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Rheumatology & Othopaedics > Connective Tissue Disorders > Flashcards

Connective Tissue Disorders Flashcards

1
Q

What are symptoms of Marfan’s?

A

Pectus excavatum
Aortic dilatation
High arched palate
Dislocated lens in eye

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2
Q

Which HLA is associated with SLE?

A

DR2 and DR3

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3
Q

What causes thrombosis in SLE?

A

Antiphospholipid ABs

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4
Q

What dermatology is associated with SLE?

A 
Photosensitive
Malar rash
Generalised erythema
Bullous LE
Chronic- Discoid rash, scarring alopecia, lupus profundus
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5
Q

What are neph effects of SLE?

A

Nephosis and nephritis
HTN
Proteinuria
Renal failure

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6
Q

What cardiac and resp SLE features?

A

PE
Pleural effusions
Pericarditis/ myocarditis
MI

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7
Q

What haematological SLE features?

A

Neutropaenia
Thrombocytopaenia
Lymphocytopaenia
Anaemia

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8
Q

Psych and Neuro SLE features?

A 
Aseptic meningitis
polyradiculopathy
autonomic disorder
CV disease
Headache
Neuropathy
Chorea
MG
CN issues
seizures
cognitive dysfunction
anxiety
psychosis
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9
Q

What are feautres of antiphospholipid syndrome?

A

arterial and venous thrombosis
Recurrent foetal loss
Thrombocytopaenia

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10
Q

What are antibodied present in antiphospholipid syndrome?

A

Cardiolipin antibodies IgG IgM
Antiphospholiipid AB
Must be positiive on 2 different occasions 12 weeks apart

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11
Q

What is livedo reticularis?

A

Purple lace like rash in SLE and autoimmune conditions

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12
Q

What are the autoabs seen in SLE?

A

Anti DsDNA is specific (ANA to screen for it)

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13
Q

What non medical management of SLE is needed?

A 
Education
UV protection
Manage in pregnancy
Assess for organ damage
Manage atheroschlerosis factors
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14
Q

What drugs are used in SLE?

A 
Topical steroid
NSAIDs
Antimalarials
Steroids
Cytotoxics- Azathioprine, methotrexate
Anticoagulants
Biologicals that target B cells: Rituximab or Belimumab
Stem cell transplant
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15
Q

What is used to treat Raynaud’s

A

Nifedipine
Sildenafil
Fluoxetine

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16
Q

What does CREST stand for in SS?

A 
Calcinosis
Raynaud's
Esophageal dysmotility
Schlerodactyly
Telangiecstasia
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17
Q

What is schleroderma?

A

Hardened thick skin on fingers

18
Q

What is sclerodactyly?

A

Swollen fingers and above

19
Q

What autoab is present in difffuse SS?

A

anti scl70 and RNA Polymerase III

20
Q

Which autoab is present in limited cutaneous SS?

A

Anti centromere AB

21
Q

What is the presentation of limited cutaneous SS?

A

Long hx of Raynaud’s
Schlerodactyly
Pulmonary arterial hypertension

22
Q

What needs to be tested annually in limited cut SS? (CREST)

A

Echo and pulmonary function tests

23
Q

What are other skin manifesations of CREST?

A

Digital ulcers, hair loss, pruritis, oedema, fingertip pitting, calcinosis, skin thickening, hyper or depigmentation, joint stiffness that progresses to contractures

24
Q

What are features of diffuse SS?

A

Fatigue, weight loss, athralgia and contractures, myocarditis, pericardial effusion, renal failure

25
Q

What is the medical management for SS?

A 
Raynauds treatment
PPI for GOR for life
ACEi to protect kidneys
Annual Echo for PAH
Skin oedema- cytotoxics or nothing
Pulmonary fibrosis- cyclophosphamide
26
Q

What autoabs are there is Sjogrens?

A

Anti Ro and anti La are diagnostic

27
Q

What causes Sjogren’s?

A

Primary

Or secondary to SLE, RA, SS, PBC or other autoimmune conditions

28
Q

What are clinical features of Sjogren’s?

A 
Dry eyes
Dry mouth
Rash
Vasculitis
Renal tubule acidosis
Interstitial lung disease
Gluten sensitivity
Lymphoma risk
29
Q

What would you US in sjogrens?

A

Salivery glands

30
Q

How do you manage sjogrens?

A

Tear and saliva replacement
Hydroxychloroquine for fatigue, myalgia and rashes
Corticosteroids or immunosupression for organ threatening symptoms

31
Q

What is polymyositis?

A

Chronic autoimmune muscle inflammation

32
Q

What is dermatomyositis?

A

Chronic autoimmune inflammation of skin and muscles

33
Q

What is diagnostic for polymyositis and dermato?

A 
CK (Creatine Kinase)
Other causes of raised CK: 
Rhabdomyositis
AKI
MI
Statins
Exercise
34
Q

Polymyositis and Dermato are paraneoplastic to which cancer?

A

Lung
Breast
Ovarian
Gastric

35
Q

How does polymyositis present?

A

Muscle pain and fatigue
Bilateral proximal muscles
Mostly shoulder and girdle pain developing over weeks

36
Q

What skin ailments present with dermatomyositis in addition to polymyositis symtoms?

A

Gottron lesios (scaly erythematous patches on knuckles, elbows and knees)
Photosensitive erythematous rash on back, shoulders and neck
Purple rash on face and eyelids
Preorbital oedema
Subcut calcinosis

37
Q

What autoabs are presetn in polymyositis?

A

Anti Jo-1 abs (polymyo)
Anti Mi-2 (dermato)
ANA in dermatomyositis

38
Q

How is polymyositis diagnosed?

A 
Clinlcally
Elevated CK
Autoabs
EMG
Muscle biopsy definitive
39
Q

How is polymyositis managed?

A 
PET scan
OT and physio
Corticosteroids
Immunosupression
IVIg
Biologicals -Infliximab
40
Q

What is SOAP BRAIN MD for SLE?

A 
Serositis (pleuritis, pericarditis)
Oral Ulcers
Arthritis
Photosensitivity
Blood low- thrombocytopaenia, anaemia
Renal protein (nephrotic syndrome)
ANA
Immunological (DsDNA etc)
Neurological (psych and neuro)
Malar Rash
Discoid Rash

Rheumatology & Othopaedics (7 decks)

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