac complications with SLE?

A

Libman-Sacks endocarditis, formation of non-infective vegetations that usually form on the mitral and aortic valves

Pericarditis

Myocarditis

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21
Q

How does lupus manifest in the kidneys?

A

Renal failure due to lupus nephritis typically in the capillary walls that is progressive. Patients will typically present with nephrotic syndrome, diagnosed with Kidney biopsy showing crescent-shaped swelling in Bowman’s space or wire-loop pattern in basement membrane.

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22
Q

What is the management of lupus nephritis?

A

Treatment is with corticosteroids and immunosuppressants such as mycophpenelate and cyclophosphamide

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23
Q

Name two antibodies involved in SLE and their specificity.

A
  • anti-nuclear antibody
  • Anti-dsDNA: highly specific (> 99%)
  • Anti-Smith: highly specific (> 99%)

Anti-dsDNA is associated with more severe manifestations like nephritis.

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24
Q

What is anti-smith antibody targeted against?

A

against protein complex in cell nucleus for mRNA processing. It is associated with more severe manifestations like nephritis and vasculitis in lupus

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25
Which antibody in SLE is linked to other connective tissue diseases?
anti-U1 RNP, associated with systemic sclerosis, lupus and Sjögren’s syndrome
26
Which antibody in SLE is associated with Sjögren’s syndrome?
anti-U1 Anti-Ro Anti-La
27
What does anti-La an indicator for?
Found in primary Sjögren’s syndrome. It indicates neonatal lupus, and can cause complete congenital heart block in foetus.
28
What are the **immunological markers** used in SLE diagnosis?
* Antinuclear antibodies (ANA) * Anti-dsDNA antibodies * Anti-Smith antibodies * Antiphospholipid antibodies ## Footnote These markers help in confirming the diagnosis and assessing disease activity.
29
What is the **key diagnostic test** for lupus nephritis?
Renal biopsy ## Footnote It is crucial for classification and management of the disease.
30
What are complement levels in SLE associated with?
complement levels (C3, C4) are low during active disease (formation of complexes leads to consumption of complement)
31
What joint complication can be seen in SLE?
Patients may develop Jaccoud’s Arthropathy, which is a deforming non-erosive arthropathy characterised by ulnar deviation of the second to fifth fingers with metacarpophalangeal joint subluxation. It can mimic rheumatoid arthritis deformities
32
What can SLE be used for in disease. Monitoring?
anti-double stranded DNA titres
33
What are the pulmonary manifestations of SLE?
Patients with antiphospholipid antibodies are at increased risk of pulmonary emboli * Rarer pulmonary manifestations include: * Acute lupus pneumonitis * Fibrosing alvoelitis with scar tissue formation in lungs * Pulmonary hypertension * Chronic interstitial pneumonitis * Obliterative bronchiolitis * Pulmonary vasculitis * Alveolar haemorrhage
34
Why is there a stroke risk in SLE?
There is a significantly increased risk of stroke, which is contributed to by: * Antiphospholipid syndrome * Hypertension * Accelerated atherosclerosis * Embolisms from Libman-Sacks endocarditis * Thrombocytopaenia * Cerebral vasculitis
35
What are the neurological complications of SLE?
Psychosis Delirium Cognitive impairment Depression and anxiety Persistent headaches
36
What are the GI manifestations of SLE?
Mouth ulcers are common and may be painful * A protein-losing enteropathy may be seen * Ascites may occur secondary to peritonitis or hypoalbuminaemia due to enteropathy or nephrotic syndrome * Pancreatitis * Splenic infarction * Hypoadrenalism * Hepatomegaly, hepatitis or cirrhosis may be seen * Vasculitis affecting the GI tract may cause colitis, mucosal ulceration, mesenteric ischaemia or oesophageal dysmotility * Antiphospholipid syndrome may cause Budd-Chiari syndrome or thrombosis of intestinal vessels
37
What is the recommended **first-line treatment** for all patients with SLE?
Hydroxychloroquine ## Footnote It helps manage symptoms and prevent flares.
38
List three **complications** of chronic steroid treatment in SLE patients.
* Osteonecrosis * Insufficiency fractures * Tendinopathies ## Footnote Long-term steroid use can lead to significant side effects.
39
What are the **neurological manifestations** present in SLE?
* Seizures * Psychosis * Cognitive impairment ## Footnote Neurological symptoms are present in 80% of patients with SLE.
40
What is the **differential diagnosis** for drug-induced lupus?
* Procainamide * Hydralazine * Isoniazid * Quinidine * Methyldopa ## Footnote Symptoms improve after withdrawal of the causative drug.
41
What are the **bedside tests** recommended for monitoring SLE?
* Blood pressure For hypertension * Urine dip for proteinuria or haematuria * Urinary protein:creatinine ratio ## Footnote These tests help in assessing renal involvement and hypertension.
42
What are the blood tests for SLE?
Full blood count looking for anaemia, leukopenia and thrombocytopaenia * U&Es to screen for renal impairment due to lupus nephritis * Liver function tests are usually normal; hypoalbuminaemiamay be seen due to GI or renal involvement * ESR is usually high; CRP may be normal or mildly raised * Clotting screen may show paradoxical prolongation of the APTT if there are anti-phospholipid antibodies present *Antinuclear antibodies, anti DSDNA and anti-sm antibodies
43
What is the imaging for SLE?
Chest X-ray in all patients as a baseline and to screen for pulmonary involvement * CT chest may be required for more detailed assessment of lung involvement; CT pulmonary angiography is required in suspected pulmonary embolism * Ultrasound of the kidney, ureters and bladder (KUB) to exclude other causes of renal impairment e.g. obstruction * X-rays of affected joints looking for arthritis and fractures * Joint space narrowing is uncommon * Soft tissue swelling and periarticular osteoporosis may be seen Echocardiogram may show pericarditis, heart failure, pericardial effusion or pulmonary hypertension
44
What is the conservative management of SLE?
*Vitamin D supplements *Advise on sun protection e.g. using sunscreen, limiting exposure and covering skin *Avoid triggers such as oestrogen-containing contraceptives * Optimise cardiovascular health with regular exercise, smoking cessation and a balanced diet * Ensure patients are up to date with vaccinations
45
What is the **role of vitamin D** in SLE management?
Supplementation may be required due to sun avoidance ## Footnote Patients with SLE are at increased risk of vitamin D deficiency.
46
What is commonly used for **lupus nephritis**?
* MMF * Azathioprine ## Footnote Most patients continue on either MMF or azathioprine for treatment.
47
What additional management may be required if **anti-phospholipid syndrome** is present?
Additional management ## Footnote Refer to a separate chapter for details on managing anti-phospholipid syndrome.
48
What **cardiovascular risk factors** may require medical treatment in lupus patients?
* Hypertension * Hyperlipidaemia ## Footnote These factors are important to manage in patients with lupus.
49
What supplementation may be required for patients on long-term **steroids**?
* Vitamin D * Bone protection ## Footnote Long-term steroid use can lead to complications that necessitate these supplements.
50
What is the main issue with **hydroxychloroquine**?
Retinal toxicity ## Footnote All patients on hydroxychloroquine should undergo regular eye screening.
51
Patients on **immunosuppressive treatments** are at increased risk of what?
* Opportunistic infections * Reactivation of latent infections ## Footnote Screening for latent infections like tuberculosis and hepatitis is essential before treatment.
52
What is overlap syndrome?
Overlap syndromes may develop in patients with SLE (where the criteria for multiple rheumatological diseases are met), for example *Sjogren's syndrome *Antiphospholipid syndrome * Mixed connective tissue disease
53
How can lupus be inherited in pregnancy?
Mixed connective tissue disease Neonatal lupus is a complication that arises in babies born to mothers with anti-Ro and/or anti-La antibodies These antibodies cross the placenta and cause a transient autoimmune syndrome Manifestations include a red rash on the scalp and around the eyes, complete heart block, deranged liver function and cytopaenias
54
What percentage of patients with **lupus nephritis** develop end-stage renal failure?
Approximately 20% ## Footnote This may require renal replacement therapy, either with dialysis or transplant.
55
What complications can arise from **accelerated atherosclerosis** in lupus patients?
* Myocardial infarction * Ischaemic stroke * Peripheral vascular disease ## Footnote These complications increase the cardiovascular risk in lupus patients.
56
What are some **overlap syndromes** that may develop in patients with SLE?
* Sjogren's syndrome * Antiphospholipid syndrome * Mixed connective tissue disease ## Footnote These syndromes meet the criteria for multiple rheumatological diseases.
57
What is **neonatal lupus**?
A complication in babies born to mothers with anti-Ro and/or anti-La antibodies ## Footnote These antibodies can cross the placenta and cause a transient autoimmune syndrome.
58
What are some **manifestations** of neonatal lupus?
* Red rash on the scalp * Rash around the eyes * Complete heart block * Deranged liver function * Cytopaenias ## Footnote These symptoms arise due to maternal antibodies affecting the newborn.
59
What are some **pregnancy complications** associated with lupus?
* Recurrent miscarriage * Pre-eclampsia * Intrauterine growth restriction * Preterm delivery ## Footnote These complications can occur in pregnancies involving mothers with lupus.
60
Patients with SLE have a **how much** increased mortality compared to the general population?
2.6x increased mortality ## Footnote This statistic highlights the serious impact of SLE on life expectancy.
61
What is the average **life expectancy** for patients with SLE?
54 years ## Footnote Although death from active lupus is rare in the UK, life expectancy is significantly shortened.
62
What is the mean age of death for patients who develop **lupus nephritis**?
40 years ## Footnote This condition is associated with a poorer prognosis.
63
What are some **poor prognostic factors** for SLE?
* Black ethnicity * Male sex * Antiphospholipid antibodies * Central nervous system involvement ## Footnote These factors are associated with a worse prognosis in SLE patients.
64
Which drug can induce lupus?
Isoniazid which causes fever, myalgia, fatigue and joint pain
65
Which antibody is present in drug induced lupus?
Positive anti-his tone antibody
66
Which dru can induce lupus?
Isoniazid, associated with anti-his tone antibodies
67
How does SLE present in utero?
Foetal bradycardia between 18 to 28 weeks gestation
68
What is seen on renal biosp in lupus?
full house' pattern refers to glomerular deposits that stain dominantly for IgG, IgA, IgM, C3 and C1q- five major stains on a renal biopsy are all positive
69
Which HLA-subtypes are associated with SLE?
HLA DR2 and 3
70
What protein deficiency is assoicated with SLE?
Early complement proteins This leads to impaired the clearance of Ag/Ab immune complexes
71
What is the biggest trigger for SLE?
UV light exposure
72
Which drug is the most common cause of drug-induced SLE?
Hydralazine which is used for hypertensive crisis & hypertension
73
What is the timecourse for drug induced SLE?
Procainamide which’s tarts 1 month after medication initiation
74
Which distal phenomenon is assoicated with SLE?
Raynaud’s
75
What is the mnemonic for SLE?
RASH OR PAIN *Rash (malar or discoid) *Arthritis (usually involving ≥ 2 joints) *Serositis (pleuritis and pericarditis) *Hematologic disorders (e.g. cytopaenias) *Oral or nasopharyngeal ulcers (usually painless) *Renal disease *Photosensitivity *Antinuclear antibodies *Immunologic disorder (anti-dsDNA, anti-Sm, antiphospholipid) *Neurologic disorder (seizures, psychosis)
76
How much criteria must be fulfilled for SLE?
4 out of 11
77
Which rashes don’t spare the nasolabial folds?
dermatomyositis rash & rosacea
78
Which type of nephropathy occurs in SLE?
Diffuse proliferative glomerulonephritis
79
80
How to differentiate LIbman-Sacks endocarditis from bacterial endocarditis?
Libman-Sacks endocarditis is a sterile Non-infectious endocarditis that affects both sides of the given valve
81
What are the top causes of death in SLE?
1) cardiovascular disease 2) infection (cytopaenias & immunosuppressive therapy) 3) renal disease 4) neurological (seizures)
82
Which antibody is sensitive for SLE?
ANA
83
Which antibody is specifici for SLE?
anti-dsDNA (associated with lupus nephritis activity) & anti-Smith
84
What antibody should be screened in lupus?
Antiphospholipid syndrome
85
What antibody test rules out lupus?
Negative ANA
86
What should patients taking hydroxychlouoquine be monitored for?
Retinal toxicity which causes bell’s eye maculoapthy
87
What is important primary care anagement for SLE?
Vaccination with pneumococcal and influenza
88
What is given for glomerulonephritis flares?
IV cyclophosphamide and steroids
89
What is sensitive for SLE?
ANA= seNsitive
90
What is specific for SLE?
Anti-dsDNA= specific
91
How are inflammatory markers change in SLE?
NORMAL CRP but raised ESR
92
What does raised CRP and myalgia in patient with lupus indicate?
Underlying infection
93
94
Which test is useful to rule out for lupus?
AN which is positive in over 99% of patients
95
What marker is used to monitor flares?
Complement levels
96
Which drug causes SLE?
procainamide hydralazine
97
What are less common drug causes of lupus?
*isoniazid *minocycline *phenytoin Progressive weakness, Raised proteinia
98
What is a common blood test findings in lupus?
Lymphophenia which is a useful diagnosing clue
99
How does paltelet count change in SLE?
Thrombocytopenia due to one marrow suppression
100
Which inflammatory conditions have thrombocytosis?
Rheumatoid arthritis IBS Reactive process to infection or malignancy
101
How to monitor flares in lupus?
Low levels of C3 and C4
102
What are the tests for EXCLUDING lupus?
ANA
103
What test strongly indicates lupus?
Anti ds-DNA
104
What is **discoid lupus erythematosus** characterized by?
Follicular keratin plugs ## Footnote It is thought to be autoimmune in aetiology.
105
True or false: Discoid lupus erythematosus very rarely progresses to **systemic lupus erythematosus**.
TRUE ## Footnote Progression occurs in less than 5% of cases.
106
List the **features** of discoid lupus erythematosus.
* Erythematous, raised rash, sometimes scaly * May be photosensitive * More common on face, neck, ears, and scalp * Lesions heal with atrophy, scarring, and pigmentation ## Footnote Scarring may cause scarring alopecia.
107
What is the first-line **management** for discoid lupus erythematosus?
Topical steroid cream ## Footnote Oral antimalarials, such as hydroxychloroquine, may be used as a second-line treatment.
108
Fill in the blank: Discoid lupus erythematosus is more common in _______.
younger females ## Footnote This condition is generally benign.
109
What should be avoided to manage discoid lupus erythematosus?
Sun exposure ## Footnote Avoiding sun exposure is crucial for management.
110
What is **discoid lupus erythematosus** characterized by?
Follicular keratin plugs ## Footnote It is thought to be autoimmune in aetiology.
110
What is **discoid lupus erythematosus** characterized by?
Follicular keratin plugs ## Footnote It is thought to be autoimmune in aetiology.
111
True or false: Discoid lupus erythematosus very rarely progresses to **systemic lupus erythematosus**.
TRUE ## Footnote Progression occurs in less than 5% of cases.
111
True or false: Discoid lupus erythematosus very rarely progresses to **systemic lupus erythematosus**.
TRUE ## Footnote Progression occurs in less than 5% of cases.
112
List the **features** of discoid lupus erythematosus.
* Erythematous, raised rash, sometimes scaly * May be photosensitive * More common on face, neck, ears, and scalp * Lesions heal with atrophy, scarring, and pigmentation ## Footnote Scarring may cause scarring alopecia.
112
List the **features** of discoid lupus erythematosus.
* Erythematous, raised rash, sometimes scaly * May be photosensitive * More common on face, neck, ears, and scalp * Lesions heal with atrophy, scarring, and pigmentation ## Footnote Scarring may cause scarring alopecia.
113
What is the first-line **management** for discoid lupus erythematosus?
Topical steroid cream ## Footnote Oral antimalarials, such as hydroxychloroquine, may be used as a second-line treatment.
113
What is the first-line **management** for discoid lupus erythematosus?
Topical steroid cream ## Footnote Oral antimalarials, such as hydroxychloroquine, may be used as a second-line treatment.
114
Fill in the blank: Discoid lupus erythematosus is more common in _______.
younger females ## Footnote This condition is generally benign.
114
Fill in the blank: Discoid lupus erythematosus is more common in _______.
younger females ## Footnote This condition is generally benign.
115
What should be avoided to manage discoid lupus erythematosus?
Sun exposure ## Footnote Avoiding sun exposure is crucial for management.
115
What should be avoided to manage discoid lupus erythematosus?
Sun exposure ## Footnote Avoiding sun exposure is crucial for management.
116
Which inflammatory marker is normal in SLE?
CRP
123
Which stroke type is linked to prothrombotic state?
Venous sinus thrombosis
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