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MSK > Connective Tissue Disease > Flashcards

Connective Tissue Disease Flashcards

1
Q

What are some common connective tissue diseases?

A 
SLE
Sjogren's syndrome
Systemic sclerosis
Dermatomyositis
Polymyositis
Mixed connective tissue disease
Anti-phospholipid syndrome
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2
Q

What are connective tissue diseases?

A

Spontaneous over actiivty of the immune system that has specific auto-antibodies

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3
Q

What is the epidemiology of SLE?

A

Females to males 9:1
Prevalence is higher in asians, afro-americans, afro-caribbeans
Uncommon in african blacks

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4
Q

What hormonal factors can lead to SLE?

A

Incidence increased in those with higher oestrogen exposure - early menarche, on oestrogen containing contraceptives and HRT

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5
Q

What is the pathogenesis of SLE?

A

Increased and defective apoptosis
Necrotic cells release nuclear material which act as auto-antigens
Autoimmunity results from exposure to nuclear and intracellular auto-antigens
B and T cells stimulated
Autoantibodies produced

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6
Q

How does SLE cause renal disease?

A

Deposition of immune complexes in mesangium (complexes of nuclear antigens and anti-nuclear antibodies)
Complexes form in circulation then are deposited
Activate complement which attracts leucocytes which release cytokines
Cytokine release perpetuates inflammation which causes necrosis and scarring

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7
Q

What part of the cell is damaged in SLE?

A

Basement membrane

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8
Q

What is the clinical critera for SLE?

A 
Acute cutaneous lupus
Chronic cutaneous lupus
Oral or nasal ulcerrs
Non-scarring alopecia
Arthritis
Serositis
Renal
Neurologic
Haemolytic anaemia
Leukopenia
Thrombocytopenia
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9
Q

What is the immunological critera for SLE?

A 
ANA
Anti-DNA
Anti-Sm
Antiphospholipid Ab
Low complement (C3, C4, CH50)
Direct coombs test
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10
Q

What are the constitutional symptoms of SLE?

A 
Fever
Malaise
Poor appetite
Weight loss
Fatigue
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11
Q

What are the cutaneous features of SLE?

A 
Photosensitivity 
Macular rash 
Discoid lupus erythematosus 
Subacute cutaenous lupus
Mouth ulcers
Alopecia
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12
Q

What are the MSK features of SLE?

A

Non-deforming polyarhritis/ polyarthralgia
Deforming rthropathy - jaccoud’s arthritis
Myopathy - weakness, myalgia and myositis

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13
Q

What can serositis cause?

A

Pericarditis
Pleurisy
Pleural effusion
Pericardial effusion

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14
Q

What renal problems can SLE cause?

A

Proteinuria of >500mg in 24hrs

Red cell casts

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15
Q

What neurological features can SLE cause?

A 
Depression/ phycosis
Migrainous headache
Seizures
Cranial or peripheral neurpathy
Mononeuritis multiplex
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16
Q

What haematological features can SLE cause?

A 
Lymphadenopathy 
Leucopenia
Lymphopenia
Haemolytic anaemia
Thrombocytopenia
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17
Q

What is anti-phospholipid syndrome?

A 
Venous and arterial thrombosis 
Recurrent miscarriage
Livido reticularis 
Thrombocytopenia
Prolonged APTT
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18
Q

What intrinsic features can make patients with connective tissue disorders more sucepitble to infection?

A

Low complement system
Impaired cell mediated immunity
Defective phagocytosis
Poor antobidy response to antigens

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19
Q

What extrinsic features can make patients with connective tissue disorders more sucepitble to infection?

A

Steroids
Other immunosuppressive drugs
Nephrotic syndrome

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20
Q

When should a positive ANA test be taken seriously in SLE?

A

If other antinuclear antibodies are positive - anti-dsDNA, anti-Sm, Anti-Ro, Anti-RNP
When the patient precents with clinical features

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21
Q

What is anti-double stranded DNA antibody (anti-dsDNA)?

A

Occurs in 60% of patients with SLE but is highly specific for SLE
Titre correlates with overall disease activity

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22
Q

What condition is anti-dsDNA associated with?

A

Lupus nephritis

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23
Q

What antibody is Anti-Ro commonly associated with?

A

Anti-La

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24
Q

What is condition is anti-ro associated with?

A

Cutaneous manifestations
Secondary sjogren’s features
Congenital heart block and neonatal LE

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25
Q

What condition are anti-Sm antibodies associated with?

A

SLE but more specificially neurological involvement

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26
Q

What conditions are anti-RNP antibodies associated with?

A

SLE
Sclerodermatous skin lesions
Raynaud’s phenomenon
Low grade myositis

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27
Q

When SLE is diagnosed what must happen?

A 
Screen for organ involvement: 
CXR
Pulmonary function tests
CT chest
Urine protein quantification 
Renal biopsy 
Echocardiogram
Nerve conduction studies
MRI brain
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28
Q

How is SLE activity monitored?

A

Anti-dnDNA levels positevly correlate with activity of disease
C3/C4 levels negatively correlate with activity
Urine examination - protein, cells and casts
FBC
Blood biochem

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29
Q

How is SLE managed?

A

Counselling
Regular monitoring
Avoid excessive sun exposure
Pregnancy issues

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30
Q

How is SLE treated pharmacologically?

A

NSAIDs and simple analgesia
Anti-malarials (hydroxychloroquine) for arthritis, cutaneous manifestations and constitutional symptoms
Steroids
Immunosuppressives

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31
Q

When would you give a small dose of steroids in SLE?

A

Skin rashes
Arthritis
Serositis

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32
Q

When would you give a moderate dose of steroids in SLE?

A

Resistant serositis

Haematologica abnormalities

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33
Q

When would you give a high dose of steroids in SLE?

A

Severe/ resistant haematologic changes, renal disease and major organ involvement

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34
Q

What immunosuppresive agens are utilised in SLE?

A

Azathioprine
Cyclophosphamide
Methotrexate
Mycophenolate mofetil

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35
Q

What are the down falls to immunosuppressives?

A

Bone marrow suppression
Increased susceptibility to infection
Teratogenic

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36
Q

What biologics are used for SLE?

A

Anti-CD20 (rituximab)

Anti-Blys (belimumab)

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37
Q

What drugs are used for mild SLE disease?

A

HCQ
Topical steroids
NSAIDs

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38
Q

What drugs are used for moderate SLE disease?

A

Oral steroids
Azathioprine
Methotrexate

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39
Q

What drugs are used for severe SLE disease?

A

IV steroids
Cyclophophamide
Rituximab
Belimumab

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40
Q

What is a positive titre of ANA?

A

1:160 and over in almost all SLE patients

41
Q

What are anti-phosphilid antibodies?

A

Lupus anticoagulants

42
Q

What are the rhuematological autoimmune diseases?

A 
SLE
Sjogrens syndrome
Systemic sclerosis
Mixed connective tissue disease 
Inflammatory myositis
Anti-phospholipid antibody syndrome
43
Q

When should an autoimmune disease be suspected?

A 
Arthralgia/ arthritis
Muscle pain/weakness
Photosensitivity
Raynaud's phenomenon
Sicca symptoms (dry eyes/mouth/throat/vaginal)
Dysphagia
SOB
Neurological symptoms
Recurrent pregnancy loss
44
Q

What is the main general screening antibody for autoimmune conditions?

A

ANA - high titres are more importnat e.g ANA titre of 1:640 is more significant that ANA of 1:80

45
Q

What antibodies are present in RA?

A

RF, Anti-CCP

46
Q

What antibodies are present in drug induced SLE?

A

Anti-histone

47
Q

What antibodies are present in dermatomyositis?

A

Anti-Jo 1

48
Q

What antibodies are present in limited scleroderma?

A

Anti-centromere

49
Q

What antibodies are present in diffuse scleroderma?

A

Anti-Scl 70

50
Q

What antibodies are present in scleroderma renal crisis?

A

Anti-RNA polymerase 3

51
Q

What antibodies are present in sjogren’s syndrome?

A

Anti-Ro
Anti-SSB
ANA
RF

52
Q

What antibodies are present in congenital heart block?

A

Anti-Ro

53
Q

What antibodies are present in mixed connective tissue disease?

A

Anti-U1RNP (dsDNA must be negative)

54
Q

What antibodies are present in microscopic polyangitis?

A

p-ANCA

55
Q

What antibodies are present in churg strauss?

A

p-ANCA and esoniphiliaq

56
Q

What antibodies are present in wegners granulomatosis?

A

c-ANCA

57
Q

What antibodies are present in SLE?

A

ANA, dsDNA, anti-smith, ribosomal P

58
Q

What are the specific assosiations with RA?

A

CCP and erosive disease, extra-articular manifestations, smoking

59
Q

What are the specific assosiations with SLE?

A

Antiphospholipid syndrome, ribosomal P and neurophychatric SLE

60
Q

What are the specific assosiations with drug induced SLE?

A

Induced by hydralazine, chloropromazine, methydopa, isoniazid, procainamide and anti-TNF

61
Q

What are the specific assosiations with dermatomyositis?

A

DM and malignancy

Anti-synthetase (ILD, myositis, mechanics hands)

62
Q

What are the specific assosiations with scleroderma?

A 
PHT (pulmonary hypertension)
ILD (intersitial lung disease)
GIT (GI tract problems) 
Cardiac 
Renal
Renal crisis (hypertension, acute rise in creatning, papilloedema)
63
Q

What are the specific assosiations with Sjogren’s syndrome?

A

Sicca symptoms

Risk of lymphoma

64
Q

What are the specific assosiations with mixed connective tissue disease?

A

Puffy hands

65
Q

What are the specific assosiations with churg strauss?

A

Asthma
Eosinophilia
Atopy

66
Q

What are the specific assosiations with wegenrs granulmoatosis?

A

Granulomas in the renal and pulmonary basement membrane

67
Q

What are the specific assosiations with micrscopic polyangitis?

A

Pulmonary, renal and segmental necrotizing glomerulonephritis

68
Q

What is the marker of disease activity in RA, drug induced SLE, scleroderma, sjogren’s syndrome, microscopic polyangitis, chrug strauss and wegners granulomatosis?

A

ESR/CRP

69
Q

What is the marker of disease activity in SLE?

A

Low C4 and C3, anti-dsDNA

70
Q

What is the marker of disease activity in dermatomyositis?

A

CK

71
Q

What is the marker of disease activity in scleroderma renal crisis?

A

Creatnine and BP

72
Q

What is the marker of disease activity in congental heart block?

A

HR

73
Q

What are the stages of raynaud’s?

A

Stage 1: ischaemia
Phase 2: cyanosis
Phase 3: rubor

74
Q

Who can get primary rayauds?

A

Teenagers - no underlying autoimmune condition

75
Q

Who can get secondary raynaud’s?

A

Older age group - underlying autoimmune disease

If ulcer or gangrene it is secondary

76
Q

What is the treatment for raynads?

A

Keep hands warm

Vasodilators - CCB, PDE5 inhibitors (sildenafil)

77
Q

What is sytemic sclerosis?

A

Skin thickening
90% present with raynaud’s
Can get diffuse or limited

78
Q

What organs cause systemic sclerosis affect?

A

Lung
Heart
Skin

79
Q

What the pattern of distribution of diffuse cutenous SSc?

A

Skin involvement on extremtities above and below elbows and kness (plus face and trunk)

80
Q

What the pattern of distribution of limited cutenous SSc?

A

Skin involvement on extremities and only below elbows and knees (plus face)

81
Q

What are the symptoms of scleroderma?

A

CREST
Calcinosis (calcium deposits in skin)
Raynaud’s phenomenon (spasm of blood vessels in response to cold)
Esophageal dysfunction (acid reflux and decrease in oesophagus motility)
Sclerodactyly (thickening and tightening of skin on fingers)
Telangiectasias (dilation of capillaries causing red marks)

82
Q

What is pulmonary hypertension?

A

Increased blood pressure in pulmonary artery on right side of heart

83
Q

What autoimmune condition can cause PHT?

A

Late complication in limited cutaneous systemic sclerosis

84
Q

How is systemic sclerosis treated?

A 
Yearly ECHO and PFTs
Treat raynaud's 
Treat reflux with PPI
Pulmonary fibrosis - immunosuppression 
PHT - postacyclin analogues, endothelin receptor antaonists
Tight control of BP - ACEI
85
Q

What are the clinical associations with anti-centromere antibody?

A

Limited scleroderma
CREST syndrome
PHT

86
Q

What are the clinical associations with antitopoisomerase?

A

Diffuse scleroderma

ILD

87
Q

What is sjogren’s syndrome?

A

Characterised by lymphocytic infiltration of exocrine glands
Keratoconjunctivitis siccs

88
Q

What are important questions to ask in the diagnosis of sjogren’s syndrome?

A

Gritty eyes?
Woken up in the night to get a glass of water?
Found it difficult to swallow bread ot have to drink water to help swallowing?
Any vaginal dryness (pre-menopausal women)?

89
Q

What are the sympoms of sjogren’s syndrome?

A 
Blepharitis - swollen eyes
Salivary gland inflammation 
Tooth decay
Lymphoma
Dry cough
Multisystem involvement
90
Q

What are the antibodies, blood markers to diagnose sjogren’s?

A 
ANA positive
Anti-Ro and Anti-La
Salivary gland ultrasound and biopsy
High ESR/PV
Raised IgG
Cytopaenia
91
Q

How is sjogren’s treated?

A

Theraputic spectrum
Artifical tears, salivary supplements and vaginal lubricants
Good dental hygiene - high flouride toothpaste
Hydrocychloroquine for fatigue and arthralgia
Immunosuppression for major organ involvement

92
Q

What is MCTD a mixture of?

A

SLE
SSc
PM (polymyositis)
RA

93
Q

What is antiphospholipid syndrome?

A

Presence of antiphospholipid antibodies on 2 occassions 12 weeks apart
Anti-cardiolipin antibody (IgG and IgM)
Lupus anticoagulant

94
Q

What are some symptoms of antiphospholipid antibody syndrome?

A

Thrombosis
Recurrent miscarriage
Thrombocytopaenia

95
Q

What can cause secondary antiphospholipid syndrome?

A

Lupus

96
Q

What is the treatment for antiphospholipid antibody syndrome?

A

Lifelong anticoagulation IF thrombosis

97
Q

What is the treatment for antiphospholipid antibody syndrome pre-pregnancy?

A

LMWH and aspirin right into the postpartum period

98
Q

What are the general principles of management for autoimmune conditions?

A 
Assess severity
URINALYSIS
CXR, Pulmonary function, ECHO
Manage CV risk 
Treatment based on major organ involvement
If yes- immunosuppression 
If no - HCQ and symptomatic management

MSK (34 decks)

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