Connective tissue disease : SS And Sjogrens Syndrome Flashcards
Systemic sclerosis
Tissue being replaced with tissue that is much less yieldy
Systemic sclerosis (SScl) causes fibrosis affecting:
skin
internal organs
vasculature
It is characterised by Raynaud’s phenomenon (+/-digital ischaemia), sclerodactyly, and cardiac, lung, gastrointestinal and renal disease.
Epidemiology:
peak age of onset is in the fourth and fifth decades
• overall prevalence is 10–20 per 100 000
4:1 female: male
Sub divided into:
Diffuse cutaneous SS - 30%
Limited cutaneous SS - 70% —> CREST (calcinosis, raynauds, oesphogus, ..)
Prognosis: 70% of patients survive 5 years
Pathophysiology
Don’t need to know
Not completely understood
SS: Clinical features
Skin:
• non-pitting oedema of fingers and flexor tendon sheaths
• then becomes shiny and taut
• there can be capillary loss.
• the face and neck are often involved, with thinning of the lips and radial furrowing
• lcSScl: skin involvement restricted to sites distal to the elbow or knee (apart from the face)
dcSScl: involvements proximal to the knee or elbow and in the trunk is called diffuse disease
SS other features
• Raynaud’s phenomenon
◦ May precede other features by many years. When severe and progressive, critical tissue ischaemia, with distal skin infarction and necrosis.
• Musculoskeletal features
◦ Arthralgia and flexor tenosynovitis are common
◦ Restricted hand function is due to skin rather than joint disease
◦ Muscle weakness and wasting can result from myositis
• Gastrointestinal involvement
◦ Erosive oesophagitis
◦ Dysphagia
◦ Malabsorption due to bacterial overgrowth ◦ Dilatation of bowel
• Pulmonary involvement
◦ pulmonary hypertension more prevalent in lcSScl than in dcSScl.
◦ complain of shortness of breath on exertion
◦ interstitial lung disease is common
• Renal involvement
• Hypertensive renal crisis
◦ Rapid increasing hypertension and renal failure
SS investigations
• Multiple organs involvement so:
• Blood tests: ◦ FBC
◦ U&E
◦ LFTs
◦ bone group
◦ urinalysis are essential
◦ ANA (anti nuclear antibodies) is positive in about 70%.
◦ Scl70 positive in 30% of patients with dcSScl
◦ anticentromere antibodies in 60% of patients with lcSScl
syndrome
• Imaging:
◦ chest X-ray/ CT chest
◦ echocardiography
Lung function tests
Barium swallow Can assess oesophageal involvement
SS Management
• Nil to stop or reverse fibrosis
• Try to slow the effects of the disease on target organs.
• Raynaud’s phenomenon and digital ulcers:
◦ Avoid cold, thermal gloves/socks, high core temperature (calcium channel blockers, losartan, fluoxetine, sildenafil. intravenous prostacycline, bosentan)
• Gastrointestinal complications:
◦ proton pump inhibitors
• Hypertension:
◦ ACE inhibitors
Joint involvement:
NSAIDS, analgesia
Progressive pulmonary hypertension
Bosentan, or heart- lung transport
Interstitial lung disease
Glucocorticoids
SS SUMMARY
• Fibrosis
• Try to stop progression
• Raynauds may severe
• Hypertensive renal crisis
Mixed connective tissue disease
• A condition in which some clinical features of SScl, myositis and SLE all occur in the same patient.
• Most patients have anti-RNP antibodies.
• Management focuses on treating the components of the disease (see other sections).
Primary Sjögren’s syndrome (PSS)
• lymphocytic infiltration and fibrosis of salivary and lacrimal glands
• typical age of onset is between 40 and 50,
• 9 : 1 female-to-male ratio
• may occur with other autoimmune diseases (secondary Sjögren’s syndrome).
• Clinical features
◦ Eye- dry or gritty eyes with conjunctivitis and blepharitis and damage to cornea
◦ Mouth – (dry xerostomia) with dental caries ◦ Fatigue
◦ Small joint pain
◦ Interstitial lung disease (rare)
40 fold increased lifetime risk of lymphoma
Investigations for Sjögren’s syndrome
• FBC
• U&E
• Rheumatoid factor
• Antinuclear antibody (ANA)
• SS-A (anti-Ro) - can cross placenta and affect feotus
• SS-B (anti-La) - can cross placenta and affect feotus
• Schirmers test
◦ (6mm + after 5 mins)
Management of Sjögren’s syndrome
• Symptomatic only
◦ Eye drops
◦ Artificial saliva sprays, saliva-stimulating tablets, and pastilles and oral gels
◦ Chewing gum
◦ Oral hygiene
◦ Vaginal dryness is treated with lubricants
◦ pilocarpine (5–30 mg daily in divided doses) is worthwhile in
early disease to amplify glandular function.
◦ Hydroxychloroquine (200 mg twice daily) is often used to
address skin and musculoskeletal features and may help fatigue
◦ Immunosuppression for progressive interstitial lung disease
(e.g. glucocorticoids and cyclophosphamide) and for interstitial nephritis
◦ If lymphadenopathy or salivary gland enlargement develops, excludes malignancy
SUMMARY FOR Sjögren’s syndrome
• Dryness and fatigue
• Look normal!
• Increased risk of lymphoma
