Brainscape's Knowledge GenomeTM


Top Flashcards (Certified)
All Flashcards

MSK > Connective Tissue Disease > Flashcards

Connective Tissue Disease Flashcards

(98 cards)

Start Studying
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
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

What are connective tissue diseases?

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

What part of the cell is damaged in SLE?

A

Basement membrane

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

What are the constitutional symptoms of SLE?

A 
Fever
Malaise
Poor appetite
Weight loss
Fatigue
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

What are the cutaneous features of SLE?

A 
Photosensitivity 
Macular rash 
Discoid lupus erythematosus 
Subacute cutaenous lupus
Mouth ulcers
Alopecia
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

What are the MSK features of SLE?

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

What can serositis cause?

A

Pericarditis
Pleurisy
Pleural effusion
Pericardial effusion

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

What renal problems can SLE cause?

A

Proteinuria of >500mg in 24hrs

Red cell casts

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

What neurological features can SLE cause?

A 
Depression/ phycosis
Migrainous headache
Seizures
Cranial or peripheral neurpathy
Mononeuritis multiplex
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

What haematological features can SLE cause?

A 
Lymphadenopathy 
Leucopenia
Lymphopenia
Haemolytic anaemia
Thrombocytopenia
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

What is anti-phospholipid syndrome?

A 
Venous and arterial thrombosis 
Recurrent miscarriage
Livido reticularis 
Thrombocytopenia
Prolonged APTT
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

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

A

Steroids
Other immunosuppressive drugs
Nephrotic syndrome

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

What condition is anti-dsDNA associated with?

A

Lupus nephritis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

What antibody is Anti-Ro commonly associated with?

A

Anti-La

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

What is condition is anti-ro associated with?

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
What condition are anti-Sm antibodies associated with?
SLE but more specificially neurological involvement
26
What conditions are anti-RNP antibodies associated with?
SLE Sclerodermatous skin lesions Raynaud's phenomenon Low grade myositis
27
When SLE is diagnosed what must happen?
``` Screen for organ involvement: CXR Pulmonary function tests CT chest Urine protein quantification Renal biopsy Echocardiogram Nerve conduction studies MRI brain ```
28
How is SLE activity monitored?
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
29
How is SLE managed?
Counselling Regular monitoring Avoid excessive sun exposure Pregnancy issues
30
How is SLE treated pharmacologically?
NSAIDs and simple analgesia Anti-malarials (hydroxychloroquine) for arthritis, cutaneous manifestations and constitutional symptoms Steroids Immunosuppressives
31
When would you give a small dose of steroids in SLE?
Skin rashes Arthritis Serositis
32
When would you give a moderate dose of steroids in SLE?
Resistant serositis | Haematologica abnormalities
33
When would you give a high dose of steroids in SLE?
Severe/ resistant haematologic changes, renal disease and major organ involvement
34
What immunosuppresive agens are utilised in SLE?
Azathioprine Cyclophosphamide Methotrexate Mycophenolate mofetil
35
What are the down falls to immunosuppressives?
Bone marrow suppression Increased susceptibility to infection Teratogenic
36
What biologics are used for SLE?
Anti-CD20 (rituximab) | Anti-Blys (belimumab)
37
What drugs are used for mild SLE disease?
HCQ Topical steroids NSAIDs
38
What drugs are used for moderate SLE disease?
Oral steroids Azathioprine Methotrexate
39
What drugs are used for severe SLE disease?
IV steroids Cyclophophamide Rituximab Belimumab
40
What is a positive titre of ANA?
1:160 and over in almost all SLE patients
41
What are anti-phosphilid antibodies?
Lupus anticoagulants
42
What are the rhuematological autoimmune diseases?
``` SLE Sjogrens syndrome Systemic sclerosis Mixed connective tissue disease Inflammatory myositis Anti-phospholipid antibody syndrome ```
43
When should an autoimmune disease be suspected?
``` Arthralgia/ arthritis Muscle pain/weakness Photosensitivity Raynaud's phenomenon Sicca symptoms (dry eyes/mouth/throat/vaginal) Dysphagia SOB Neurological symptoms Recurrent pregnancy loss ```
44
What is the main general screening antibody for autoimmune conditions?
ANA - high titres are more importnat e.g ANA titre of 1:640 is more significant that ANA of 1:80
45
What antibodies are present in RA?
RF, Anti-CCP
46
What antibodies are present in drug induced SLE?
Anti-histone
47
What antibodies are present in dermatomyositis?
Anti-Jo 1
48
What antibodies are present in limited scleroderma?
Anti-centromere
49
What antibodies are present in diffuse scleroderma?
Anti-Scl 70
50
What antibodies are present in scleroderma renal crisis?
Anti-RNA polymerase 3
51
What antibodies are present in sjogren's syndrome?
Anti-Ro Anti-SSB ANA RF
52
What antibodies are present in congenital heart block?
Anti-Ro
53
What antibodies are present in mixed connective tissue disease?
Anti-U1RNP (dsDNA must be negative)
54
What antibodies are present in microscopic polyangitis?
p-ANCA
55
What antibodies are present in churg strauss?
p-ANCA and esoniphiliaq
56
What antibodies are present in wegners granulomatosis?
c-ANCA
57
What antibodies are present in SLE?
ANA, dsDNA, anti-smith, ribosomal P
58
What are the specific assosiations with RA?
CCP and erosive disease, extra-articular manifestations, smoking
59
What are the specific assosiations with SLE?
Antiphospholipid syndrome, ribosomal P and neurophychatric SLE
60
What are the specific assosiations with drug induced SLE?
Induced by hydralazine, chloropromazine, methydopa, isoniazid, procainamide and anti-TNF
61
What are the specific assosiations with dermatomyositis?
DM and malignancy | Anti-synthetase (ILD, myositis, mechanics hands)
62
What are the specific assosiations with scleroderma?
``` PHT (pulmonary hypertension) ILD (intersitial lung disease) GIT (GI tract problems) Cardiac Renal Renal crisis (hypertension, acute rise in creatning, papilloedema) ```
63
What are the specific assosiations with Sjogren's syndrome?
Sicca symptoms | Risk of lymphoma
64
What are the specific assosiations with mixed connective tissue disease?
Puffy hands
65
What are the specific assosiations with churg strauss?
Asthma Eosinophilia Atopy
66
What are the specific assosiations with wegenrs granulmoatosis?
Granulomas in the renal and pulmonary basement membrane
67
What are the specific assosiations with micrscopic polyangitis?
Pulmonary, renal and segmental necrotizing glomerulonephritis
68
What is the marker of disease activity in RA, drug induced SLE, scleroderma, sjogren's syndrome, microscopic polyangitis, chrug strauss and wegners granulomatosis?
ESR/CRP
69
What is the marker of disease activity in SLE?
Low C4 and C3, anti-dsDNA
70
What is the marker of disease activity in dermatomyositis?
CK
71
What is the marker of disease activity in scleroderma renal crisis?
Creatnine and BP
72
What is the marker of disease activity in congental heart block?
HR
73
What are the stages of raynaud's?
Stage 1: ischaemia Phase 2: cyanosis Phase 3: rubor
74
Who can get primary rayauds?
Teenagers - no underlying autoimmune condition
75
Who can get secondary raynaud's?
Older age group - underlying autoimmune disease | If ulcer or gangrene it is secondary
76
What is the treatment for raynads?
Keep hands warm | Vasodilators - CCB, PDE5 inhibitors (sildenafil)
77
What is sytemic sclerosis?
Skin thickening 90% present with raynaud's Can get diffuse or limited
78
What organs cause systemic sclerosis affect?
Lung Heart Skin
79
What the pattern of distribution of diffuse cutenous SSc?
Skin involvement on extremtities above and below elbows and kness (plus face and trunk)
80
What the pattern of distribution of limited cutenous SSc?
Skin involvement on extremities and only below elbows and knees (plus face)
81
What are the symptoms of scleroderma?
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
What is pulmonary hypertension?
Increased blood pressure in pulmonary artery on right side of heart
83
What autoimmune condition can cause PHT?
Late complication in limited cutaneous systemic sclerosis
84
How is systemic sclerosis treated?
``` 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
What are the clinical associations with anti-centromere antibody?
Limited scleroderma CREST syndrome PHT
86
What are the clinical associations with antitopoisomerase?
Diffuse scleroderma | ILD
87
What is sjogren's syndrome?
Characterised by lymphocytic infiltration of exocrine glands Keratoconjunctivitis siccs
88
What are important questions to ask in the diagnosis of sjogren's syndrome?
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
What are the sympoms of sjogren's syndrome?
``` Blepharitis - swollen eyes Salivary gland inflammation Tooth decay Lymphoma Dry cough Multisystem involvement ```
90
What are the antibodies, blood markers to diagnose sjogren's?
``` ANA positive Anti-Ro and Anti-La Salivary gland ultrasound and biopsy High ESR/PV Raised IgG Cytopaenia ```
91
How is sjogren's treated?
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
What is MCTD a mixture of?
SLE SSc PM (polymyositis) RA
93
What is antiphospholipid syndrome?
Presence of antiphospholipid antibodies on 2 occassions 12 weeks apart Anti-cardiolipin antibody (IgG and IgM) Lupus anticoagulant
94
What are some symptoms of antiphospholipid antibody syndrome?
Thrombosis Recurrent miscarriage Thrombocytopaenia
95
What can cause secondary antiphospholipid syndrome?
Lupus
96
What is the treatment for antiphospholipid antibody syndrome?
Lifelong anticoagulation IF thrombosis
97
What is the treatment for antiphospholipid antibody syndrome pre-pregnancy?
LMWH and aspirin right into the postpartum period
98
What are the general principles of management for autoimmune conditions?
``` 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)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2025 Bold Learning Solutions. Terms and Conditions