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Rheumatology > SLE > Flashcards

SLE Flashcards

1
Q

What year did the ACR/EULAR produce revised SLE classification guidelines?

A

2019

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2
Q

What year were the original SLE ACR/EULAR guidelines published?

A

1997

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3
Q

How are SLE cutaneous findings classified?

A
  1. ACLE - acute cutaneous lupus erythematosus
  2. SCLE - subacute cutaneous lupus erythematosus
  3. CCLE - chronic cutaneous lupus erythematosus - includes discoid lupus (DLE)
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4
Q

What ethnicities is SLE more prevalent in?

A

Asians, African Americans African Carribeans and Hispanic Americans have higher rates of SLE compared to Caucasians.

(NB: Lupus is thought to be rare in Africa)

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5
Q

What is the concordance of SLE between monozygotic twins?

A

High (85.7)

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6
Q

What are the genetic associations in SLE?

A

Over 40 loci have been associated with SLE as identified by genome-wide association studies. They affect lymphocyte signalling, interferon (IFN) signalling, clearance of immune complexes and products of apoptosis. Each gene contributes a relative risk of less than 2 and can be associated with multiple autoimmune diseases. These include:
1) Deficiencies of the complement component C1q, C4A and C4B and C2;
2) Three prime repair exonuclease 1 (TREX1) mutations (enzyme needed to degrade DNA)
3) HLA-DR2 and HLA-DR3
4) Genes involved with high levels or enhanced responsiveness to IFN-a (such as STAT4, PTPN22, IRF5) .

There is also dysregulation in epigenetic factors including DNA methylation and expression changes in various microRNAs (miRNAs).

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7
Q

What is the relative risk of SLE in patients on oestrogen-containing contraception?

A

Relative risk of 1.5

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8
Q

What is the relative risk of SLE in post-menopausal administration of oestrogen and early menarche (age </= 10yrs)?

A

Post-menopausal administration of oestrogen and early menarche (age </= 10yrs) double the risk of SLE development.

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9
Q

What are some examples of environmental triggers for SLE?

A

1) VIRUSES activate the type 1 IFN pathway, an important anti-viral immune mechanism. This is the same pathway thought to be critical in promoting SLE activity. Recurrent EBV and CMV infections have been associated with a higher SLE development.

2) Patients with SLE have higher serum levels of lipopolysacharide (LPS), a cell wall component of Gram-negative bacteria.

3) The microbiome seems to be implicated as well - women with SLE have a lower Firmicutes to Bacteroidetes ratio in their gut. In addition, recent data suggest Enterococcus gallinarum that have translocated through the gut epithelium may be associated with SLE.

4) Ultraviolet light exposure commonly triggers cutaneous manifestations of SLE, likely through upregulation of IFN-K.

5) Several drugs are associated with DIL.

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10
Q

What drugs are associated with drug-induced lupus (DIL)?

A

DEFINITE: procainamide, hydralazine, minocycline, diltiazem, penicillamine, isoniazid, quinidine, anti-TNF-a therapies, IFN-a, methyldopa, chlorpromazine, practolol.

PROBABLE: anticonvulsants, phenytoin, ethosuximide, carbamazepine, antithyroid drugs, antimicrobial agents, sulphonamides, rifampin, nitrofurantoin, B-blockers, lithium, captopril, IFN-y, hydrochlorothiazide, glyburide, sulfasalazine, terbinafine, amiodarone, ticlopidine, docetaxel.

POSSIBLE: Gold salts, penicillin, tetracycline, reserpine, valproate, statins, griseofulvin, gemfibrozil, valproate, ophthalmic timolol, 5-aminosalicylate.

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11
Q

What other conditions can overlap with SLE?

A

SLE can overlap with signs and symptoms from other connective tissue diseases. For example, SLE can overlap with:
1. RA (“rhupus”)
2. Primary Sjogren’s syndrome
3. Raynaud’s disease
4. Systemic sclerosis
5. Polymyositis/dermatomyositis

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12
Q

What is the classic presentation of arthritis and arthralgia in SLE?

A

Arthralgia may involve any joint, but symmetric involvement of the hands, wrists and knees is more typically seen. Arthritis tends to be symmetric, migratory and nonerosive. Although deformities are rare, most deformities associated with SLE are reversible. Both reversible “swan-neck” and “boutonniere” deformities can be seen. Jaccoud’s arthropathy occurs when there is reversible ulnar deviation and subluxation of the second to fifth metocarpophalangeal (MCP) joints.

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13
Q

What is the most common presenting symptom in SLE?

A

Arthritis and arthralgia

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14
Q

Can SLE involve the muscles?

A

Diffuse myalgia are commonly seen in SLE, sometimes in association with fibromyalgia. Myositis is uncommon, but muscle inflammation otherwise typical of primary polymyositis/dermatomyositis may be associated with SLE. Myopathy induced from steroids or hydroxychloroquine (HCQ) can be seen.

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15
Q

Which subtype of cutaneous lupus are most strongly associated with SLE (systemic lupus)?

A

ACLE - >90% of patients with ACLE have SLE. The most common presentation is the malaria rash, which is present in 50% of patients with SLE.

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16
Q

What are some differentials for malar rash?

A

1) Acne rosacea
2) Seborrhoeic dermatitis
3) Polymorphous light eruption
4) Contact dermatitis

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17
Q

What percentage of patients with subacute cutaneous lupus erythematous (SCLE) have SLE?

Conversely, what percentage of SLE patients have SCLE?

A

Halft (50%) of SCLE patients have SLE.

10% of SLE patients have SCLE.

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18
Q

Many patients with SCLE will have what antibody?

A

SSA (Sjogren’s syndrome-related antigen).

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19
Q

What is the most common for of chronic cutaneous lupus erythematosis?

A

Discoid lupus

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20
Q

Describe what discoid lupus looks like?

A

The rash starts as erythematous papule or plaques that become infiltrated and have an adherent scale. Follicular plugging is prominent. The lesions expand, leaving central hypo pigmentation, atrophic scarring and permanent alopecia. They commonly occur on the scalp, along the hair-line and within the conchal bowls.

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21
Q

What percentage of patients with discoid lupus develop SLE?

A

Only 10% of patients with discoid lupus will develop SLE; however, the presence of disseminated DLE lesions, including those that occur below the head and neck area, is associated with increased likelihood of developing SLE.

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22
Q

What are some examples of nonspecific cutaneous eruptions in patients with SLE?

A

Nonspecific cutaneous eruptions are very common in patients with SLE and include non-scarring alopecia, livedo reticularis and vasculitis. Soft-tissue swelling and erythema can also occur in between the joints of the fingers (compared to Gottron’s papule seen in dermatomyositis).

23
Q

Renal involvement occurs in what percentage of SLE patients?

A

Renal involvement occurs in over 50% of SLE patients, and is a major cause of morbidity and mortality in SLE patients. Findings range from asymptomatic proteinuria to frank nephritic and nephrotic syndrome.

24
Q

What are the different classes of lupus nephritis?

A

Class I - Minimal mesangial lupus nephritis (MMLN)

Class II - Mesangial proliferative lupus nephritis (MPLN)

Class III - Focal lupus nephritis (FLN)

Class IV - Diffuse lupus nephritis (DLN)

Class V - Membraneous lupus nephtirits (MLN)*

Class VI - Advanced sclerotic lupus nephritis

NB: Class V may occur in combination with class III or IV, in which case both will be diagnosed.

25
Q

Although the classification of lupus nephritis is based on glomerular involvement, what other portions of the kidneys can be affected?

A

Tubulointerstitial nephritis is often seen in lupus nephritis and is associated with progressive clinical course, including the development of worsening creatinine and hypertension.

Vascular disease can present as an immune complex deposition within the endothelium of the vessel, immunoglobulin microvascular casts, vasculitis or a thrombotic microangiopathy similar to TTP or aHUS. Necrotising vasculoopathy is a poor prognostic sign.

26
Q

What are other causes of worsening renal function in SLE? When should a renal biopsy be performed?

A

Renal biopsies should be performed when renal function is worsening, urinary sediments are seen or when the clinical pictures is unclear.

Other causes of worsening renal function in SLE patients include NSAID toxicity, uncontrolled HTN and TTP.

27
Q

In patients who have allogenic renal transplant for lupus nephritis, what is the recurrence rate of nephritis?

A

Recurrence of nephritis in allograft transplantation occurs in 3.8% to 44% of patients.

28
Q

What are common gastrointestinal (GI) signs and symptoms in SLE?

A

Anorexia, nausea or vomiting.

29
Q

What medications used in SLE can cause pancreatitis?

A

1) NSAIDs
2) Azathioprine (AZA)
3) Glucocorticoids
4) Mycophenolate

30
Q

Does SLE cause LFT derangement?

A

Abnormal hepatic function tests can be seen, particularly in the setting of concurrent NSAID use. Frank autoimmune hepatitis is rare in the setting of SLE.

31
Q

What can cause abdominal pain in a patient with SLE?

A

1) Infection, thrombosis (sometimes associated with anti-phospholipid syndrome [APLS]), medication side effects, appendicitis, peptic ulcer disease (from steroids or NSAIDs) and gastroenteritis are the most common causes. Consider CMV infection in patients on immunosuppression with abdominal pain and GI bleeding.

2) Abdominal pain due to SLE is usually from peritoneal inflammation, though this is much less common than pleural inflammation.

3) Less commonly, mesenteric vasculitis can occur. This can present with lower abdominal pain. Symptoms can be mild in nature because of the inflammatory blunting associated with immunosuppressive medications. However, these patients can rapidly associated with immunosuppressive medications. However, these patients can rapidly deteriorate and end up with bowel infarction, perforation and peritonitis. Abdominal CT with arteriogram may be necessary to differentiate from more benign causes of abdominal pain. High-dose steroid use may mask serious abdominal conditions.

32
Q

What is the most common pulmonary symptom in SLE?

A

Pleuritic chest pain is the most common pulmonary symptom. This may be due to costochondritis and can be distinguished from pleuritic by palpating the painful area.

33
Q

What is the most common pleural sign in SLE?

A

The most common sign is pleural effusion and is usually associated with pleuritic. Most pleural effusions are asymptomatic. Pleural fluid is typically exudative with 3000 to 50000 WBC, normal glucose, decreased complement levels and positive ANA.

34
Q

Aside from pleuritis and pleural effusion, what are some less common pulmonary manifestations in SLE (<10%)?

A

Acute pneumonitis and alveolar haemorrhage can present identically. Fever, cough, dyspnoea, pleurisy and late inspiratory crackles occur in both settings. CT has a ground-glass appearance, which is a nonspecific finding. Bronchoscopy may be useful for directly observing blood on repeated bronchoalveolar lavage, associated with alveolar haemorrhage.

35
Q

What subset of patients with SLE are more likely to get ILD?

A

The prevalence of ILD is increased in those who were diagnosed with SLE as a child and now are in their 20s and 30s. These patients will have a restrictive pattern on PFTs.

36
Q

What is shrinking lung syndrome in SLE?

A

Shrinking lung syndrome is rare and characterised by dyspnoea, episodic pleuritic chest pain and progressive reduction in lung volume in the absence of interstitial fibrosis or pleural disease on CT.

37
Q

What is the most frequent cause of symptomatic cardiac disease in patients with SLE?

A

Pericardial disease is the most frequent cause of symptomatic cardiac disease in patients with SLE. Typically presents with positional substernal chest pain that may be associated with an audible rub on auscultation. Often co-presents with other serositis symptoms (pleuritic and pleural effusion). Echocardiogram usually reveals a pericardial effusion that is not haemodynaically compromising. Pericardial effusion is one of the most common ECG findings and may occur in asymptomatic patients.

38
Q

Is pericardial tamponade a common presenting manifestation of SLE?

A

No - it is very unusual for pericardial tamponade to be a presenting manifestation of SLE. Purulent, neoplastic or TB causes should be considered.

39
Q

Is myocarditis common in SLE?

A

Myocarditis is an uncommon asymptomatic manifestation of SLE usually seen with pericarditis. The most typical presentation is a resting tachycardia out of proportion to temperature with nonspecific ECG findings and cardiomegaly. Global hypokninesis one ECG is a common finding.

40
Q

Is coronary artery disease (CAD) a common cardiac manifestation in SLE?

A

CAD is a common cardiac manifestations in SLE patients and most often due to accelerated atherosclerosis, not coronary artery vasculitis.

Young women with SLE have have up to a 55-fold increase in the risk of CAD compared to their nonlupus cohort. Suspicion for CAD must be high in any SLE patient, even in young females.

41
Q

Systolic murmurs are common, occurring in up to 40% of SLE patients. What can cause systolic murmurs in SLE?

A

Causes of systolic murmurs in SLE include:

1) Functional murmurs from anaemia, fever, tachycardia or cardiomegaly; but structural valvular disease is the most common. Valve thickening can be seen in 70% of patients on transoesophageal echocardiogram (TTE).

2) Mitral valve involvement, particularly mortal valve prolapse, is the most common valvular ethology of systolic murmur.

42
Q

What are patients with SLE-valvular disease at increase risk of?

A

Valvular disease in SLE patients confers an increased risk of serious complications from cerebrovascular disease, peripheral embolism, heart failure and infectious endocarditis.

43
Q

What is Libman-Sacks endocarditis?

A

Libman-Sacks endocarditis are verrucous lesions found near the edge of the mitral, aortic or tricuspid valves and can lead to regurgitation in severe cases. Lesions are usually asymptomatic but can break off producing systemic emboli. These lesions are sterile, unlike in bacterial endocarditis. However, the risk of infective endocarditis is also increased.

44
Q

Does the development of valvular lesions in SLE correlate with disease activity?

A

No

45
Q

What is Raynaud’s phenomenon and is it common in SLE?

A

Yes, Raynaud’s phenomenon is common and manifests in the hands, feet, ears, nipples or nose. Colour changes can be induced by cold temperatures or stress. Two colour changes (red, white or blue) must be reported to confirm the diagnosis.

46
Q

In mothers with positive anti-SSA (Ro) antibodies, what is the risk of the foetus developing neonatal lupus with congenital heart block?

A

1% to 7.5%

47
Q

CNS lupus patients may have which autoantibody?

A

Antiribosomal P protein antibody

48
Q

What is the most common psychiatric symptom seen in SLE patients?

A

Depression

49
Q

When do most acute psychiatric disturbances in SLE occur?

A

Within the first 2 years of diagnosis.

50
Q

How should you approach the management of SLE-associated psychosis?

A

SLE-associated psychosis is a medical emergency. Treatment with antipsychotics and pulse-dose glucocorticoids must be started as soon as possible to prevent permanent brain damage.

51
Q

What are some neuropsychiatric manifestations of SLE?

A

CENTRAL:
- Headache
- Cognitive dysfunction
- Mood disorders
- Seizure disorders
- Cerebrovascular disease
- Anxiety disorder
- Acute confusional state
- Psychosis
- Myelopathy
- Aseptic meningitis
- Movement disorder
- Demyelinating syndrome

PERIPHERAL:
- Polyneuropathy
- Cranial neuropathy
- Mononeuropathy
- Acute inflammatory demyelinating polyradiculoneuropathy
- Autonomic disorder
- Myasthenia gravis
- Plexopathy

52
Q

What percentage of SLE patients have stroke?

A

3% to 20% of SLE patients

53
Q

What percentage of SLE patients have seizures?

What autoantibodies are seizures in SLE associated with?

A

6% to 51% of SLE patients have seizures.

Seizures in SLE are associated with antiphospholipid and anti-Smith (anti-Sm) antibodies.

Rheumatology (5 decks)

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