Rheumatology > Lupus > Flashcards

Lupus Flashcards

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1
Q

What is the hallmark feature that differentiates acute cutaneous lupus erythematosus (ACLE)?

A

Bright red malar rash across cheeks + nasal bridge that spares nasolabial folds; non-scarring and strongly associated with systemic lupus activity.

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2
Q

Which autoantibody is most strongly associated with subacute CLE (SCLE)?

A

Anti-Ro/SSA (often with anti-La/SSB).

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3
Q

How do SCLE lesions differ from discoid lupus (DLE) lesions?

A

SCLE is non-scarring, annular or psoriasiform, photosensitive; DLE is scarring with follicular plugging, atrophy, dyspigmentation, and alopecia.

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4
Q

What is the classic histological feature of discoid lupus erythematosus?

A

Hyperkeratosis with follicular plugging, basement membrane thickening, dermal fibrosis, pigment incontinence.

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5
Q

Which CLE subtype carries a risk of squamous cell carcinoma (SCC)?

A

Discoid lupus erythematosus (chronic plaques, 2–3% risk).

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6
Q

Which CLE subtype presents with deep firm subcutaneous nodules that heal with atrophy?

A

Lupus panniculitis (profundus).

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7
Q

What is the key distinguishing feature of chilblain lupus compared to perniosis?

A

Persistent acral violaceous lesions beyond cold exposure; associated with anti-Ro antibodies.

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8
Q

What is the hallmark feature of tumid lupus?

A

Smooth, erythematous oedematous plaques with no surface scale or scarring; abundant dermal mucin on histology.

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9
Q

Which CLE subtype is associated with anti–type VII collagen antibodies?

A

Bullous lupus erythematosus.

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10
Q

What is the key neonatal manifestation of maternal anti-Ro/SSA positivity?

A

Neonatal lupus with annular rash and congenital heart block.

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11
Q

Which investigation is most specific for systemic lupus if positive in non-lesional sun-protected skin?

A

Lupus band test (granular Ig/complement deposition at DEJ).

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12
Q

What is the first-line systemic therapy for CLE?

A

Hydroxychloroquine (≤5 mg/kg real body weight/day).

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13
Q

What is the hallmark feature that differentiates acute cutaneous lupus erythematosus (ACLE)?

A

Bright red malar rash across cheeks + nasal bridge that spares nasolabial folds; non-scarring and strongly associated with systemic lupus activity.

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14
Q

Which autoantibody is most strongly associated with subacute CLE (SCLE)?

A

Anti-Ro/SSA (often with anti-La/SSB).

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15
Q

How do SCLE lesions differ from discoid lupus (DLE) lesions?

A

SCLE is non-scarring, annular or psoriasiform, photosensitive; DLE is scarring with follicular plugging, atrophy, dyspigmentation, and alopecia.

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16
Q

What is the classic histological feature of discoid lupus erythematosus?

A

Hyperkeratosis with follicular plugging, basement membrane thickening, dermal fibrosis, pigment incontinence.

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17
Q

Which CLE subtype carries a risk of squamous cell carcinoma (SCC)?

A

Discoid lupus erythematosus (chronic plaques, 2–3% risk).

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18
Q

Which CLE subtype presents with deep firm subcutaneous nodules that heal with atrophy?

A

Lupus panniculitis (profundus).

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19
Q

What is the key distinguishing feature of chilblain lupus compared to perniosis?

A

Persistent acral violaceous lesions beyond cold exposure; associated with anti-Ro antibodies.

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20
Q

What is the hallmark feature of tumid lupus?

A

Smooth, erythematous oedematous plaques with no surface scale or scarring; abundant dermal mucin on histology.

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21
Q

Which CLE subtype is associated with anti–type VII collagen antibodies?

A

Bullous lupus erythematosus.

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22
Q

What is the key neonatal manifestation of maternal anti-Ro/SSA positivity?

A

Neonatal lupus with annular rash and congenital heart block.

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23
Q

Which investigation is most specific for systemic lupus if positive in non-lesional sun-protected skin?

A

Lupus band test (granular Ig/complement deposition at DEJ).

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24
Q

What is the first-line systemic therapy for CLE?

A

Hydroxychloroquine (≤5 mg/kg real body weight/day).

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25
What is the entry criterion for the 2019 EULAR/ACR SLE classification criteria?
ANA ≥1:80 on HEp-2 cells at least once.
26
How many points are required on the 2019 EULAR/ACR SLE classification criteria for a diagnosis?
≥10 points with at least 1 clinical criterion.
27
Name the high-weight criteria in 2019 EULAR/ACR classification relevant for exams.
Renal biopsy class III/IV (10 points), acute cutaneous lupus (6), anti-dsDNA (6), anti-Sm (6), neuro involvement (6).
28
Which antibody is highly specific but insensitive for SLE?
Anti-Sm antibody (specific ~95%, insensitive).
29
Which antibody correlates with lupus nephritis activity?
Anti-dsDNA antibody (specific and tracks renal activity).
30
What complement abnormalities are associated with active lupus?
Low C3 and/or C4 levels (consumption during active disease).
31
What is the typical CRP pattern in a lupus flare?
CRP usually normal; if elevated, think infection or serositis.
32
List the major cutaneous manifestations of SLE.
Malar rash (sparing nasolabial folds), SCLE, DLE, photosensitivity, oral ulcers, non-scarring alopecia.
33
What are the classic musculoskeletal features of SLE?
Non-erosive arthritis/arthralgia; Jaccoud’s arthropathy (reducible deformities).
34
List the renal manifestations of SLE.
Proteinuria, haematuria, lupus nephritis (class I–VI by ISN/RPS biopsy classification).
35
List the neuropsychiatric manifestations of SLE.
Seizures, psychosis, cognitive impairment, mononeuritis multiplex, transverse myelitis.
36
List the cardiopulmonary manifestations of SLE.
Pleuritis, pericarditis, Libman–Sacks endocarditis (sterile vegetations), pulmonary hypertension, accelerated atherosclerosis.
37
List the haematological manifestations of SLE.
Autoimmune haemolytic anaemia, leukopenia, lymphopenia, thrombocytopenia.
38
Which antibodies are associated with obstetric complications in SLE?
Antiphospholipid antibodies (anticardiolipin, lupus anticoagulant, anti-β2GP1).
39
Which antibodies are associated with neonatal lupus and congenital heart block?
Anti-Ro/SSA (± anti-La/SSB).
40
What renal biopsy class is diffuse proliferative lupus nephritis?
Class IV (≥50% glomeruli involved with proliferative/necrotising lesions).
41
What renal biopsy class is membranous lupus nephritis?
Class V (subepithelial immune deposits, nephrotic syndrome).
42
Describe the Euro-Lupus regimen for lupus nephritis induction.
Cyclophosphamide 500 mg IV every 2 weeks ×6 (low cumulative dose).
43
Describe the NIH/high-dose cyclophosphamide regimen.
Cyclophosphamide 0.5–1 g/m² IV monthly ×6 (higher toxicity).
44
What are the options for induction therapy in class III/IV lupus nephritis?
High-dose steroids + mycophenolate mofetil (MMF) OR cyclophosphamide (Euro-Lupus or NIH regimen).
45
What are the options for maintenance therapy in lupus nephritis?
Mycophenolate or azathioprine, with low-dose steroids.
46
What adjunctive measures should be used in lupus nephritis?
ACEi/ARB for proteinuria, statins for dyslipidaemia, SGLT2 inhibitors in CKD, PJP prophylaxis with CYC/steroids.
47
What is the cornerstone therapy for all SLE patients unless contraindicated?
Hydroxychloroquine (≤5 mg/kg real body weight/day).
48
What are the ocular monitoring recommendations for hydroxychloroquine?
Baseline eye exam, then annually after 5 years (earlier if high risk).
49
What is the role of belimumab in SLE?
Anti-BLyS biologic; reduces mucocutaneous/systemic disease; PBS-listed for refractory SLE.
50
What is the role of anifrolumab in SLE?
Anti-interferon receptor biologic; effective for moderate–severe SLE; not PBS-listed in Australia.
51
What is the role of rituximab in SLE?
Anti-CD20; off-label; used for refractory haematological or neuropsychiatric lupus.
52
What medications must be avoided in pregnancy with SLE?
Methotrexate, mycophenolate, cyclophosphamide; use azathioprine and HCQ instead.
53
What is the recommended management of obstetric APS in SLE?
Low-dose aspirin + prophylactic or therapeutic LMWH throughout pregnancy.
54
What monitoring is required for anti-Ro/SSA positive pregnancies?
Serial fetal echocardiography from 16–26 weeks (risk of congenital heart block).
55
What are the major causes of death in SLE?
Active renal/CNS lupus, infection, accelerated cardiovascular disease.
56
What is Libman–Sacks endocarditis?
Sterile verrucous vegetations on heart valves (often mitral/aortic).
57
What are the typical radiographic features of SLE arthritis?
Non-erosive changes; reducible deformities (Jaccoud’s).
58
What malignancy risks are increased in SLE?
Non-Hodgkin lymphoma, cervical dysplasia, skin cancers with chronic CLE.
59
What is the prognosis of SLE in the modern era?
10-year survival >85–90%; main mortality from renal/CNS lupus, infection, and CVD.
60
What is the recurrence risk of congenital heart block in neonatal lupus?
15–20% in subsequent pregnancies.
61
What proportion of SLE patients develop renal involvement?
Up to 50% during disease course.
62
What proportion of patients with DLE develop SLE?
5–20%.
63
What is the approximate systemic progression risk in SCLE?
30–40% (usually mild systemic features).
64
What is cutaneous lupus erythematosus (CLE)?
An autoimmune spectrum of lupus skin disease, which may be confined to skin or part of systemic lupus erythematosus (SLE).
65
What are the three main categories of CLE?
Acute CLE (ACLE), Subacute CLE (SCLE), Chronic CLE (CCLE – discoid, hypertrophic, panniculitis, chilblain, tumid), plus rare forms (bullous, neonatal, overlap).
66
What is the hallmark clinical feature of acute CLE (ACLE)?
Malar rash: bright red plaques across cheeks and nasal bridge, sparing nasolabial folds; non-scarring, minimal scale; strongly linked with systemic lupus flare.
67
What is the typical serology in ACLE?
ANA+, anti-dsDNA+, low complement (C3/C4).
68
What histological features are seen in ACLE?
Interface dermatitis, basal keratinocyte damage, dermal oedema, dermal mucin; lupus band positive on DIF.
69
What are the two morphologies of subacute CLE (SCLE)?
Annular/polycyclic plaques with trailing scale and central clearing OR papulosquamous (psoriasiform) lesions.
70
What are distinguishing features of SCLE compared to DLE?
SCLE is non-scarring, highly photosensitive, annular/psoriasiform, strongly anti-Ro+, often drug-induced; DLE is scarring with follicular plugging, central atrophy, dyspigmentation, alopecia.
71
Which drugs can induce SCLE?
Thiazides, terbinafine, proton pump inhibitors, TNF inhibitors.
72
What proportion of SCLE progresses to systemic lupus?
Around 30–40%, usually mild systemic involvement.
73
What is the classic morphology of discoid lupus erythematosus (DLE)?
Coin-shaped erythematous plaques with adherent scale and follicular plugging (‘carpet tack’), central atrophy and scarring, dyspigmentation, scarring alopecia.
74
What is the risk of systemic progression and malignancy in DLE?
5–20% progress to systemic lupus; 2–3% develop SCC in longstanding plaques.
75
What are key histological features of DLE?
Hyperkeratosis, follicular plugging, basement membrane thickening, dermal fibrosis, pigment incontinence; DIF positive at dermo-epidermal junction.
76
What is hypertrophic or verrucous lupus?
Hyperkeratotic, warty plaques that mimic hypertrophic lichen planus; histology shows pseudoepitheliomatous hyperplasia with lupus interface change.
77
What is lupus panniculitis (profundus)?
Deep firm subcutaneous nodules, often face/upper arms/buttocks, that heal with lipoatrophy/atrophic scarring; histology shows lobular panniculitis with hyaline fat necrosis.
78
How is chilblain lupus distinguished from perniosis?
Chilblain lupus lesions persist beyond cold exposure and are associated with anti-Ro positivity, unlike transient idiopathic chilblains.
79
What is tumid lupus?
Smooth erythematous oedematous plaques without scale, follicular plugging, or scarring; very photosensitive; histology shows abundant dermal mucin with minimal interface change.
80
What is bullous lupus erythematosus?
An acute vesiculobullous eruption in lupus patients, due to anti–type VII collagen antibodies; histology shows subepidermal neutrophil-rich blisters; DIF linear/granular at DEJ.
81
What is neonatal lupus?
Transplacental anti-Ro/SSA (± La/SSB) causes annular rash in infants and congenital heart block; maternal disease often SCLE or asymptomatic.
82
What is the lupus band test and how is it interpreted?
Direct immunofluorescence showing granular Ig/complement at the dermo-epidermal junction; positive in lesional CLE; positivity in non-lesional sun-protected skin suggests systemic lupus.
83
What is the first-line systemic therapy for CLE?
Hydroxychloroquine ≤5 mg/kg real body weight/day; baseline eye exam, annual after 5 years.
84
What is the role of other antimalarials in CLE?
Add quinacrine or switch to chloroquine if HCQ inadequate; combination therapy sometimes used.
85
What are the main second-line systemic immunosuppressants for CLE?
Methotrexate, mycophenolate mofetil, azathioprine; thalidomide/lenalidomide for refractory disease (toxicity limits use).
86
Which biologics have shown benefit in refractory CLE?
Belimumab (anti-BLyS, PBS-listed for SLE, not CLE alone); Anifrolumab (anti-IFN receptor, effective but not PBS-listed).
87
What general measures are essential in CLE management?
Strict photoprotection (SPF 50+, UPF clothing), smoking cessation, vitamin D supplementation.
88
What systemic progression risks should be remembered by subtype?
ACLE almost always systemic; SCLE ~30–40% systemic (mild); DLE 5–20%; chilblain/tumid/lupus panniculitis variable but lower risk.
89
What is a classic exam pearl about smoking and CLE treatment?
Smoking reduces hydroxychloroquine efficacy — always address in management stems.
90
What dermoscopy features may be seen in DLE?
Perifollicular white scaling, follicular plugs, telangiectasia, loss of follicular openings in scarring areas.
91
What high-yield numbers are important in CLE?
DLE → SLE 5–20%; SCLE → systemic 30–40%; SCC risk in DLE 2–3%; neonatal lupus CHB recurrence 15–20%; HCQ retinal toxicity <2% at 10 years (≤5 mg/kg).
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