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connective tissue diseases Flashcards

1
Q

who is affected by joint hypermobility, when does it present

A

usually women

presents in childhood or in 3rd decade

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2
Q

what are the causes of joint hypermobility

A

idiopathic
marfan’s
Ehlers danlos syndrome

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3
Q

what are the symptoms of joint hypermobility

A

arthralgia

premature OA

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4
Q

what are the investigations of joint hypermobility

A

modified beightons score = 4 or more

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5
Q

what are the questions in the modified beightons score

A

> 10º hyperextension of the elbows
Passively touch the forearm with the thumb, while flexing the wrist.
Passive extension of the fingers or a 90º or more extension of the fifth finger
Knees hyperextension ≥ 10º)
Touching the floor with the palms of the hands when reaching down without bending the knees.

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6
Q

what is the treatment of joint hypermobility

A

physio

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7
Q

what is soft tissue rheumatism

A

general term to describe pain that is caused by inflammation/damage to ligaments, tendons, ,muscles or nerves located near a joint

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8
Q

what is the most common site of soft tissue rheumatism

A

shoulder

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9
Q

what is the treatment of soft tissue rheumatism

A 
pain control
rest
physio
steroid injections 
surgery
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10
Q

what is sjogren’s syndrome

A

long-term autoimmune disease that is characterised by lymphocytic infiltration of the exocrine glands

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11
Q

what are the symptoms of sjogren’s syndrome

A 
gritty eyes
blepharitis = red/inflamed eyes
vaginal dryness
thirst
dry mouth
salivary gland inflammation
tooth decay
dry cough
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12
Q

what are the investigations of sjogren’s syndrome

A 
schrimer's test = ocular dryness
antibodies
salivary gland ultrasound + biopsy  = if antibodies are negative
raised IgG 
high ESR & PV
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13
Q

what auto-antibodies are associated with sjogren’s syndrome

A

ANA
Anti-Ro
Anti-la

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14
Q

what is the treatment for sjogren’s syndrome

A

artificial tears and salivary supplements
vaginal lubricants
good dental hygiene + strong fluoride toothpaste
hydroxychloroquine = fatigue and arthralgia
immunosuppression = if a major organ is involved

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15
Q

what is mixed connective tissue disease

A

symptoms of SLE, RA, scleroderma and polymyalgia

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16
Q

what antibody is associated with mixed connective tissue disease

A

Anti-RMP

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17
Q

what is the treatment for mixed connective tissue disease

A

annual ECHO’S and pulmonary function test = check for pulmonary hypertension and IDL

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18
Q

what condition is associated with scleroderma

A

raynauds

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19
Q

what is scleroderma also know as

A

systemic sclerosis

SSc

20
Q

what are the two types of scleroderma

A

diffuse cutaneous systemic sclerosis

limited cutaneous systemic sclerosis

21
Q

describe the symptoms of diffuse cutaneous systemic sclerosis

A

skin involvement on extremities above and below elbows and knees, face, and trunk
early significant organ involvement

22
Q

what antibody is associated with diffuse cutaneous systemic sclerosis

A

anti-scl-70

23
Q

describe the symptoms of limited cutaneous systemic sclerosis

A

skin involvement on extremities only below elbows and knees + face

24
Q

what antibody is limited cutaneous systemic sclerosis associated with

A

anti-centromere

25
what are the symptoms of scleroderma
CREST: calcinosis = Ca2+ deposits in skin Raynauds Oesophageal dysfunction = acid reflux + decrease in motility sclerodactyly = thickening and tightening of skin on fingers + hands telangiectasia pulmonary fibrosis and hypertension scleroderma renal crisis
26
what is the treatment of scleroderma
yearly ECHO and pulmonary function testing immunosuppression GI involvement = PPI renal involvement = BP control (ACEI)
27
what is systemic lupus erythematous (SLE)
chronic, systemic autoimmune disease that can affect any part of the body
28
who is affect by SLE
women | non-african blacks
29
what is the cause of SLE
autoantibodies directed at structural parts of DNA
30
who is at higher risk of SLE, give examples
higher oestrogen exposure: early menstruation oestrogen containing contraceptive HRT
31
what are the symptoms of SLE
``` Systemic (fever, weight loss etc) mucocutaneous muscle weakness and pain renal = lupus nephritis haematological ```
32
what are the mucocutaneous symptoms of SLE
``` o Photosensitivity o Malar rash – “butterfly rash”, doesn’t affect eyelids o Discoid lupus erythematosus o Subacute cutaneous lupus o Mouth ulcers o Alopecia ```
33
what are the haematological symptoms of SLE
``` o Lymphadenopathy o Leucopenia o Lymphopenia o Haemolytic anaemia o Thrombocytopenia ```
34
what are the investigations of SLE
``` urinalysis = identify glomerulonephritis antibodies normal CRP biopsy screen for organ involvement ```
35
what antibodies are associated with SLE
ANA anti-double stranded antibody (anti-sDNA)= very specific Anti-ENA = Anti-Ro, Anti-sm, Anti-RNP anti-phospholipid antibodies = anti-cardiolipid, lupus anticoagulant, anti-beta 2 glycoprotein
36
how is SLE monitored
``` clinical assessment anti-dsDNA = correlates with activity C3/C4 levels = correlates with activity urinalysis = glomerulonephritis monitor BP and cholesterol ```
37
what is the treatment of SLE
regular monitoring avoid excessive sun exposure drugs
38
what drugs are used to treat mild SLE
hydroxychloroquine topical steroids NSAIDs
39
what drugs are used to treat moderate SLE
oral steroids azathroprine methotrexate
40
what drugs are used to treat severe SLE
IV steriods cyclophosphamide rituximab belimumab
41
what is anti-phospholipid syndrome
a disorder which manifests clinically as recurrent venous or arterial thrombosis and/or foetal loss. HIGHER RATE OF CLOTS
42
what are the symptoms of anti-phospholipid syndrome
increased frequency of stroke or MI recurrent PE or thrombosis = can lead to pulmonary hypertension catastrophic ASP Miscarriage = usually late but can occur at any time migraine livedo reticularis
43
what are the investigations of anti-phospholipid syndrome
thrombocytopenia prolonged APTT antibodies
44
what antibodies are associated with anti-phospholipid syndrome, when must they be present to indicate diagnosis
lupus anticoagulant anti-cardiolipin anti-beta 2 glycoprotein must be present on 2 occasions at least 12 weeks apart
45
what is the treatment of anti-phospholipid syndrome
episode of thrombosis = anti-coagulantion (warfarin) no episode of thrombosis = no treatment

MSK (mine) (17 decks)

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