Raynauds, Sjogrens And Systemic Sclerosis Flashcards by Callum Donoher

What is the typical presentation for Raynaud’s phenomenon?

30 yr old lady
Intermittent cold fingers worse in cold weather
Otherwise fit and well

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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

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What are the 3 stages of colour changes in raynauds?

White
Blue
Red

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What causes the colour changes seen in raynauds due to the episodic vasoconstriction?

White (ischaemia)
Blue (stasis/desaturation of haemoglobin)
Red ( reactive hyperaemia)

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What autoimmune rheumatica diseases is raynauds linked to?

SLE
Systemic sclerosis
Rheumatoid arthritis
Sjogrens

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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

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What is the non medical management or advice given to patients with raynauds?

Smoking cessation
Hand warmers
Warm socks

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What medications can be given to manage raynauds?

Oral vasodilators

Parenteral vasodilators

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What are some oral vasodilators that can be given to treat raynauds?

CCBs:
-Nifedipine
-Amlodipine
-Diltiazem

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What is an example of a parenteral vasodilator that can be used for raynauds?

Prostacyclin

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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

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What is systemic sclerosis?

Autoimmune connective tissue disease involving. Inflammation and fibrosis of connective tissues skin and internal organs

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What is scleroderma?

Hardening/thickening of the skin

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What is scleroderma?

Hardening/thickening of the skin

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What are the 2 types of systemic sclerosis?

Limited cutaneous systemic sclerosis

Diffuse cutaneous systemic sclerosis

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Which of the 2 types of systemic sclerosis is the worst?

Diffuse cutaneous systemic sclerosis

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What is the mnuemonic/syndrome that limited cutaneous systemic sclerosis used to be known. By?

CREST syndrome

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What are the features of limited cutaneous systemic sclerosis/ CREST syndrome?

Calcinosis (fingers)
Raynauds
oEsophageal dysmotility
Sclerodactyly
Telangiectasia

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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)

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How does scleroderma appear in a patient with systemic sclerosis?

Skin hardens
Tight shiny skin
No normal skin folds

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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

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What is Telangiectasia in systemic sclerosis?

Dilated blood vessels in the skin. Less than 1mm in diameter often seen on the face

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What is the calcinosis seen with systemic sclerosis?

Calcium deposits under skin most commonly at finger tips

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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