RHEUM: SLE and Sjogrens Flashcards
What are some extra - articular features of SLE?
LOADS IN THE BOOKLET - GUESS A FEW systems based!
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
What are some extra - articular features of Sjogrens Syndrome?
Mouth / Eyes
- Dry mouth / eyes
Skin
- Raynaud’s
What is Lupus (SLE)?
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
What are the common symptoms and signs with SLE?
SOAP BRAIN MD mnemonic
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
What investigations for SLE?
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
Why is ESR more useful than CRP in SLE?
Often ESR raised (+ plasma viscosity ) in pts where CRP can be normal
Why is FBC particularly useful for SLE?
Simple clue as abnormal in almost all patients
Common complications of SLE?
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
How to diagnose SLE?
SLICC or ACR criteria
confirm Antinuclear antibodies and establish clinical features suggestive of SLE
Treatment of SLE ?
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
What is Raynaud’s pnenomenon? What is the typical colour change
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
What diseases are associated with Raynaud’s?
Scleroderma
SLE
Dermatomyositis and polymyositis
Sjorgen’s syndrome
> 30 yrs when develop think underlying disease
Physical causes of Raynaud’s?
Physical cause:
heavy vibrating tools
sticky blood e..g cryoglobulinaemia
Drug cause of Raynaud’s?
Beta blockers
Treatment for Raynaud’s?
Non drug:
Stay warm
stop smoking
Drug:
Dihydropyridine CCB-nifedipine
Phosphodiesterase-5-inhibitors
Prostacylins
Raynaud’s episodes usually last ___(1)____
The pattern is ___(2)_____ and ____(3)____
___(4)____ is rare
Raynaud’s episodes usually last ___minutes____
The pattern is _bilateral__ and _symmetrical____
___Tissue Necrosis ____ is rare
What are some complications of Raynaud’s?
Digital ulcers
infection
gangrene
Patients with which _S_____ and __S_____ can develop myositis ?
Patients with which __Scleroderma__ and __SLE__ can develop myositis ?
What is the pathophysiology of Sjogren’s syndrome?
Chronic, autoimmune inflammatory disorder. Inflammation leads to diminished lacrimal and salivary gland secretion.
What are the types of Sjogren’s syndrome?
Primary - not related to another disease
Secondary - related to an underlying disease
Which gender most commonly presents with Sjogren’s syndrome?
Women - up to 80%
How can Sjogren’s syndrome present?
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
What investigations would you do for patient presenting with dry eyes, dry mouth and tiredness? You also note her parotid glands are enlarged.
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
How is Sjogren’s managed?
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.
What other conditions may a patient with Sjogrens also have?
RA, SLE = v common
Other autoimmune conditions - coeliac disease, primary biliary cirrhosis, AI Thyroid disease
A woman with Sjogrens is pregnant with her first child. What would you have to inform her of, regarding her condition and baby?
Anti Ro antibodies can:
- increase the risk of foetal loss.
- cause complete heart block in the foetus
- cause neonatal lupus syndrome in newborn.
A salivary gland biopsy is sometimes taken in suspected Sjogrens patients. What can be seen in the biopsy?
A characteristic picture of Sjogrens - focal lymphocytic infiltration of exocrine glands. Google this for histology slide
Be aware of the SLICC criteria:
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:
- Acute cutaneous lupus
- Chronic cutaneous lupus
- Oral ulcers: palate
- Nonscarring alopecia (diffuse thinning or hair fragility with visible broken hairs)
- 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.
- Serositis
- Renal
- Neurologic
- Hemolytic anemia
- Leukopenia (< 4000/mm3 at least once)
- Thrombocytopenia (<100,000/mm3) at least once
Immunological Criteria:
- ANA above laboratory reference range
- Anti-dsDNA above laboratory reference range, except ELISA: twice above laboratory
- Anti-Sm
- Antiphospholipid antibody: any of the following
- Low complement
- Direct Coombs test in the absence of hemolytic anemia
