Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

Inflammation block > Autoimmune rheumatic diseases:SLE > Flashcards

Autoimmune rheumatic diseases:SLE Flashcards

1
Q

List the main systemic autoimmune diseases

A
  • systemic Lupus erythematosus
  • Antiphospholipid syndrome
  • Systemic sclerosis
  • Inflammatory myopathies
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

What is the CRP & ESR in Lupus

A

CRP=0
ESR=100
-There are very few conditions that give you this rare pattern 4
-When the CRP>100 this tells you that the Lupus patient has infection

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

What are the characteristics of SLE?

A
  • about 10 times more common in females than men
  • cytopenia
  • Lymphopenia
  • Neutropenia
  • Thrombocytopenia & low complement c3 & c4
  • Lupus patients are very sensitive for anti-nuclear antibody (but these aren’t specific to Lupus)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

What may someone with mild SLE present with?

A
  • Mucocutaneous
  • Arthritis
  • Serositis
  • Mouth ulcers
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

What may someone with severe SLE present with?

A
  • Nephritis
  • Vasculitis
  • CNS lupus
  • Thrombosis
  • Cardiopulmonary
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What are the basic screening tests in an auto-antibody disease?

A
  • Anti-nuclear antibody test

- RF test

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Outline the ACR classification criteria

A
  • Malar erythema
  • Discoid lesions
  • Photosensitivity
  • Oral or nasopharyngeal ulceration
  • Arthritis
  • Serositis
  • Nephritis
  • CNS
  • Cytopaenia
  • ANA
  • Specific autoantibodies: anti-DNA, Sm, aCL, LA
  • Classification>/= 4 criteria needed
  • Classification criteria are NOT diagnostic criteria
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Describe the immunological criteria for SLE according to SLICC

A
  • ANA
  • Anti-dsDNA
  • Anti-Sm
  • Antiphospholipid Abs
  • Low complement
  • Direct Coombs’ test
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

What is the direct Coombs’ test

A
  • Used to test for autoimmune hemolytic anemia( a condition where the immune system breaks down red blood cells, leading to anemia).
  • Used to detect antibodies or complement proteins attached to the surface of red blood cells.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

How useful are Anti-dsDNA to diagnosis of Lupus

A

specific: 50-70%

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

What is an extractable nuclear antigen panel?

A
  • Detects the presence of auto-antibodies in the blood that react with proteins in the cell nucleus
  • Certain autoimmune disorders are characteristically associated with the presence of one or more anti-ENA antibodies
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Outline the potential abs that may appear in an extractable nuclear antigen panel for SLE& their clinical features

A

Ro/SSA= photosensitivity
LA/SSB= neonatal Lupus
RNP= Raynaud’s/overlap
Sm=SLE specific (African ancestry)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

When diagnosing SLE it may be seen that a patients has abs to something eg a cell component. List some of these and their clinical expression

A

Antibodies to…

  • DNA= Renal, systemic
  • Nucleosomes= Renal,skin]
  • C1q= Renal
  • Alpha-actinin= renal
  • Sm=renal
  • NMDA receptor=CNS
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

What is a potential link between EBV and Lupus

A
  • EPV can trigger Lupus

- can also be a mimic of paediatric/juvenile onset Lupus

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Contrast the classic cytokine in RA & SLE

A

RA= TNF-alpha

Lupus=type 1 IFN

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Outline the course of SLE

A

Environmental triggers (sunlight, drugs,chemicals,smoking and viruses) & a genetic component together with defective apoptosis(resulting in autoantigens being exposed and a failure to clear these) lead to the onset of SLE

  • PRE CLINICAL= autoantibodies present
  • CLINICAL DISEASE= organ involvement; flares; damage
  • CO-MORBIDITIES= infections, atherosclerosis, malignancies
  • end result= potential death overtime
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

Describe the pathogenesis of SLE

A
  • Defective apoptosis leads to autoantigens
  • The germinal centre consists of Follicular dendritic cells and autoreactive B cells.
  • With the help of type 1 interferons these germinal centre cells produce autoantibody producing plasma B cells
  • These cells produce immune complexes& complement
  • Complement/immune complexesbind to tissues and vascular inflammation or Lupus nephritis results
18
Q

The cause of SLE is unknown, but outline the implicated factors

A
  • Female hormones ( ratio 10:1 for F:M): disease onset after menarche, decline in disease activity with menopause, women have 2X chromosomes so double the genetic load
  • Genetics: increased risk of sibling developing SLE; monozygotic 10 fold risk vs dizygotic from twin studies; HLA-a1,b8,dr3 and complement deficiency gene are all in the HLA and that’s the strongest associated with an increased risk of Lupus
  • Drugs
  • Environmental factors
  • Ethnic origin
19
Q

What ancestry is SLE common in?

A

African & Asian ancestry

  • they have a polymorphism in FCGR2B gene which leads to decreased inhibitory function leading to B cell activation
  • This polymorphism gives them protection against severe malaria but an increased risk of SLE
  • Lupus is uncommon in Africa but is more common & more severe in migrants from African & Asian ancestry
20
Q

What are the different types of Cutaneous Lupus?

A
  • Discoid Lupus: central area of depigmentation; chronic; rarely seen on legs
  • Subacute cutaneous Lupus: most photosensitive type; often flare up in summer ;no rash in sun protected areas
21
Q

Outline Chilblain Lupus

A
  • Variant of discoid Lupus
  • Most patients have Raynauds
  • The lesions feel cold
  • Looks like vasculitis but isn’t
22
Q

Outline Jaccoud’s arthritis

A

-Arthritis=common in Lupus& you often get deformities
(RA destroys joint,cartilages and tendons so you get fixed deformities)
-These patients instead would have normal functioning of their hands cos Lupus primarily affects the tendons

23
Q

What may a patient present with if they have Lupus vasculitis?

A
  1. ) Cutaneous vasculitis
  2. )Small vessel vasculitis:
    - splinter haemorrhages
    - Digital infarcts
    - Palpable purpura
    - Vasculitic ulcers
    - Urticarial vasculitis
24
Q

Outline the predictors of poor outcome/mortality in SLE

A
  • Female
  • Black ethnicity
  • Infections
  • Younger age
  • Disease duration<1 year
  • Renal disease
25
Q

Outline peripheral vascular disease in SLE

A
  • Critical limb/digital ischaemia
  • Gangrene
  • Amputations
26
Q

What other system can Lupus affect

A

every system

  • Pulmonary involvement= Lupus pneumonitis, Lupus serositis
  • Cardiac: pericarditis, myocarditis, endocarditis
  • Lupus nephritis
27
Q

What are the presenting features of Lupus nephritis ?

A

-Asymptomatic proteinuria
-Nephrotic syndrome
-Renal failure
-Rapidly progressing glomerulonephritis
-Dysmorphic/fragmented red cells
-Granular casts
Consider renal biopsy unless contraindicated.
Diagnosis=activity
Prognosis=damage

28
Q

What can be used in the assessment of Lupus

A
  1. ) Assessing disease activity:
    - BILAG
    - SLEDAI
  2. ) Assessing QoL:
    - Lupus QoL
    - SF36, HAQ
  3. )Damage:
    - ACR/SLICC
    - Damage index
29
Q

What are the characteristics of neuropsychiatric lupus?

A
  • Psychosis
  • Seizures
  • Strokes
  • Psychological disorders
  • Transverse myelitis
30
Q

How can we treat SLE

A

1.) Less severe stage=
-sun avoidance, topical steroids, calcineurin inhibitors & retinoids, Hydroxychloroquine(stop smoking), Mepacrine; analgesics- NSAIDS contraindicated
2.) Increasingly worse=
-corticosteroids: mycophenolate mofetil ;thalidomide ;Azathioprine; Dapsone; Retinoids; UVA
3.)Much worse=
Biologics these are B cell targets eg Belimumab, Rituximab, Cyclophosphamide

31
Q

What is key to remember in corticosteroid therapy in SLE?

A

-Use the lowest effective dose for the shortest period of time (cos it’s a major contributor to damage)

32
Q

Outline the use of antimalarials in SLE

A
  • Hydroxychloroquine= standard of care, everyone gets this
  • Mepacrine/Quinacrine (gives you a yellow discolouration)
  • Combination antimalarials
  • Long term use well tolerated inc pregnancy
  • 65% decreased risk of cardiac neonatal Lupus
  • Decreases relapse rate & risk of renal flares
  • Improves lipid& glucose profiles
  • Thromboprophylaxis
  • Reduces risk of damage&mortality
  • 1st line Rx for all SLE patients
  • Smoking reduces anti-malarial efficacy
33
Q

What threat does mycophenolate pose to women attempting to conceive

A
  • Can cause fertility issues
  • Can lead to teratogenicity in human pregnancies
  • Treatment with such agents should be avoided in women for at least 6 weeks prior to attempted conception.
34
Q

Outline the use of Belimumab in SLE

A
  • Biologics
  • Only one licensed for use
  • Blocks BLyS (B lymphocyte stimulator)
  • Apoptosis of autoreactive B cells
35
Q

Outline the charactersitics of the antiphospholipid syndrome

A

-A disorder of the immune system that causes an increased risk of blood clots
-Thrombosis & pregnancy morbidity
-10-15% of Lupus patients have it
-Positive antiphospholipid abs= anticardiolpin abs( aCL); Lupus anticoagulant(LA); Anti-beta2 glycoprotein 1 abs
could result in:
1.)Uteroplacental insufficiency; IUGR= Fetal death, still birth
2.)Pre-eclampsia, abruption, premature delivery, thrombosis

36
Q

Outline treatment of CNS lupus

A
  • Focal disease aPL +ve= strokes/TIAs - anticoagulation needed
  • Diffuse disease= organic brain syndrome- Immunosuppression needed
37
Q

How do we handle pregnant women who are aPL +ve

A
  1. ) No thrombosis or pregnancy loss: careful monitoring & aspirin
  2. ) Previous maternal thrombosis: Hep& aspirin
  3. ) Recurrent embryonic loss (<10weeks): Hep& aspirin; low dose aspirin
  4. ) Late fetal death/IUGR/severe pre-eclampsia: Hep& aspirin
  5. ) Post partum: 6/52 aspirin
38
Q

What inflammatory myopathies may result from Lupus

A
  • Dermatomyositis
  • Polymyositis
  • Cancer associated myositis
  • Inclusion body myositis
  • Muscle weakness
  • Raynaud’s
  • Joint pains
  • Fevers
  • Rashes
39
Q

How can we treat systemic sclerosis ?

A
  • Early= low dose steroids immunosuppressives

- Late= symptomatic Rx BP control

40
Q

Outline the characteristics of systemic sclerosis

A

High risk of mortality due to:

  • early diffuse disease( <2yrs)
  • Rapid cutaneous progression
  • Renal crisis
  • GI involvement
  • Early ILD
  • Pulmonary hypertension
  • Pulmonary fibrosis
  • Intestinal disease
41
Q

What is CREST syndrome and what does it stand for?

A

aka Limited cutaneous systemic sclerosis

  • A multisystem connective tissue disorder
  • Refers to the five main features: Calcinosis, Raynaud’s phenomenon, Esophageal dysmotility, Sclerodactyly, and Telangiectasia.

Inflammation block (27 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions