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cp3 @ uon - musculoskeletal & mdd > Multisystem Connective Tissue Disease > Flashcards

Multisystem Connective Tissue Disease Flashcards

1
Q

What is SLE?

A

Common autoimmune condition directed at a range of tissues

-unknown aetiology

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2
Q

What systems are involved in SLE?

A 
Skin (75%)
CNS (60%)
Pulmonary (50%)
Renal (30%)
Cardiac (25%)
Oral mucosal (20%)
Haematological
MSK
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3
Q

What is the most common feature of SLE?

A

Fatigue

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4
Q

What are the skin features of SLE?

A 
Chronic discoid LE
   -red, scaly plaque on face/scalp
Malar rash
   -symmetrical, raised erythematous rash across cheeks/bridge of nose
Photosensitivity reactions
Alopecia
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5
Q

What are the oral mucosal features of SLE?

A

Superficial erosions/ulcers

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6
Q

What are the CNS features of SLE?

A

Non-organic disorders (depression/anxiety)
Organic brain disorders (tonic-clonic epilepsy)
Vascular occlusion & infarction w/ APL syndrome

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7
Q

What are the renal features of SLE?

A

Glomerular disease

  • follows many patterns
  • commonly interstitial nephritis
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8
Q

What are the haematological features of SLE?

A

Thrombocytopenia
Leukopenia
AI haemolytic anaemia
-normocytic hypochromic anaemia

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9
Q

What are the MSK features of SLE?

A

Arthritis (90%)
-begins in fingers, wrists & knees
Myalgia (50%)

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10
Q

What are the pulmonary features of SLE?

A

Recurrent pleurisy & pleural effusions

Pulmonary fibrosis

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11
Q

What are the cardiac features of SLE?

A

Pericarditis
-rarer lesions are aortic valve lesions/cardiomyopathy
Atherosclerotic risk

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12
Q

What bloods are appropriate in suspected SLE?

A 
FBC (normo hypo anaemia, thrombocytopenia, leukopenia)
ESR/CRP (raised)
U&Es
Serum ANA/RF
Anti-dsDNA
RNa antibodies (anti Ro/anti-La)
Serum complement levels
APL antibodies
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13
Q

What investigations are appropriate in suspected SLE?

A

Bloods
Urine dip/BP
Histology (fibrinoid necrosis)

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14
Q

What are the management options for SLE?

A

Avoid excessive sunlight/CV risk factors
Monitor for signs of infection/treat early
MILD DISEASE
-NSAIDs & hydroxychloroquine
SEVERE DISEASE
-Prednisolone w/ DMARDs (azathioprine)

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15
Q

What is Antiphospholipid Syndrome?

A

Presence of autoantibodies that have a specificity for phospholipids

  • thrombotic tendeny
  • can be 1o or occur 2o to SLE
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16
Q

What are the common clinical features of APL syndrome?

A 
Venous thrombosis (arms/legs, ?PE)
Arterial thrombosis (less common, strokes in young ppl)
Pregnancy loss (2nd/3rd trimester)
Livedo reticularis
Thrombocytopenia
Migraines
Epilepsy
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17
Q

What investigations may be appropriate in suspected APL syndrome?

A

ESR (normal)
ANA (-ve)
APTT (inc)
Coomb’s (+ve)
Anticardiolipin antibodies
-high titres on 2 occasions, >12wks apart
Lupus anticoagulant antibodies (+ve in 20%)

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18
Q

What are the management options for APL syndrome?

A 
Warfarin (target INR 3-4) - if hx of severe thrombosis
Low dose aspirin
Lifestyle advice
   -avoid prolonged immobilisation
   -oestrogen containing drugs
LMWH & aspirin given in pregnancy
   -early delivery planned
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19
Q

What is the long term prognosis of APL syndrome?

A

Poor

-1/3 have organ damage w/i 10yrs of diagnosis

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20
Q

What is primary Sjogren’s syndrome?

A

Dry eyes (keratoconjunctivitis sicca) in absence of any autoimmune disease

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21
Q

What is secondary Sjogren’s syndrome?

A

Presence of sicca sx alongside AI disease

-most commonly RA, SLE, scleroderma or polymyositis

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22
Q

What phenotype is Sjogren’s associated with?

A

HLA-B8/DR3

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23
Q

How does Sjogren’s present?

A 
Dry eyes & mouth
Salivary/parotid gland enlargement
Vaginal dryness
Systemic features
   -arthralgia
   -raynaud's
   -oesophageal motility issues
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24
Q

What are the appropriate investigations in suspected Sjogren’s?

A 
Schirmer tear test
   -filter paper placed inside lower eyelid
   -<1cm in 5mins = defective tear production
Bloods
   -RF (raised)
   -ANA (raised)
   -anti-RO (+ve)
   -anti-LA (+ve)
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25
Q

What are the management options for Sjogren’s?

A

Artificial tears

Saliva-replacement

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26
Q

What is the prognosis for Sjogren’s?

A

Good
-salivary & lacrimal function stabilises
1/6 develop Non-Hodgkin’s B-cell lymphoma

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27
Q

What is systemic sclerosis?

A

Multisystem disease characterised by skin sclerosis & Reynaud’s disease

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28
Q

What is the underlying pathophysiology of systemic sclerosis?

A

Perivascular fibrosis leads to ischaemic damage

  • skin most commonly affected (dermal thickening)
  • major organs also affected (lung, heart, oesophagus, kidneys)
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29
Q

What are the two main subtypes of systemic sclerosis?

A

Limited cutaneous scleroderma (70%)

Diffuse cutaneous scleroderma (30%)

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30
Q

How do patients w/ limited cutaneous scleroderma present?

A 
Long hx of Reynaud's &amp; skin tightening at extremities
   -microstomia if face involved
EARLY SX
   -systemic sx (fatigue)
   -GORD
   -ulcers on digital tips
LATER SX
   -oesophageal sx
   -small bowel malabsorption
   -pulmonary fibrosis/pulmonary HTN
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31
Q

What are the management options for limited cutaneous scleroderma?

A

Digital sympathectomy/vasodilators (Reynaud’s)
Removal of calcinoses
Treatment of oesophageal problems

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32
Q

How do patients w/ diffuse cutaneous scleroderma present?

A

Short hx of Reynaud’s w/ rapidly progressive skin sclerosis
-peaks at 2yrs
Severe systemic sx (lethargy, wt loss, anorexia)

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33
Q

What are the common complications of diffuse cutaneous scleroderma?

A

Myocardial fibrosis
Pulmonary fibrosis
Renal fibrosis
OCCUR W/I 3 YEARS

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34
Q

What are the management options for diffuse cutaneous scleroderma?

A

Immunosuppression

Sympathectomy + vasodilators

35
Q

What investigations are appropriate in limited cutaneous scleroderma?

A

Anti-centromere antibodies +ve (60%)

36
Q

What investigations are appropriate in diffuse cutaneous scleroderma?

A

Anti-Scl-70 antibodies (40%)

RNA polymerase antibodies

37
Q

What is the prognosis of systemic sclerosis?

A

Highest mortality of all AI rheumatic diseases

-limited cutaneous scleroderma has higher survival (70% at 10yrs)

38
Q

What phenotype are Polymyositis & Dermatomyositis associated with?

A

HLA-B8/DR3 phenotype

39
Q

What is Polymyositis?

A

Inflammation of striated muscle causing proximal muscle weakness

40
Q

How does Polymyositis present?

A 
Weakness in absence of any pain
   -associated wasting
   -insidious/acute onset
Malaise
Wt loss
Fever
Difficulty squatting/climbing stairs
41
Q

What is the major complication of Polymyositis?

A

Respiratory failure due to respiratory muscle weakness

42
Q

What is Dermatomyositis?

A

Polymyositis w/ associated skin involvement

  • classic heliotropic rash
  • associated periorbital oedema
  • vasculitic patches over knucles (Gottron’s papules)
43
Q

Which conditions are Polymyositis/Dermatomyositis associated with?

A

SLE
RA
Systemic sclerosis
Various malignancies

44
Q

What investigations are appropriate in suspected Polymyositis/Dermatomyositis?

A 
Bloods
   -CK (raised)
   -ESR (rarely raised)
   -ANA (+ve)
   -RF (50% +ve)
   -Myositis specific antibodies
Electromyography
MRI
Needle muscle biopsy
Malignancy screen
45
Q

What are the management options for Polymyositis/Dermatomyositis?

A

Prednisolone/DMARDs

IVIG therapy

46
Q

What is Inclusion Body Myositis?

A

Condition affecting white males >50yrs w/ insidious onset of proximal & distal muscle wasting
-may be asymmetrical

47
Q

What investigations are appropriate in suspected Inclusion Body Myositis?

A

ANA (sometimes +ve)
Myositis specific antigens (not +ve)
Muscle biopsy

48
Q

What is the prognosis of Inclusion Body Myositis?

A

Progressive

Rarely responds to prednislone/DMARD

49
Q

What are Polymalgia Rheumatic & Giant Cell Arteritis?

A

Common systemic disease of elderly

-types of large vessel vasculitis associated w/ giant cell arteritis on temporal artery biopsy

50
Q

How do PMR & GCA relate?

A

15-30% of pts w/ PMR develop CGA

50-70% of pts w/ GCA develop PMR

51
Q

How does PMR present?

A

Pt always <50yrs
Sudden onset severe pain & stiffness in shoulders/neck/hips/lumbar spine
-worse in morning, lasts >30mins
Systemic sx (fatigue, fever, wt loss)

52
Q

What are the signs on examination of PMR?

A

Muscles tender to palpation

-especially in upper arm

53
Q

What sx are required for the diagnosis of PMR?

A

Bilateral shoulder/pelvic girdle aching alongside morning stiffness/evidence of acute phase response present >2wks

54
Q

How does GCA present?

A

Severe headaches

Scalp/temporal tenderness (alongside PMR)

55
Q

What causes the sx of PMR?

A

Synovial & peri-articular inflammation

56
Q

What causes the sx of GCA?

A

Granulomatous vasculitis of medium & larger arteries

57
Q

What are the signs on examination of GCA?

A

Tenderness/swelling of one/both temporal arteries

-inflammation of overlying scalp

58
Q

What is the main complication of GCA?

A

Sudden painless loss of vision

-due to ischaemic of optic nerve

59
Q

What investigations are appropriate in suspected PMR/GCA?

A 
Normocytic/normochromic anaemia
ESR/CRP (raised)
ALP/GGT (raised)
RF/ANA/anti-CCP (-ve)
CK (-ve)
MRI/USS (peri-articular inflammation)
60
Q

What is the definitive diagnostic test for GCA?

A

Temporal artery biopsy

  • intimal hypertrophy w/ giant cells & inflam infiltrate
  • skip lesions occur
61
Q

What are the management options for PMR/GCA?

A 
Oral prednisolone (high dose initially)
   -PMR = 15mg OD
   -GCA = 60mg OD (40 mg OD if no visual sx)
Bone protection &amp; PPIs
62
Q

How does PMR/GCA respond to prednisolone?

A

Response to steroids usually rapid

  • if no response w/i 48hrs consider alternative dx
  • dose reduction in line w/ disease response
63
Q

How long is treatment required for PMR/GCA?

A 
PMR = 2yrs
GCA = 5yrs
64
Q

What complication should all patients w/ GCA be screened for?

A

Aortic/large vessel involvement

65
Q

What systemic sx suggest a possible vasculitic condition?

A 
General (malaise, fever, wt loss, myalgia, arthralgia)
Skin (palpable purpura, ulceration)
GI (mouth ulcers, abdo pain, diarrhoea)
Resp (haemoptysis, dyspnoea)
ENT (epistaxis, crusting)
Cardiac (chest pain)
Neuropathies
66
Q

How can vasculitic conditions be classified?

A 
Primary
Secondary
   -malignancy
   -infection
   -CTDs (SLE most common)
According to size of vessel
67
Q

What are the vasculitic conditions affecting large arteries?

A

GCA
Tajayasu’s arthritis
-young adults w/ upper limb claudication/stroke

68
Q

What are the vasculitic conditions affecting medium arteries?

A

Classic Polyarteritis Nodosa
-multi-systemic sx, multiple microaneurysms
Kawasaki Disease
-fever, rash, lymphandeopathy, palmar erythema in children
-main complication is coronary artery aneurysm

69
Q

What are the vasculitic conditions affecting small vessels (ANCA +ve)?

A 
Wegener's granulomatosis
   -affects lung, kidneys, ENT systems
   -granuloma formation on biopsies
Churg-Strauss syndrome
   -late onset asthma/atopy
   -cardio-pulmonary involvement
Microscopic Polyangiitis
   -pulmonary-real syndrome (haemoptyiss/haematuria)
70
Q

What are the vasculitic conditions affecting small vessels (leucocytoclastic)?

A

HSP
Cryoglobulinaemia
-associated w/ hep C
-rash, arthralgia, neuropathy

71
Q

What are the appropriate investigations in suspected vasculitic conditions?

A 
BP
Urine dip (MC&amp;S)
Bloods
   -FBC (leukocytosis in 1o disease, leukopenia in CTD)
   -LFTs (hepatitis is common 2o cause)
   -inflammatory markers
   -immunology
Tissue biopsy
72
Q

What are the management options for vasculitic conditions?

A 
Depends on size of vessel involved
Corticosteroids
   -prolonged dose reduction over 12mo
Cyclophosphamide
   -ANCA +ve disease
   -can lead to infertility
IVIG (Kawasaki's)
Plasma exchange (if life threatening)
73
Q

What is Synovial Fluid Analysis used for?

A

Diagnosis of acute bacterial sepsis OR crystal associated disease
-often combined w/ corticosteroid injection

74
Q

How can Synovial Fluid Analysis aid diagnosis?

A

Colour
-turbid in inflammation, analyse w/ urgent gram stain & culture
Viscosity
-decreases in inflammation
Blood Stained
-due to trauma, severe inflammation, bleeding tendencies
-if non-uniform indicates traumatic aspiration

75
Q

What FBC abnormalities may be present in rheumatological disease?

A 
Thrombocytopenia
   -disease activity in SLE/APL
   -Felty's syndrome in RA
   -methotrexate
Leukopenia
   -lymphopenia in lupus
   -leucocytosis in flare SLE
Anaemia
   -chronic disease
   -iron deficiency
   -megaloblastic anaemia
   -haemolysis
76
Q

What LFT abnormalities may be present in rheumatological disease?

A

ALP

-elevated in RA/polymyositis/bone disease

77
Q

What U&Es abnormalities may be present in rheumatological disease?

A

Abnormal due to glomerulonephritis in CTDs

-urine dip better indicator of pathology

78
Q

What RF abnormalities may be present in rheumatological disease?

A

Positive in 70% of RA pts (78% specific)

-raised in SLE/Sjogrens

79
Q

What is RF?

A

IgM antibody against IGG, forms immune complexes contributing to disease process

  • indicates worse prognosis
  • transiently raised in infections
80
Q

What anti-CCP abnormalities may be present in rheumatological disease?

A

Positive in 60% of RA pts (90-98% specific)

  • positive in 40% of pts that are RF -ve
  • may precede onset of RA by 12yrs
  • indicates worse prognosis if raised at presentation
81
Q

What ANA abnormalities may be present in rheumatological disease?

A

Titres >1 in 80 = +ve BUT >320 = AI disease

  • useful to rule out lupus (nearly 100% ANA +ve)
  • raised in RA, Sjogren’s, scleroderma, polymyositis
82
Q

What anti ds-DNA abnormalities may be present in rheumatological disease?

A

Specific for lupus

  • only present in severe disease
  • marker of poor prognosis
  • changes w/ disease activity
83
Q

What extractable nuclear antigen abnormalities may be present in rheumatological disease?

A

Anti-SM specific to SLE
Anti-Ro & Anti-La (SS1 & SS2)
-present in Sjogren’s
Can indicate specific tissue involves
-anti-Jo1 in myositis = pulmonary fibrosis
-anti-topoisomerase in scleroderma = pulmonary/cardiac involvement

cp3 @ uon - musculoskeletal & mdd (26 decks)

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