Autoimmune rheumatic diseases:SLE Flashcards by Ayoyinka Ololade

List the main systemic autoimmune diseases

systemic Lupus erythematosus
Antiphospholipid syndrome
Systemic sclerosis
Inflammatory myopathies

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What is the CRP & ESR in Lupus

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

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What are the characteristics of SLE?

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)

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What may someone with mild SLE present with?

Mucocutaneous
Arthritis
Serositis
Mouth ulcers

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What may someone with severe SLE present with?

Nephritis
Vasculitis
CNS lupus
Thrombosis
Cardiopulmonary

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

Anti-nuclear antibody test

- RF test

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Outline the ACR classification criteria

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

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Describe the immunological criteria for SLE according to SLICC

ANA
Anti-dsDNA
Anti-Sm
Antiphospholipid Abs
Low complement
Direct Coombs’ test

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What is the direct Coombs’ test

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.

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How useful are Anti-dsDNA to diagnosis of Lupus

specific: 50-70%

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What is an extractable nuclear antigen panel?

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

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

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

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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

Antibodies to…

DNA= Renal, systemic
Nucleosomes= Renal,skin]
C1q= Renal
Alpha-actinin= renal
Sm=renal
NMDA receptor=CNS

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What is a potential link between EBV and Lupus

EPV can trigger Lupus

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

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Contrast the classic cytokine in RA & SLE

RA= TNF-alpha

Lupus=type 1 IFN

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Outline the course of SLE

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

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Describe the pathogenesis of SLE

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

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

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

What ancestry is SLE common in?

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

What are the different types of Cutaneous Lupus?

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

Outline Chilblain Lupus

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

Outline Jaccoud’s arthritis

-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

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

) Cutaneous vasculitis
)Small vessel vasculitis:
- splinter haemorrhages
- Digital infarcts
- Palpable purpura
- Vasculitic ulcers
- Urticarial vasculitis

Outline the predictors of poor outcome/mortality in SLE

Female
Black ethnicity
Infections
Younger age
Disease duration<1 year
Renal disease

Outline peripheral vascular disease in SLE

- Critical limb/digital ischaemia - Gangrene - Amputations

What other system can Lupus affect

every system - Pulmonary involvement= Lupus pneumonitis, Lupus serositis - Cardiac: pericarditis, myocarditis, endocarditis - Lupus nephritis

What are the presenting features of Lupus nephritis ?

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

What can be used in the assessment of Lupus

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

What are the characteristics of neuropsychiatric lupus?

- Psychosis - Seizures - Strokes - Psychological disorders - Transverse myelitis

How can we treat SLE

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

What is key to remember in corticosteroid therapy in SLE?

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

Outline the use of antimalarials in SLE

- 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

What threat does mycophenolate pose to women attempting to conceive

- 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.

Outline the use of Belimumab in SLE

- Biologics - Only one licensed for use - Blocks BLyS (B lymphocyte stimulator) - Apoptosis of autoreactive B cells

Outline the charactersitics of the antiphospholipid syndrome

-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

Outline treatment of CNS lupus

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

How do we handle pregnant women who are aPL +ve

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

What inflammatory myopathies may result from Lupus

- Dermatomyositis - Polymyositis - Cancer associated myositis - Inclusion body myositis - Muscle weakness - Raynaud's - Joint pains - Fevers - Rashes

How can we treat systemic sclerosis ?

- Early= low dose steroids immunosuppressives | - Late= symptomatic Rx BP control

Outline the characteristics of systemic sclerosis

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

What is CREST syndrome and what does it stand for?

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.