RHEUM: SLE and Sjogrens Flashcards

1
Q

What are some extra - articular features of SLE?

LOADS IN THE BOOKLET - GUESS A FEW systems based!

A

Mouth / Eyes
- mouth uclers

Skin

  • Digital ulcers
  • Malar flush / photosensitivity
  • Alopecia
  • Raynaud’s

Genito-urinary

  • renal failure / hypertension
  • Micro -haematuria / proteinuria

Obstetric
-Miscarriage / pre-eclampsia

Neuro

  • headache
  • seizures
  • psychosis
  • TIA / CVA

Cardio- Resp
SOB - PE / Pul effusion / Pulmonary HTN / alveolitis/ pleuritic chest pain

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2
Q

What are some extra - articular features of Sjogrens Syndrome?

A

Mouth / Eyes
- Dry mouth / eyes

Skin
- Raynaud’s

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3
Q

What is Lupus (SLE)?

A

Inflammatory autoimmune connective tissue disease.

Characterised by inadequate T cell suppressor activity and increased B cell activity.

Anti-nuclear antibodies target proteins in own cell nucleus

Remissions and flares
complex multi organ involvement / varied presentations

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4
Q

What are the common symptoms and signs with SLE?

SOAP BRAIN MD mnemonic

A

SOAP BRAIN

Serositis -pleurisitis / pericarditis

Oral ulcers - esp palatte, painless

Arthritis - small joint non-erosive

Photosensitivity - malar / discoid rash

Blood disorders - Low WCC, lymphopenia

Renal involvement - glomerulonephritis

Autoantibodies - ANA +ve in 90%

Immunological - low complements e..g C3 C4 low

Neurologic - seizures / psychosis

M - malar rash

D - discoid rash

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4
Q
A
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5
Q

What investigations for SLE?

A

Bloods:
Raised ESR / plasma viscosity
FBC - normocytic anaemia of chronic disease / leukopenia

Autoantibodies
ANA +ve (90% are)
Anti dsDNA (specific to SLE) rises with active disease
Antiphospholipid antibodies can occur in SLE - VTE risk

Complement
C3 C4 decreased in active disease

Urinanalysis and protein: creatinine ration for proteinuria in lupus nephritis

Skin biopsy / renal biopsy can be diagnostic

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6
Q

Why is ESR more useful than CRP in SLE?

A

Often ESR raised (+ plasma viscosity ) in pts where CRP can be normal

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7
Q

Why is FBC particularly useful for SLE?

A

Simple clue as abnormal in almost all patients

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8
Q

Common complications of SLE?

A

page 220 of Z2F

CVD- chronic inflammation in vessels - HTV- CAD
Anaemia of chronic Disease
Pericarditis 
Plueritis 
Interstitial lung disease
Lupus nephritis
Neuro psychiatric SLE 
Recurrent miscarriage 
VTE
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9
Q

How to diagnose SLE?

A

SLICC or ACR criteria

confirm Antinuclear antibodies and establish clinical features suggestive of SLE

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10
Q

Treatment of SLE ?

A

Drug:

NSADIS
Steroids (prednisolone) short courses for flares
DMARD : hydroxychloroine (rash and arthralgia)

Can move onto Methotrexate / Mycophenolate mofetil / Azathoprine
Biologics for severe disease e.g. Rituximab

Non drug:
Sun avoidance - malar rash
Lifestyle advice - CVD

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11
Q

What is Raynaud’s pnenomenon? What is the typical colour change

A

Painful vasospasm of digits. Idiopathic, possibly familial, women ++

Colour change in response to cold stimulus:
White = reduced blood flow
Blue = venous stasis
Red = rewarming hyperaemia

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12
Q

What diseases are associated with Raynaud’s?

A

Scleroderma
SLE
Dermatomyositis and polymyositis
Sjorgen’s syndrome

> 30 yrs when develop think underlying disease

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13
Q

Physical causes of Raynaud’s?

A

Physical cause:
heavy vibrating tools
sticky blood e..g cryoglobulinaemia

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14
Q

Drug cause of Raynaud’s?

A

Beta blockers

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15
Q

Treatment for Raynaud’s?

A

Non drug:
Stay warm
stop smoking

Drug:
Dihydropyridine CCB-nifedipine

Phosphodiesterase-5-inhibitors
Prostacylins

16
Q

Raynaud’s episodes usually last ___(1)____
The pattern is ___(2)_____ and ____(3)____
___(4)____ is rare

A

Raynaud’s episodes usually last ___minutes____
The pattern is _bilateral__ and _symmetrical____
___Tissue Necrosis ____ is rare

17
Q

What are some complications of Raynaud’s?

A

Digital ulcers
infection
gangrene

18
Q

Patients with which _S_____ and __S_____ can develop myositis ?

A

Patients with which __Scleroderma__ and __SLE__ can develop myositis ?

19
Q

What is the pathophysiology of Sjogren’s syndrome?

A

Chronic, autoimmune inflammatory disorder. Inflammation leads to diminished lacrimal and salivary gland secretion.

20
Q

What are the types of Sjogren’s syndrome?

A

Primary - not related to another disease

Secondary - related to an underlying disease

21
Q

Which gender most commonly presents with Sjogren’s syndrome?

A

Women - up to 80%

22
Q

How can Sjogren’s syndrome present?

A

Common presentation is common! Most common are called sicca symptoms - dry eyes (xerophthalmia), dry mouth (xerostomia) and fatigue.

MADFRED mnemonic for all common symptoms 
Myalgia 
Arthralgia 
Dry eyes 
Fatigue 
Raynaud's phenomenon 
Enlarged parotids  
Dry mouth
23
Q

What investigations would you do for patient presenting with dry eyes, dry mouth and tiredness? You also note her parotid glands are enlarged.

A

This is a presentation of Sjogren’s ! Investigations would include:
Antibody screen - Anti Ro and Anti La antibodies common. May also have RF and anti ds-DNA
Schirmer’s test - measure tear volume (would be reduced if pt has Sjogrens where the lacrimal glands are diminished)
Salivary gland biopsy

24
Q

How is Sjogren’s managed?

A

Treated based on symptoms

  • educational = avoid dry or smokey atmospheres.
  • symptomatic = artificial tears, artificial saliva, skin emollients, vaginal lubricants
  • autoimmune profile = immunosuppressants and steroids are rarely needed.
25
Q

What other conditions may a patient with Sjogrens also have?

A

RA, SLE = v common

Other autoimmune conditions - coeliac disease, primary biliary cirrhosis, AI Thyroid disease

26
Q

A woman with Sjogrens is pregnant with her first child. What would you have to inform her of, regarding her condition and baby?

A

Anti Ro antibodies can:

  • increase the risk of foetal loss.
  • cause complete heart block in the foetus
  • cause neonatal lupus syndrome in newborn.
27
Q

A salivary gland biopsy is sometimes taken in suspected Sjogrens patients. What can be seen in the biopsy?

A

A characteristic picture of Sjogrens - focal lymphocytic infiltration of exocrine glands. Google this for histology slide

28
Q

Be aware of the SLICC criteria:

A

The SLICC criteria for SLE classification requires: 1) Fulfillment of at least four criteria, with at least one clinical criterion AND one immunologic criterion OR 2) Lupus nephritis as the sole clinical criterion in the presence of ANA or anti-dsDNA antibodies.

Clinical Criteria:

  1. Acute cutaneous lupus
  2. Chronic cutaneous lupus
  3. Oral ulcers: palate
  4. Nonscarring alopecia (diffuse thinning or hair fragility with visible broken hairs)
  5. Synovitis involving two or more joints, characterized by swelling or effusion OR tenderness in two or more joints and thirty minutes or more of morning stiffness.
  6. Serositis
  7. Renal
  8. Neurologic
  9. Hemolytic anemia
  10. Leukopenia (< 4000/mm3 at least once)
  11. Thrombocytopenia (<100,000/mm3) at least once

Immunological Criteria:

  1. ANA above laboratory reference range
  2. Anti-dsDNA above laboratory reference range, except ELISA: twice above laboratory
  3. Anti-Sm
  4. Antiphospholipid antibody: any of the following
  5. Low complement
  6. Direct Coombs test in the absence of hemolytic anemia