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Phase 4 > Connective tissue diseases > Flashcards

Connective tissue diseases Flashcards

1
Q

Which antibodies are typically found in systemic lupus erythematosus?

A

ANA and anti-DNA antibodies

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2
Q

Is SLE more common in women or men?

A

Women

F>M, 9:1

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3
Q

What age are people most likely to develop SLE?

A

Child-bearing age

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4
Q

What is the pathophysiology of SLE?

A

Antigen-driven immune-mediated disease. Immunoglobulin G antibodies to double-stranded DNA as well as nuclear proteins.
Tolerance to self antigens in the B-cell pool is maintained.
T-helper cell dysregulation of B cells may arise - resulting in autoimmunity.

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5
Q

What are the non-specific symptoms and signs of SLE?

A 
Malaise
Weight loss
Myalgia
Fever
Fatigue
Non-tender regional lymph nodes
Seizures
Depression
Cranial nerve issues
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6
Q

What are the specific symptoms and signs of SLE?

A 
Malar rash
Discoid rash 
Photosensitivity
Renal failure
Arthritis
Oral ulcers
Serositis
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7
Q

What are the diagnosis criteria for SLE?

A

4 or more criteria, at least one clinical and one laboratory criteria, OR biopsy-proven lupus nephritis with positive ANA or anti-DNA.

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8
Q

What are the clinical criteria for SLE?

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

What are the immunological criteria for SLE?

A 
ANA
Anti-DNA
Anti-Sm
Antiphospholipid antibody
Low complement
Direct Coombs test
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10
Q

What is the management for an acute flare of SLE?

A

Hydroxychloroquine if serositis/joint involvement.
Cyclophosphamide and high dose prednisolone if renal involvement/CNS.
NSAIDs

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11
Q

What is the long term management of SLE?

A

NSAIDs
Hydroxychloroquine
Low dose steroids
Azathioprine, methotrexate, mycophenolate.

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12
Q

What is the pathophysiology of anti-phospholipid syndrome?

A

Antibodies to phospholipid binding plasma proteins on platelets and monocytes results in the development of venous, arterial, and microvascular thromboses, and/or pregnancy associated morbidity.

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13
Q

How is anti-phospholipid syndrome diagnosed?

A

If at leaset 1 vascular thrombosis or pregnancy morbidity is present in association with the presence of anti-phospholipid antibodies on 2 or more occasions, 12 weeks apart.

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14
Q

How is pregnancy morbidity defined?

A

The loss of 3 or more embryos before the 10th week of gestation.
And/or 1 or more otherwise unexplained fetal deaths beyond the 10th week of gestation.
And/or the premature birth of a morphologically normal neonate before the 34th week of gestation because of eclampsia, severe pre-eclampsia, or placental insufficiency.

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15
Q

Which investigations would be carried out to test for anti-phospholipid syndrome?

A

Lupus anticoagulant
ANA and anti-double stranded DNA antibodies.
Anticardiolipid antibodies.
FBC may show thrombocytopenia.
Urea and creatinine may be increased if nephropathy.
Doppler/CTPA or MR angio
Echo - heart valve abnormalities

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16
Q

How is antiphospholipid syndrome managed?

A

Treat thrombosis - low molecular weight heparin and then warfarin after.
If incidental finding of APL antibodies then watch and wait unless pregnant and need aspirin.
May need immunosuppression in catastrophic flare ups.

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17
Q

What is systemic sclerosis?

A

Functional and structural abnormalities of small blood vessels, fibrosis of skin and internal organs with production of autoantibodies..

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18
Q

What are the features of limited cutaneous systemic sclerosis?

A 
Calcinosis
Reynaud's
Oesophageal and gut dysmotility
Sclerodactyly
Telangectasia

Risk of pulmonary hypertension.

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19
Q

What is the vascular involvement of systemic sclerosis?

A

Raynaud’s phenomenon
Ischaemic digital ulcers
Hypertensive crisis
Pulmonary arterial hypertension

20
Q

Which organs can fibrosis involve in systemic sclerosis?

A

Lungs
Heart
GI tract

21
Q

How is systemic sclerosis diagnosed?

A

Primarily clinical
Elevated ESR/CRP
May have raised ANA or RhF
Anti-centromere (skin involvement and better prognosis)
Anti-Scl70 (worse prognosis and lung fibrosis)

22
Q

What is the management of systemic sclerosis?

A 
No cure
Immunosuppressive regimes - cyclophosphamide/methotrexate
Control BP - ACE inhibitor or ARB
Sildenafil or calcium channel blocker
Low dose steroids
Monitor BP and renal function
Annual echo and spirometry
23
Q

What is the pathophysiology of Raynaud’s?

A

Vasospasm brought on by cold temperataures causes digits to change colour to white from lack of blood flow.
Affected areas subsequently turn blue due to de-oxygenation and/or red due to reperfusion.

24
Q

What percentage of the population is affected by Raynaud’s?

A

1-3%

25
Q

Is Raynaud’s more common in men or women?

A

Women

26
Q

What is secondary Raynauds?

A

Associated with underlying connective tissue disease.
Upregulation of inflammatory cytokines increased in secondary.
Diffuse scleroderma have increased adrenoreceptor activity.
May pre-date connective tissue disease.

27
Q

What are the investigations of Raynauds?

A 
If the patient has new onset Raynaud's, is >40 years old and/or has features from history or exam that suggest secondary causes:
ANA with titre and pattern
FBC
ESR
Serum creatinine
Urinalysis
28
Q

Which features that may be found in Raynaud’s are predictive for scleroderma?

A

Anti-centromere antibody

Dilated capillaries at the peri-ungal region.

29
Q

How is Raynaud’s treated?

A 
Aspirin
Statin
Calcium channel blockers
May need sildenafil is severe secondary
NSAIDs for analgesia
30
Q

What is Sjogren’s?

A

Chronic inflammatory and auto-immune disorder characterised by diminished lacrimal and salivary gland secretion (sicca complex) due to lymphocytic infiltration.

31
Q

How does sjogren’s present?

A

With fatigue, dry eyes and dry mouth.

32
Q

How is Sjogren’s diagnosed?

A

Positive ANA’s, Ro and anti-La auto-antibodies, decreased saliva and tear production, lymphocytic infiltration in labial salivary gland biopsy.

33
Q

What is the treatment for sjogren’s?

A

Mainly symptomatic, unless vasculitis/MSK involvement, in which case steroids and immunosuppression

34
Q

Which additional symptoms may be present in Sjogren’s?

A 
Dry skin
Dry nose
Dry throat
Dry vagina
Arthralgia
Myalgia
Peripheral neuropathies
Pulmonary, thyroid and renal disorders
Lymphoma
35
Q

What is the pathophysiology of polymyositis?

A

Major histocompatitibility complex-1 mediate cytotoxicity by CD8 T cells.
Immunological synapses form between CD8 T cells and MHC-1 expressed on muscle fibres - inflammation and necrosis.

36
Q

Which malignancies are associated with polymyositis?

A

Non-Hodgkin’s lymphoma
Lung cancer
Bladder cancer

37
Q

What is the biggest risk factor for dermatomyositis?

A

UV radiation intensity

38
Q

What is the pathophysiology of dermatomyositis?

A

Primary antigen target consists of components of the vascular endothelium of the larger endomysial blood vessels.
Lymphatic response results in capillary necrosis, microinfarction, inflammation and cell then spread into muscles.

39
Q

What are the characteristic skin lesions of dermatomyositis?

A

Heliotrope rash with eyelid oedema
Facial rash
Gottron’s papules (erythema of knuckles accompanied by a raised violaceous scaly eruption)
Erythematous rash over the knees, elbows, malleoli, and at the base of the neck and upper chest, forming a V sign.
Nail fold changes such a dilation of capillary loops of periungual area.

40
Q

Which cancers are associated with dermatomyositis?

A

Ovarian cancer
Pancreatic cancer
Non-Hodgkin’s lymphoma

41
Q

What are the clinical features of inflammatory polymyopathies?

A

Proximal symmetrical muscle weakness.
Increased difficulties in performing motor tasks predominantly requiring proximal muscles - e.g. getting up from a chair, climbing steps, combing hair.
Find motor tasks that depend on distal muscles affected - e.g. sewing, knitting, writing (late in the course of disease)

42
Q

What are the extramuscular symptoms of polymyopathies?

A 
Arthralgia
Dysphagia
Shortness of breath
Palpitatins
Syncope
Weight loss
Fatigue
Skin lesions
MI symptoms
Pain and muscle tenderness
43
Q

How is diagnosis of inflammatory polymyopathies confirmed?

A

Elevated serum muscle derived enzymes (CK)
25% have anti Jo antibodies
Typical EMG findings
Inflammation and necrosis on muscle biopsy.

44
Q

What are the investigations for polymyopathies?

A 
Creatinine kinase
Anti Jo antibodies
EMG
Muscle biopsy
Lactate dehydrogenase
Myoglobin
Aspartate aminotransferase
Alanine aminotransferase
45
Q

What is the management of inflammatory polymyopathies?

A

Corticosteroids - first line treatment. IV or oral depending on severity of presentation.
IV immunoglobulin, methotrexate, and azathioprine are used as second-line therapy or if life threatening attacks.
Third line therapies include cyclophosphamide, mycophenolate, ciclosporin and tacrolimus

Phase 4 (12 decks)

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