Autoimmune connective tissue diseases Flashcards

(61 cards)

1
Q

What MSK complication can arising from SLE

A

Osteonecrosis of the femoral head

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2
Q

What hypersensitivity reaction is involved in SLE

A

Type 3
- Immune complexes are deposited in tissue as they fail to clear.

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3
Q

What drugs are associated with lupus

A

Procainamide

Isoniazid

Hydralazine

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4
Q

SLE is more prevalent in which populations?

A

African and Asians

Female> Male (9:1)

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5
Q

Dermatological features of SLE

A

Photosensitive malar rash
- Nasolabia sparing

Discoid lesions

Non-scarring alopecia

Reynaud’s phenomenon

Livedo reticularis

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6
Q

MSK presentation of SLE

A

Migratory joint pain (non-erosive arthritis)
- Knees, wrist, PIPJ

Myalgia

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7
Q

The most common heart manifestations of SLE are…

A

Pericarditis (most common)

Libman Sachs endocarditis ( Nonbacterial thrombotic endocarditis)

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8
Q

The most common renal manifestation of SLE is…

A

Proliferative glomerulonephritis

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9
Q

Neuropsychiatric manifestations of SLE

A

CVA

Mood disorder
- Depression, anxiety

Psychosis

Transver myelitis

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10
Q

Neonatal/ congenital lupus can present with what cardiac manifestation?

A

Complete heart block
- As anti-RO antibodies can cross placenta

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11
Q

The most specific serology for SLE is…

A

Anti-double stranded DNA antibodies

Anti-smith antibodies (ENA)

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12
Q

Antiphospholipid antibodies seen in SLE

A

Anticardiolipin

Lupus anticoagulant

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13
Q

The most sensitive serology for SLE is…

A

Anti-nuclear antibodies (ANA)
- 99% are positive.

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14
Q

What markers can be used to monitor disease activity of SLE

A

ESR

C3, C4 levels
- Lower in acute phase as they are used uo

Anti-dsDNA titres

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15
Q

Pregnant women with SLE have to be given _____ (what and when) to reduce the risk of pre-eclampsia

A

Low dose aspirin from week 12.

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16
Q

Respiratory manifestations of SLE

A

Pleurisy (pleuritic chest pain)

Interstitial fibrosis

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17
Q

First line treatment for non-organ threatening disease in SLE is…

A

Hydroxychloroquine

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18
Q

First line treatment option for organ involvement in SLE is…

A

Cyclophosphamide

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19
Q

Complications of sjogren’s syndrome

A

Eyes
- conjunctivitis
- Corneal ulcers

Oral
- Halitosis
- Dental cavities
- Candida

Vagina
- Vaginitis
- Candidiasis

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20
Q

Extra-glandular presentation of Sjogren’s [5]

A

Joint pain/ arthritis

Vasculitis

Neuropathy

Interstitial lung disease

Nephritis

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21
Q

Causes of secondary Sjogren’s syndrome

A

Rheumatoid arthritis

SLE

systemic sclerosis

Polymyositis

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22
Q

Sjogren’s syndrome is associated with a 40-60 fold increase in…

A

Lymphoid malignancy

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23
Q

Serological diagnosis of Sjogren’s

A

ANA antibodies (70% sensitivity)
- Anti-Ro
- Anti-La

RF

Hypergammaglobulinaemia

Low C4

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24
Q

Histological diagnosis of Sjogren’s syndrome

A

Salivary lymph gland biopsy
- Shows focal lymphocytic infiltrates

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25
Which two ENA antibodies are associated with Sjogren's syndrome
Anti-Ro (SSA) Anti-La (SSB)
26
Schirmer's test ________ is suggestive of insufficient tear production, as seen in Sjogren's syndrome
<10mm
27
Management of Sjogren's syndrome
Artifical saliva and tears Hydroxychloroquine
28
Features of CREST syndrome
C- Calcinosis R- Raynaud's phenomenon E- Esophageal dysmotility S- Sclerodactyly T- Telangectasia
29
Types of systemic sclerosis
Limited cutaneous Diffuse cutaneous
30
Limited cutaneous sclerosis affects (distal/proximal) region and has a _______ progression
Affects distal regions and has slow progression
31
What type of systemic sclerosis presents with CREST syndrome
Limited cutaneous sclerosis
32
Diffuse cutaneous sclerosis affects (distal/proximal) region and has a _______ progression
Affects the proximal region and shows a rapid progression
33
What type of systemic sclerosis has a high risk of renal crisis
Diffuse systemic sclerosis
34
Serology diagnosis for diffuse cutaneous sclerosis
ANA antibodies Anti-scl 70 Anti-RNA polymerase III antibodies
35
Serology diagnosis for limited cutaneous sclerosis
ANA antibodies Anti-centromere antibodies
36
Elevated ___________ is strongly predictive of a _______ in diffuse cutaneous sclerosis
Anti-RNA polymerase III antibodies, strongly predictive of a renal crisis.
37
Raynaud's phenomenon can be managed using...
CCB- Nifedipine
38
ILD in systemic sclerosis can be managed using...
High dose corticosteroid + cyclophosphamide
39
Scleroderma renal crisis can be managed using...
ACE inhibitor
40
Types of idiopathic inflammatory myositis
Inclusion body myositis Dermatomyositis Polymyositis
41
Inclusion body myositis presents with...
Asymmetrical proximal limb weakness + exercise intolerance Diminished deep tendon reflexes Dysphagia
42
Inclusion body myositis presents with _________ muscle weakness, whereas polymyositis presents with _______ muscle weakness
IBM= asymmetrical proximal weakness Polymyositis= symmetrical proximal weakness.
43
Polymyositis features
Symmetrical muscle weakness Bulbar and intercostal muscle weakness
44
Dermatomyositis features
Dermatological - Photosensitive, V-shaped rash (anterior chest) - Heliotrope rash on eyelids Systemic - Fever, arthalgia, weight loss Cardiac - Arrhythmias, DCM Gottron's papules
45
Gottron's papules are associated with ______
Dermatomyositis
46
In myositis, an electromyography would show...
Polyphasic action potentions
47
Myositis will show elevated muscle enzymes, which include...
Creatinine kinase ALT LDH
48
In myositis, serological investigations show elevated...
Anti-nuclear antibodies Anti-synthase antibodies
49
Definite diagnosis of myositis involves...
Muscle biopsy and history
50
Patients diagnosis with myositis have an increased risk of _______ so must be screened
Malignancies
51
Patients with myositis have an increase risk of which malignancies
- Breast - Lung - Ovary
52
_______ is used for the initial pharmacological management of myositis
Corticosteroids (prednisolone)
53
Drug causes of erythema nodosum
Penicillin COCP Phenytoin Sulphonamides
54
Treatment of erythema nodosum
NSAIDs
55
Investigations for sarcoidosis
Rule out TB - Mantoux tuberculin test Bloods - Bone profile= raised calcium and vit D - Raised serum ACE - LFTs- abnormal if liver affected Serology - Raised interleukin-2 receptor ECG Imaging - Chest X-ray - CT Respiratory function test Lymph node biopsy and histology - Non-caseating granuloma with epitheliod cells.
56
CXR in sarcoidosis will show...
Bilateral hilar lymphadenopathy
57
What group of people are most commonly affected by sarcoidosis?
Age 20-40 Afro-caribbean Women
58
First-line management of sarcoidosis
Oral corticosteroids
59
Second line management in sarcoidosis
DMARDs - Methotrexate - Azathioprine - Leflunomide - Mycophenolate
60
What are the features of Lofgren's syndrome
Seen in sarcoidosis: - Erythema nodosum - Bilateral hilar lymphadenopathy - Polyarthralgia
61
What are the features of Heerfordt's syndrome
Seen in sarcoidosis: - Submandibular/ parotid gland inflammation - Uveitis - CN7 palsy