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YEAR 1 MED > Connective tissue diseases > Flashcards

Connective tissue diseases Flashcards

1
Q

What is systemic lupus erythematosus (SLE)?

A
  • SLE = an autoimmune disease
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2
Q

What is the epidemiology for SLE and what are some triggers for flare-ups?

A
  • more common in females

Triggers…
- oestrogen-containing contraception (oral contraceptive pill)
- overexposure to sunlight (UV-B light)
- infections
- stress

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3
Q

What are the clinical features of SLE (and what is the most common cause of lupus-related death)?

A
  • general: systemic upset (fever, myalgia, fatigue, weight loss), joint and/or skin involvement
  • dermatological: photosensitive malar rash (butterfly rash across cheeks), ulcers, discoid rash, palpable purpura, livedo reticularis
  • joints: arthritis / arthralgia
  • cardiovascular: Raynauds, pericarditis
  • respiratory: pleural effusions, pneumonitis
  • renal: lupus nephritis (most common cause of lupus-related death)
  • neurological: seizures, migraines, peripheral neuropathies, psychosis/depression/anxiety
  • haematological: anaemia of chronic disease, lymphopenia, haemolytic anaemia, thrombocytopenia, leukopenia
  • GI problems
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4
Q

Classic ‘butterfly’ malar rash of SLE…

A
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5
Q

Discoid lupus lesions, seen in SLE…

A
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6
Q

What are some common secondary diseases to SLE?

A
  • secondary Sjorgen’s syndrome
  • secondary anti-phospholipid syndrome
  • mixed connective tissue disease (features of SLE, systemic sclerosis, and poly/dermatomyositis)
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7
Q

How does drug-induced lupus differ from normal lupus?

A
  • presents as a milder form of SLE
  • triggered by chronic use of certain drugs (symptoms not present before starting drug and stop after discontinuing the drug)
  • Bloods: +ve anti-histone, -ve anti-dsDNA and anti-Sm
  • note: malar rash, renal disease, neurological involvement are rare
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8
Q

What investigations should be done for suspected SLE?

A
  • FBC and clotting screen: anaemia of chronic disease, prolonged partial thromboplastin time (PTT) suggest lupus anticoagulant so prompts need for check of APLS antibodies
  • U+Es: screen for renal involvement
  • ESR / CRP: sugests active disease
  • auto-antibodies: anti-dsDNA, anti-Sm
  • complement lvls: low C3 and C4
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9
Q

What is the management for SLE?

A
  • education: avoid triggers
  • pharmacological: NSAIDs, hydroxychloroquine, corticosteroids (for acute flares), cyclophosphamide (if severe SLE)
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10
Q

What is antiphospholipid syndrome (APLS)?

A
  • APLS = an autoimmune condition characterised by thrombosis (venous/arterial), fetal loss, and thrombocytopenia (associated with raised levels of antiphospholipid antibodies)
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11
Q

What are the clinical features of antiphospholipid syndrome (APLS)?

A
  • Clots: venous thrombosis (eg. DVT or pulmonary), arterial thrombosis (eg. myocardial infarction or stroke)
  • Livedo reticularis: a mottled appearance of skin on lower limbs
  • Obstretic loss: recurrent miscarriages, premature births
  • Thrombocytopenia:
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12
Q

What are the investigations for suspected APLS?

A
  • antibodies: lupus anticoagulant and anticardiolipin
  • FBC: thrombocytopenia
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13
Q

What is the management for APLS?

A
  • Reduce risk factors for thromboembolism: avoid oral-contraceptive pill/HRT, long periods of immobility, smoking
  • Treat hypertension: diuretics, ACE inhibitors
  • Treatment of thrombosis: anticoagulant (eg. warfarin. DOACs)
  • note: warfarin is teratogenic, use low molecular weight heparin during pregnancy
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14
Q

What is Sjorgen sydrome?

A
  • Sjorgen syndrome = an autoimmune disease, characterised by decreased salivary and lacrimal gland secretion
  • manifests as dry eyes and dry mouth
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15
Q

What is Sicca syndrome?

A
  • the presence of dry eyes or mouth as a result of non-autoimmune disease (such as smoking or drugs)
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16
Q

What are the clinical features of Sjorgen syndrome?

A
  • Occular: reduced tear secretion (dry, gritty eyes), bacterial conjunctivitis common
  • Oral: dryness of mouth (xerostomia), can cause dysphagia
  • Other: vaginal dryness, reduced GI mucus secretion
  • Extraglandular features: systemic upset (fever, myalgia, malaise, fatigue), arthritis, Raynauds
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17
Q

What are the investigations for suspected Sjorgen syndrome?

A
  • Bloods: ESR/CRP raised, Rf, anti-Ro/anti-La
  • Schirmer’s test: shows reduction in tear production
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18
Q

Schirmer test…

A
  • one end of a strip of filter paper is placed beneath the lower eyelid
  • wetting the paper by less than 5mm in 5 minutes suggests reduced tear secretion
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19
Q

What is the management for Sjorgen syndrome?

A
  • dry eyes: hypromellose eye drops
  • dry mouth: artificial saliva, drink frequently
  • note: treat other symptoms individually (eg. arthritis - hydroxychloroquine)
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20
Q

What are the clinical features of polymyositis and dermatomyositis?

A
  • MSK features: bilateral, proximal (hip and shoulder girdle) muscle weakness, gradual/progressive onset
  • note: difficulty rising from a chair or walking up stairs, also difficult to reach things above head height
  • extra-MSK: systemic upset, pulmonary, Raynauds, underlying malignancy (10-15% of patients)
  • later symptoms: respiratory muscles (poor ventilation), oesophageal muscles (dysphagia)
  • dermatomyositis: heliotrope rash, Gottron’s papules, macular rash, ulcers
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21
Q

Gottron papules…

A
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22
Q

Heliotrope rash…

A
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23
Q

Macular erythematous rash…

A
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24
Q

What are the investigations for polymyositis and dermatomyositis?

A
  • muscle enzymes (creatine kinase): elevated due to myositis
  • muscle biopsy: most definitive diagnostic test
  • MRI: can locate muscle oedema for biopsy
  • autoantibodies: anit-Jo-1
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25
Q

What is the management for polymyositis and dermatomyositis?

A
  • long-term corticosteroids with methotrexate or azathioprine as a steroid-sparing agent
  • physiotherapy
  • (cyclophosphamide: prescribed for patients with severe interstitial lung disease)
26
Q

What is scleroderma and what is systemic sclerosis?

A
  • scleroderma = hardening of the skin (cutaneous)
  • systemic sclerosis = multi-system autoimmune disease (abnormalities of blood vessels and fibrosis of skin and organs)
27
Q

What are the features of limited cutaneous system sclerosis (CREST syndrome)?

A
  • Calcinosis
  • Raynaud’s phenomenon
  • oEsophageal dysmotility (dysphagia)
  • Sclerodactyly (shiny/tight/swollen skin of hands/feet)
  • Telangiectasia
  • note: symptoms limited to hands/forearms, feet/legs, and head/neck
28
Q

Microstomia (tightness of skin around mouth)…

A
29
Q

Sclerodactyly (localised thickening and tightening of the skin around fingers)…

A
30
Q

What are the features of diffuse systemic sclerosis (and what is the emergency condition involving renal function that can occur)?

A
  • Fx of systemic sclerosis
  • dermatological: skin fibrosis (loss of skin creases, shiny skin), sclerodactyly, telangiectasia, microstomia, calcinosis, flexion contractures at IPs due to skin changes
  • cardiovascular: Raynaud’s phenomenon, pericarditis
  • GI: oesophageal dysmotility, bowel hypomotility
  • respiratory: pulmonary fibrosis, pulmonary hypertension
  • renal: scleroderma renal crisis (monitor renal function)
31
Q

What are the investigations for suspected systemic sclerosis?

A
  • Bloods: anti-centromere (limited), anti-Scl-70 (diffuse)
  • diagnosis is made clinically and confirmed with serology
32
Q

Which type of systemic sclerosis (limited or diffuse) has a better prognosis?

A
  • limited systemic sclerosis has a relatively good prognosis
  • diffuse systemic sclerosis has a relatively poor prognosis
33
Q

What is the management for systemic sclerosis?

A
  • symptomatic treatment
34
Q

What is the management for Raynaud phenomenon?

A
  • Hand warmers (gloves)
  • Vasodilators: calcium-channel blockers, IV prostacyclin (iloprost) for severe ischaemia
35
Q

What is the management for scleroderma renal crisis (medical emergency)?

A
  • Antihypertensives (give immediately): ACE inhibitors, calcium-channel blockers, (temporary dialysis may be required too)
36
Q

What is vasculitis?

A
  • Vasculitis = inflammation of blood vessels
  • it can be primary or secondary (such as with RA or SLE)
37
Q

What are the two severe consequences of vascular inflammation?

A
  • vessel stenosis leading to occlusion and distal infarction (ischaemia)
  • aneurysm formation can lead to rupture of vessels and haemorrhage
38
Q

How are the vasculitides classified?

A

Large-vessel vasculitis:
- Giant cell (temporal) arteritis and polymyalgia rheumatica
- Takayasu arteritis
Medium-vessel vasculitis:
- Polyarteritis nodosa
- Kawasaki disease
Small-vessel vasculitis:
- Granulomatosis with polyangiitis (GPA, or Wegener granulomatosis)
- Eosinophilic granulomatosis with polyangiitis (EGPA, or Churg-Strauss syndrome)
- Microscopic polyangiitis
- Henoch-Schonlein purpura
- Essential cryoglobulinaemic vasculitis

39
Q

What are the symptoms of giant cell arteritis (GCA)?

A
  • severe unilateral headache (temporal headache) and scalp tenderness
  • jaw claudication (pain on chewing food)
  • the temporal artery is thickened and may be pulselessness
  • visual change (optic artery ischaemia, blindness can occur)
  • note: often occurs with PMR
40
Q

What is the management of giant cell arteritis (GCA)?

A
  • high dose corticosteroids urgently: to reduce the risk of blindness
  • followed by 1-2 year gradual taper of corticosteroids
  • note: give bisphosphonates with corticosteroids
41
Q

What are the symptoms of polymyalgia rheumatica (PMR)?

A
  • occurs exclusively in patients over 50 yrs
  • shoulder/hip girdle stiffness and pain (EMS >1hr)
  • examination: no muscle weakness
  • note: polymyositis presents as weakness, not pain
42
Q

What is the management of polymyalgia rheumatica?

A
  • steroids
43
Q

What investigations should be done for suspected GCA and PMR?

A
  • temporal artery biopsy
  • ESR: usually raised
  • Doppler ultrasound
  • FBC: anaemia common
44
Q

What should be prescribed along with longer-term steroid doses?

A
  • bisphosphonates and vitamin D / calcium supplements
  • (to prevent osteoporosis)
45
Q

What is Takayasu arteritis and what is the epidemiology, symptoms, and treatment?

A
  • affects aorta and its main branches (vascular inflammation)
  • most common in Asian women between 10 and 40 yrs
  • symptoms: night sweats, arthritis/myalgia, visual defects, abnormalities in peripheral pulses
  • treatment: steroids
46
Q

What is polyarteritis nodosa and what are the symptoms / treatment?

A
  • most common in men aged 40-60 yrs
  • symptoms: renal failure, coronary (ischaemic heart disease, myocardial infarction), GI (abdo pain, nausea), MSK (arthritis, myalgia), CNS (eye and skin complaints)
  • treatment: corticosteroids, cyclophosphamide (severe cases)
47
Q

What is Kawasaki disease (mucocutaneous lymph node syndrome), epidemiology, symptoms, treatment?

A
  • affects children under the age of 5yrs
  • symptoms: desquamation of the skin of hands/feet, conjunctival congestion, cervical lymphadenopathy, strawberry tongue is characteristic finding
  • treatment: IV immunoglobulin and low-dose aspirin
48
Q

Strawberry tongue (characteristic finding in Kawasaki disease)…

A
49
Q

What is the presentation of granulomatosis with polyangiitis (Wegener’s granulomatosis)?

A

Classic triad…
- upper respiratory tract involvement: chronic sinusitis, epistaxis, saddle-nose deformity
- lower respiratory tract involvement: cough, haemoptysis, pleuritis
- Glomerulonephritis: haematuria, proteinuria (frothy urine)

50
Q

What is the treatment for granulomatosis with polyangiitis (Wegener granulomatosis)?

A
  • milder: methotrexate and azathioprine
  • severe: corticosteroids and cyclophosphamide
51
Q

Saddle nose deformity (seen in GPA)…

A
52
Q

What are the 3 stages seen in eosinophilic granulomatosis with polyangiitis (EGPA, formerly known as Churg-Strauss syndrome)?

A
  • Atopic phase: allergic rhinitis, adult-onset asthma
  • Eosinophilic phase: abnormally high eosinophils in peripheral blood samples (weight loss, night sweats, diarrhoea, wheeze may be present)
  • Vasculitis phase: rashes, peripheral neuropathy, renal failure, abdo pains
  • (note: treatment is the same as it is for GPA)
53
Q

What is microscopic polyangiitis (MPA)?

A
  • one of the more common forms of arteritis affecting smaller arteries (p-ANCA +ve)
  • symptoms: systemic upset, kidney inflamm (glomerulonephritis), skin lesions (palpable purpura), peripheral nerve damage, lung involvement
54
Q

c-ANCA (binds to PR3) and p-ANCA (binds to MPO) antibodies…

A
  • c-ANCA: associated with granulomatosis with polyangiitis (Wegener’s)
  • p-ANCA: associated with eosinophilic granulomatosis with polyangiitis (Churg-Strauss), and microscopic polyangiitis
55
Q

What is Henoch-Schonlein purpura (HSP)?

A
  • typically presents with a palpable purupuric rash on the legs and buttocks in children and adolescents
  • GI involvement (abdo pains)
  • glomerulonephritis (occurs in about 40%): self-limiting
  • treatment: supportive
56
Q

What is the presentation of hypermobility syndrome, diagnosis, and treatment?

A
  • Presentation: joint and ligament injuries, pain, fatigue, thin/stretchy skin, GI issues (diarrhoea or constipation)
  • Diagnosis: Beighton score >4 (out of 9)
  • Treatment: physio and OT (strengthening muscles can protect joints), avoid high-impact sports
57
Q

What are the symptoms of Bechet’s disease and treatment?

A
  • oral and genital ulceration
  • uveitis
  • erythema nodosum
  • treatment: steroids
58
Q

What is Marfan’s syndrome?

A
  • an autosomal dominant CTD caused by mis-sense mutation of the fibrillin 1 gene
59
Q

What are the features of Marfan’s syndrome?

A
  • Physical appearance: tall and thin with long arms and legs, arachnodactyly, high arch palate
  • cardiovascular features: mitral valve prolapse, aortic problems
  • ophthalmic features: lens dislocation, glaucoma
  • MSK features: hypermobility, contractures
60
Q

What is the management for Marfan’s syndrome?

A
  • conservative: education (avoid high-impact exercise)
  • pharmacological: antihypertensives

YEAR 1 MED (49 decks)

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