Raynauds, Sjogrens And Systemic Sclerosis Flashcards
What is the typical presentation for Raynaud’s phenomenon?
30 yr old lady
Intermittent cold fingers worse in cold weather
Otherwise fit and well
What is the pathophysiology for raynauds?
Stress/cold leads to vasospasm / excess vasoconstriction o blood vessels in fingers and toes
Impaired production of vasodilators like NO and prostacyclin
What are the 3 stages of colour changes in raynauds?
White
Blue
Red
What causes the colour changes seen in raynauds due to the episodic vasoconstriction?
White (ischaemia)
Blue (stasis/desaturation of haemoglobin)
Red ( reactive hyperaemia)
What autoimmune rheumatica diseases is raynauds linked to?
SLE
Systemic sclerosis
Rheumatoid arthritis
Sjogrens
What are some features a patient might have if they have secondary raynauds (caused by other autoimmune rheumatic diseases)?
Digital ulcers
Abnormal nail fold
Arthralgia or arthritis
Alopecia
Mouth ulcers
Fever
Malaise
Dry eyes or mouth
What is the non medical management or advice given to patients with raynauds?
Smoking cessation
Hand warmers
Warm socks
What medications can be given to manage raynauds?
Oral vasodilators
Parenteral vasodilators
What are some oral vasodilators that can be given to treat raynauds?
CCBs:
-Nifedipine
-Amlodipine
-Diltiazem
What is an example of a parenteral vasodilator that can be used for raynauds?
Prostacyclin
What surgical procedure can be useful for treating severe cases of raynauds in the lower limbs?
Sympathectomy
Since an enchanced sympathetic response leads to vasoconstriction of the blood vessels in the hands and feet
What is systemic sclerosis?
Autoimmune connective tissue disease involving. Inflammation and fibrosis of connective tissues skin and internal organs
What is scleroderma?
Hardening/thickening of the skin
What is scleroderma?
Hardening/thickening of the skin
What are the 2 types of systemic sclerosis?
Limited cutaneous systemic sclerosis
Diffuse cutaneous systemic sclerosis
Which of the 2 types of systemic sclerosis is the worst?
Diffuse cutaneous systemic sclerosis
What is the mnuemonic/syndrome that limited cutaneous systemic sclerosis used to be known. By?
CREST syndrome
What are the features of limited cutaneous systemic sclerosis/ CREST syndrome?
Calcinosis (fingers)
Raynauds
oEsophageal dysmotility
Sclerodactyly
Telangiectasia
How does diffuse cutaneous systemic sclerosis present differently to limited cutaneous systemic sclerosis?
Has all of the CREST signs but also has internal organ involvement:
-cardiovascular problems (HTN, Coronary artery disease)
-respiratory issues (Pulmonary fibrosis, pulmonary HTN)
-renal issues (scleroderma renal crisis)
How does scleroderma appear in a patient with systemic sclerosis?
Skin hardens
Tight shiny skin
No normal skin folds
What is sclerodactyly seen in patients with systemic sclerosis?
Tightening of skin around joints in the fingers
Fat pads in fingers lost
Skin can break and ulcerate
What is Telangiectasia in systemic sclerosis?
Dilated blood vessels in the skin. Less than 1mm in diameter often seen on the face
What is the calcinosis seen with systemic sclerosis?
Calcium deposits under skin most commonly at finger tips
How and why does oesophageal dysmotility occur in systemic Sclerosis?
What does it cause as a consequence?
Atrophy, dysfunction of smooth muscle in oesophagus and fibrosis of oesophagus
Dysphagia
Chest pain
Acid reflux (GORD)
Oesophagitis
What is scleroderma renal crisis?
Seen. With systemic sclerosis
Medical emergency with severe HTN and renal failure
A patient can have raynauds with systemic sclerosis or without systemic sclerosis, what examination can be done to determine whether the patient with raynauds has systemic sclerosis or not?
Nailfold capillaroscopy
How can nailfold capillaroscopy determine if a raynauds patient has systemic sclerosis?
Nailfold will have abnormal capillaries, Avascular areas and microheamorrhages if have systemic sclerosis
Raynauds without systemic sclerosis will have normal nailfold capillaries
What is the go to medications for raynauds?
Nifedipine
What medications can worsen symptoms of raynauds?
Beta blockers
What autoantibodies can be screened for to look for systemic sclerosis?
ANA (non specific)
Anti-centromere antibodies
Anti-Scl-70 antibodies
Which autoantibody is most associated with limited cutaneous systemic sclerosis?
Anti-centromere antibodies
Which autoantibody is most associated with diffuse cutaneous systemic sclerosis and more severe disease?
Anti-Scl-70 antibodies
How do you diagnose systemic sclerosis?
Clinical features (CREST), lung issues, HTN, renal issues
Antibodies
Nail-fold capillaroscopy
What is the non medical management for systemic sclerosis?
Smoking cessation
Gentle skin stretching to maintain range of motion
Regular emollients
Avoid cold triggers (raynauds)
Physiotherapist
Occupational therapy
What medications are given in diffuse systemic sclerosis?
What is the issue with steroids?
DMARDs (methotrexate)
Biologics (rituximab)
Steroids? (Inc risk of scleroderma renal crisis)
What is the approach for the medical management for systemic sclerosis?
Treating symptoms and complications rather than the condition
How may a patietn with systemic sclerosis present?
GORD with oesophagitis
Constipation
SOB
Raynauds
What medications would you give to a patient with systemic sclerosis if they are having GORD and constipation?
PPI (omeprazole)
Prokinetic medications like metoclopramide
What medications would you give a patient who has systemic sclerosis who has hypertension and scleroderma renal crisis?
ACEi (ramipril)
If you think a patient might have systemic sclerosis and the have SOB, what investigations will you request?
What’s it looking for?
HRCT
Pulmonary fibrosis
What medications would you give and what would you be weary of and why when you discover a patient has pulmonary fibrosis due to systemic sclerosis?
DMARDs like methotrexate or biologics like rituximab
Would be cautious giving corticosteroids like prednisolone due to steroids increasing the risk of scleroderma renal crisis
What medications would you give and what would you be weary of and why when you discover a patient has pulmonary fibrosis due to systemic sclerosis?
DMARDs like methotrexate or biologics like rituximab
Would be cautious giving corticosteroids like prednisolone due to steroids increasing the risk of scleroderma renal crisis
What are the most severe common complications of systemic sclerosis that lead to mortality?
Pulmonary hypertension and fibrosis
Scleroderma renal crisis
What is Sjögren’s syndrome?
Autoimmune condition affecting the exocrine glands mainly the lacrimal and salivary glands due to lymphocyte infiltration
What parts of the body can Sjögren’s syndrome affect?
Lacrimal
Salivary
Vaginal
Pancreas
What can dry eyes and dry mouth be called due to Sjögren’s syndrome?
Sicca symptoms
Who is Sjögren’s syndrome most common in?
Females 40-50
What are the 2 types of Sjögren’s syndrome?
Primary Sjögren’s syndrome and secondary Sjögren’s syndrome
What is primary Sjögren’s syndrome and what is secondary Sjögren’s syndrome?
Primary = happens in isolation
Secondary = occurs due to other diseases like SLE and rheumatoid arthritis
What are some clinical features of Sjorgens syndrome?
Dry eyes dry mouth (sicca)
Parotid enlargement
Cough or. Dysphagia (dry membranes)
Joint pains
Raynauds
Associated with lymphoma
What investigations are done on a patient who you think has Sjögren’s syndrome?
Routine bloods
Autoantibodies
Schirmer test
What auto antibodies are you going to test for if you think the patient might have Sjögren’s syndrome?
Anti-Ro antibodies
Anti-La antibodies
ANA
Rheumatoid factor
What is schirmer test and what is a significant result?
Folded filter paper isteretd under lower eyelid for 5mins
Moisture will travel by diffusion along paper
Less than 10mm is significant
15mm or more in normal healthy adult
How do you manage the dry eyes with Sjögren’s syndrome?
Artificial tears (polyvinyl alcohol eye drops)
What medication can. Be used to stimulate tear and saliva production?
What class of mediation is this?
Pilocarpine
Muscarinic agonist stimulating parasympathetic nerves
What medication can be given to a pateitn with Sjögren’s syndrome that has Arthralgia?
Hydroxychloroquine
What aer some complications of Sjögren’s involving exocrine gland dysfunction?
Keratoconjuctivitis sicca and corneal ulcers
Oral problems like dental cavities and candida infections
Vaginal problems candida infection and sexual dysfunction
What are some rare complications where Sjögren’s syndrome affects organs?
Pneumonia
Bronchiectasis
Non-Hodgkins lymphoma
Peripheral neuropathy
Vasculitis
Renal impairment
