Connective Tissue Disorders Flashcards

1
Q

Give examples of connective tissue disorders?

A

Rheumatoid arthritis
Systemic/ discoid lupus erythematous
Systemic sclerosis
Sjogrens syndrome

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2
Q

What is rheuatmoid arthritis?

A

Auto-immune condition

Multi-system inflammatory disease of synovial and adjacent tissues

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3
Q

Aetiology of rheumatoid?

A

Females > males

Peak 35-50 years

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4
Q

What causes rheumatoid?

A

Auto-immune

Autoantibodies - rheumatoid factor

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5
Q

Issue with looking for rheumatoid factor?

A

Not very specific

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6
Q

What more accurate marker in rheumatoid?

A

Anti-CCP

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7
Q

Clinical features of rheumatoid?

A
Slow onset
Pain and stiffness of small joints
Fatigue
Anaemia
Weight loss
Muscle loss and wasting
Lung problems 
Lymphadenopathy
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8
Q

Most common joint affects in rheumatoid?

A

Finger joints

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9
Q

Is TMJ affected in rehumatoid?

A

in 30% have involvement

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10
Q

What hand signs might see in rheumatoid?

A

Ulnar deviation/ swan neck deformity

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11
Q

Extra-articular manifestation R.A?

A
Weight loss
Fatigue
Lymphadenopathy 
Rheumatoid nodules
Sjogrens
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12
Q

How diagnose rheumatoid?

A

Bloods - raised ESR/ CRP, anti-CCP positive, rheumatoid factor positive

Look radiographic changes

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13
Q

What guideline for management of rheumatoid?

A

NICE guidelines

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14
Q

What do NICE suggest management rheumatoid?

A
Education 
Exercise - maintain muscle
Physio
Surgery 
Dietary advice
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15
Q

When is surgery indicatied for RA?

A

If progressive deformity

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16
Q

What medications are used RA?

A

DMARDs
Corticosteroids
Biological agents
Symptomatic relief

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17
Q

What is DMARD and give examples?

A

Disease modifiying anti-rheumatic drug

e.g methotrexate/ hydroxychlorquine

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18
Q

Give example biological agent?

A

Anti-TNFa

e.g Infliximab

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19
Q

What medications are given for symptomatic relief of RA?

A

NSAIDs

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20
Q

What is the oro-facial impact of RA?

A

Access
Antalo-axial joint dislocation
Impaired manual dexterity - OH
Secondary Sjogrens

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21
Q

What may see is TMJ affected RA?

A

AOB

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22
Q

Felty’s syndrome can be seen with RA - what might see?

A

Increased risk oral infections

- oral ulceration/ angular chelitis

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23
Q

What are two forms of lupus erythematous?

A

Systemic and discoid

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24
Q

What is lupus erythematous?

A

Immunologically mediated condition

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25
Q

Where does discoid lupus affect?

A

Skin and oral mucosa

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26
Q

What condition does discoid lupus look similar to?

A

Lichen planus

27
Q

How does discoid lupus present on skin?

A

Scaly, erythaematous patch

Atrophic, hypo-pigmented area

28
Q

Issue w/ lesions of discoid lupus on skin?

A

Can be premalignant

29
Q

How is discoid lupus diagnosed?

A

Clinical appearance
Biopsy
Circulation auto-antibodies

30
Q

What auto-antibodies would be present in discoid lupus?

A

ANA

dsDNA

31
Q

Management of discoid LE?

A

Can’t cure
Improve symptoms - difflam/ topical steroids to reduce inflammation
Similar tx lichen planus

32
Q

What systemic features see in systemic LE?

A
Malar rash - cheeks/ bridge nose 
Polyarthirits
Photosensitivity
Oral lesion
Renal/ cardiac lesions
33
Q

How common is it to have oral lesions w/ systemic LE?

A

40%

34
Q

How do oral lesions present in systemic LE?

A

Uni/bilateral white patch w/ central area erythema/ ulceration

35
Q

Pharmacological management of systemic LE?

A

Hydroxychloroquine
Corticosteroids
Cytotoxic - azathioprine/ methoitrexate

36
Q

What drugs can cause lupus-like reactions?

A
Carbamazepine
Hydralazine
Penicillamine
Procanimadie 
Methyldopa
37
Q

What is systemic sclerosis?

A

Autoimmune disorder casuing dense collagen deposits in tissues of body

38
Q

What are types of SS?

A

Localised

Diffuse

39
Q

What is localised SS?

A

Limited skin face, hands and feet

40
Q

What is diffuse SS?

A

Extensive skin involvement w/ progression to visceral organs

41
Q

What has better prognosis diffuse or localised SS?

A

Localised –10-year survival = 75%

Diffuse – 10 year survival = 55%

42
Q

What syndrome is linked to localised SS?

A

CREST syndrome

43
Q

What See in CREST syndrome?

A
C - calcinosis (calcium deposits)
R - raynauds 
E - oEsophageal dysfunction 
S - sclerodactyly 
T - telangiectasia
44
Q

What are orofacial manifestations SS?

A
Facial skin rigidity 
Thinning lips
Loss facial wrinkets
Hypo-mobile tongue 
Dysphagia
Xerostomia 
PDL widening
Microstomia
45
Q

What are issues caused by microstomia in SS?

A

Poor access OH - increased caries/ perio

Issues: speech, eating

46
Q

How dx SS?

A

Difficult - clinical dx
Skin biopsy
PDL widening

47
Q

Management of SS?

A

Look improve circulation - nifedipine

Suppress immunity - corticosteroids/ DMARDs

48
Q

How tx microstomia in SS?

A

Exercise - stretching

Surgical approach - commissuotomy

49
Q

What is Sjogrens syndrome?

A

Autoimmune disease of exocrine glands (salivary/ lacrimal)

50
Q

Difference between primary and secondary sjogrens?

A

Primary - absence underlying connective tissue disorder

Secondary - presence of underlying connective tissue disorder (RA/ SLE/ SS)

51
Q

What see in sjogrens?

A

Focal lymphocytic infiltration of salivary and lacrimal glands

52
Q

Is it more common to see sjogrens in SLE or RA?

A

SLE
SLE 30% pt have secondary sjogrens
RA 15% have secondary sjogrens

53
Q

Causes of sjogrens?

A

Genetic predisposition

Viral agent - herpes virus/ retrovirus/ EBV

54
Q

Pathogenesis of sjogrens?

A
Lymphocytic infiltration of exocrine glands 
Hypertrophy of ductal epithelium 
Formation epimyoepithelial islands 
Acinar atrophy and fibrosis 
Hyperactivity of B-cells
55
Q

Symptoms of xerostomia related sjogrens?

A
Difficulty swallowing
Sensitivity spicy food
Altered taste - metalic
Burning mucosa
Lack taste
Salivary gland swelling
56
Q

Oral sign sjogrens?

A

Oral mucosa - dry/ atropy/ ulcerated
Tongue - dry/ red/ lobulated
Increased caries
Salivary gland - swelling

57
Q

What criteria is used for diagnosis of sjogrens?

A

European diagnostic criteria

58
Q

How diagnosis sjogrens?

A

Biopsy of salivary glands

59
Q

What are the 6 classifications of signs and symptoms in sjogrens?

A
I - ocular symptoms
II - oral symptoms  
III - ocular signs
IV - histopathology
V - salivary gland involvement 
VI - autoantibodies
60
Q

In regards to classification what makes it primary sjogrens?

A

Must have 4 out of 6

MUST have positive histopathology and autoantibodies

61
Q

In regards to classification what makes it secondary sjogrens?

A
Have underlying CT 
AND
presence of ocular OR oral symptoms 
AND
2 out of III, IV, V
62
Q

What are those w/ sjogrens at higher risk of?

A

Lymphoma - predominantly B cells

63
Q

Management of Sjogrens?

A

Palliative -

  • Increase lubtrication - salivary substitutes
  • Maintain oral/detanl health- OHE/ F- toothpaste
  • Review candida status

Therapeutic

  • Pilocarpine
  • Immunomodulating agents