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Medical Sciences B > Connective Tissue Disorders > Flashcards

Connective Tissue Disorders Flashcards

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USMLE® Step 1 flashcards
1
Q

What is the aetiology of Systemic Lupus Erythematosis?

A
  • unknown, multifactorial
  • genetic, environment, viral
  • HLA DR2 and 3
  • hormonal (high oestrogen metabolites)
  • oral contraceptives
  • African ancestry
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2
Q

What is the pathology of Systemic Lupus Erythematosis?

A
  • 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
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3
Q

What are the clinical features of Systemic Lupus Erythematosis?

A
  • 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
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4
Q

What is telangiectasia?

A

spider veins

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5
Q

What is drug induced SLE?

A
  • primary complaints are arthralgia and pleuropericardial symptoms
  • NO CNS or renal symptoms
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6
Q

What is different about SLE in pregnancy?

A
  • 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
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7
Q

What is discoid lupus erythematosis?

A
  • 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
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8
Q

What is progressive systemic sclerosis?

A

another name for scleroderma

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9
Q

What is scleroderma?

A
  • 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
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10
Q

What is the pathology of scleroderma?

A
  • 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
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11
Q

What are the clinical features of scleroderma?

A

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,

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12
Q

Compare the targets of SLE vs Scleroderma

A

SLE targets small blood vessels

Scleroderma targets perivascular tissue (beside vessels)

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13
Q

Compare the aetiologies of SLE and Scleroderma

A

Both unknown

SLE: genetic, environment, viral, hormonal
HLA DR2 and 3

Scleroderma: autoimmune
HLA types

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14
Q

Compare the age of onset of SLE and Scleroderma

A

SLE: peak onset 12-40, present in 20-30yrs

Scleroderma: 30-50yrs

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15
Q

Compare the sex ratio of SLE and Scleroderma

A

SLE is 9:1

Scleroderma is 3-4:1

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16
Q

What are the common clinical features of SLE and Scleroderma?

A
  • systemic features: fever, fatigue, ill, weight loss, anaemia
  • inflammatory arthritis
  • telangiectasia
  • Raynaud’s phenomenon
17
Q

What are the defining features of SLE?

A
  • purple, red, raised rash
  • butterfly rash on face
  • patchy lung consolidation
  • joint, cardio, renal, nervous system involvement
  • lymphadenopathy and hepatospleomegaly
18
Q

What are the defining features of scleroderma?

A
  • 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
19
Q

What is CREST syndrome?

A
  • 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
20
Q

What is polyartheritis nodosa?

A
  • systemic necrotizing vasculitis

* causes dysfunction or infarction of tissues

21
Q

What is Giant cell arteritis?

A
  • 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
22
Q

What is Wegener’s granulomatosis?

A
  • necrotising granulomatous vasculitis
  • small to medium vessels
  • autoimmune or hypersensitivity
  • respiratory system
  • glomerulonephritis
23
Q

What is granulomatosis with polyangitis?

A

another name for Wegener’s granulomatosis

24
Q

What is dermatomyositis?

A

Combined polymyositis and skin changes:
• discoloured papules over knuckles
• violet eye lid rash with local oedema
• erythematous upper body rash

25
Q

What is polymyositis?

A
  • progressive chronic inflammatory or skeletal muscle
  • with other connective tissue disorder (eg. SLE, scleroderma)
  • pain, tender, weak muscles of neck, pharynx, limb girdle
26
Q

What is Sjogren’s syndrome?

A
  • 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
27
Q

What is mixed connective tissue disease?

A 
• autoimmune aetiology
Overlapping clinical features with:
• SLE
• scleroderma
• polymyositis
• Raynaud's phenomenon
28
Q

What is Behcet’s disease/syndrome?

A
  • 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
29
Q

What is polymyalgia rheumatic?

A
  • elderly
  • pain in proximal muscles and joints
  • maybe temporal arteritis

Medical Sciences B (23 decks)

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