Connective Tissue Disorders Flashcards
What is the aetiology of Systemic Lupus Erythematosis?
- unknown, multifactorial
- genetic, environment, viral
- HLA DR2 and 3
- hormonal (high oestrogen metabolites)
- oral contraceptives
- African ancestry
What is the pathology of Systemic Lupus Erythematosis?
- vasculitis of arterioles, capillaries, venules
- fibrinoid necrosis with inflammatory cellular infiltrate
- deposition of immune complexes and fibrinoid material in tissues
- vasculitis, synovitis, pleuritis, pericarditis
- chronic, often fatal
- precipitated or exacerbated by sunlight, infection, stress
What are the clinical features of Systemic Lupus Erythematosis?
- fever, fatigue, ill, arthralgia, weight loss, anaemia
- migratory symmetrical polyarthritis
- telangiectasia and butterfly rash
- nephrotic syndrome, acute nephritic syndrome, chronic renal failure with hypertension
- pleurisy with effusion; patchy consolidation and plate collapse
- Pericarditis, endocarditis, raynauds phenom.
- arteritis, ishaemia, peripheral nerve lesions, convulsions, hallucinations, convulsions
- lymphadenopathy, hepatosplenomegaly
What is telangiectasia?
spider veins
What is drug induced SLE?
- primary complaints are arthralgia and pleuropericardial symptoms
- NO CNS or renal symptoms
What is different about SLE in pregnancy?
- high risk of foetal loss and maternal illness
- 25% are poor outcomes
- risk of intrauterine growth retardation
- neonates at risk of: heart block, thrombocytopenia, leukopenia, rash
- lupus flares during or after pregnancy
What is discoid lupus erythematosis?
- benign version of SLE
- autoimmune aetiology
- skin, face mostly
- lesions, patches, scarring, pigmentary changes
- lesions from trauma, stress, sunburn, cold
- NO visceral involvement
- small % develop SLE
What is progressive systemic sclerosis?
another name for scleroderma
What is scleroderma?
- inflammatory connective tissue of skin, lungs, GIT, heart, kidneys, msk
- preceded by Raynauds phenomenon
- unknown aetiology: maybe autoimmune
- genetic, HLA types
- vascular hypersensitivity to cold and serotonin
- axial skeleton
What is the pathology of scleroderma?
- low-grade inflammatory reaction of perivascular tissue with atrophy and fibrosis of adjacent collagen
- progressive intimal thickening with fibrosis and thrombosis
- fibrous tissue deposition
- muscles inflammed and degenerated
- synoviaux infiltrated with fibrin
What are the clinical features of scleroderma?
Hands: symmetric oedema, thickening, SAUSSAGE fingers (oedema, ulceration)
Fibrosis:
• thick tight skin, flexion contractures, mask face
• dysphagia, reflux esophagitis, constipation, distension, obstruction, malabsorption, diarrhoea, diverticula
• aspiration pneumonia, pulmonary hypertension, RV failure
• renal failure, arrhythmias, pericarditis, cardiomyopathy, valve lesions, nerve lesions
Skin:
• telangiectasia, vitiligo, hyperpigmentation
• nodular calcific masses ulcerate (fingers, forarm, ischium)
Joints: arthralgia, inflame. arthritis,
Compare the targets of SLE vs Scleroderma
SLE targets small blood vessels
Scleroderma targets perivascular tissue (beside vessels)
Compare the aetiologies of SLE and Scleroderma
Both unknown
SLE: genetic, environment, viral, hormonal
HLA DR2 and 3
Scleroderma: autoimmune
HLA types
Compare the age of onset of SLE and Scleroderma
SLE: peak onset 12-40, present in 20-30yrs
Scleroderma: 30-50yrs
Compare the sex ratio of SLE and Scleroderma
SLE is 9:1
Scleroderma is 3-4:1
What are the common clinical features of SLE and Scleroderma?
- systemic features: fever, fatigue, ill, weight loss, anaemia
- inflammatory arthritis
- telangiectasia
- Raynaud’s phenomenon
What are the defining features of SLE?
- purple, red, raised rash
- butterfly rash on face
- patchy lung consolidation
- joint, cardio, renal, nervous system involvement
- lymphadenopathy and hepatospleomegaly
What are the defining features of scleroderma?
- thickening of hands
- sausage fingers, ulcerated
- infiltrated synovium
- mask face, tight mouth
- reduced ROM from tight skin
- nodular subcutaneous calcific masses that may ulcerate through skin
- dysphagia, GIT problems
- respiratory, cardio, renal manifestations
What is CREST syndrome?
- benign version of scleroderma
- Candinosis, Raynaud’s, Esophageal dysfunction, Sclerodactyly, Telangectasis
- sclerosis limited to fingers and face
- calcification of subcutaneous tissues
- intestinal manifestations
- visceral involvement later on
What is polyartheritis nodosa?
- systemic necrotizing vasculitis
* causes dysfunction or infarction of tissues
What is Giant cell arteritis?
- affects the elderly
- chronic, granulomatous inflammation of blood vessels (external carotid)
- also polymyalgia rheumatica
- severe temporal headache, burning scalp, dilated tender temporal artery
- chewing painful
- vision maybe affected
What is Wegener’s granulomatosis?
- necrotising granulomatous vasculitis
- small to medium vessels
- autoimmune or hypersensitivity
- respiratory system
- glomerulonephritis
What is granulomatosis with polyangitis?
another name for Wegener’s granulomatosis
What is dermatomyositis?
Combined polymyositis and skin changes:
• discoloured papules over knuckles
• violet eye lid rash with local oedema
• erythematous upper body rash
What is polymyositis?
- progressive chronic inflammatory or skeletal muscle
- with other connective tissue disorder (eg. SLE, scleroderma)
- pain, tender, weak muscles of neck, pharynx, limb girdle
What is Sjogren’s syndrome?
- generalized or localized to mucous membranes, salivary and lacrimal glands
- dry eyes, mouth
- arthritis, dysphagia, hepatobiliary, pancreatic disease
- sensory neuropathies, renal complications, splenomegaly, lymphadenopathy, lymphoma
What is mixed connective tissue disease?
• autoimmune aetiology Overlapping clinical features with: • SLE • scleroderma • polymyositis • Raynaud's phenomenon
What is Behcet’s disease/syndrome?
- chronic inflammatory disorder of small blood vessels
- unknown aetiology
- recurrent aphthous ulcers of oral, laryngeal, genitalia
- uveitis, vasculitis, optic atrophy
- skin lesions
- msk, GIT, CNS affected
What is polymyalgia rheumatic?
- elderly
- pain in proximal muscles and joints
- maybe temporal arteritis
