Connective Tissue Disorders

Question 1

What are symptoms of Marfan’s?

Answer 1

Pectus excavatum
Aortic dilatation
High arched palate
Dislocated lens in eye

Question 2

Which HLA is associated with SLE?

Answer 2

DR2 and DR3

Question 3

What causes thrombosis in SLE?

Answer 3

Antiphospholipid ABs

Question 4

What dermatology is associated with SLE?

Answer 4

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

Question 5

What are neph effects of SLE?

Answer 5

Nephosis and nephritis
HTN
Proteinuria
Renal failure

Question 6

What cardiac and resp SLE features?

Answer 6

PE
Pleural effusions
Pericarditis/ myocarditis
MI

Question 7

What haematological SLE features?

Answer 7

Neutropaenia
Thrombocytopaenia
Lymphocytopaenia
Anaemia

Question 8

Psych and Neuro SLE features?

Answer 8

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

Question 9

What are feautres of antiphospholipid syndrome?

Answer 9

arterial and venous thrombosis
Recurrent foetal loss
Thrombocytopaenia

Question 10

What are antibodied present in antiphospholipid syndrome?

Answer 10

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

Question 11

What is livedo reticularis?

Answer 11

Purple lace like rash in SLE and autoimmune conditions

Question 12

What are the autoabs seen in SLE?

Answer 12

Anti DsDNA is specific (ANA to screen for it)

Question 13

What non medical management of SLE is needed?

Answer 13

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

Question 14

What drugs are used in SLE?

Answer 14

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

Question 15

What is used to treat Raynaud’s

Answer 15

Nifedipine
Sildenafil
Fluoxetine

Question 16

What does CREST stand for in SS?

Answer 16

Calcinosis
Raynaud's
Esophageal dysmotility
Schlerodactyly
Telangiecstasia

Question 17

What is schleroderma?

Answer 17

Hardened thick skin on fingers

Question 18

What is sclerodactyly?

Answer 18

Swollen fingers and above

Question 19

What autoab is present in difffuse SS?

Answer 19

anti scl70 and RNA Polymerase III

Question 20

Which autoab is present in limited cutaneous SS?

Answer 20

Anti centromere AB

Question 21

What is the presentation of limited cutaneous SS?

Answer 21

Long hx of Raynaud’s
Schlerodactyly
Pulmonary arterial hypertension

Question 22

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

Answer 22

Echo and pulmonary function tests

Question 23

What are other skin manifesations of CREST?

Answer 23

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

Question 24

What are features of diffuse SS?

Answer 24

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

Question 25

What is the medical management for SS?

Answer 25

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

Question 26

What autoabs are there is Sjogrens?

Answer 26

Anti Ro and anti La are diagnostic

Question 27

What causes Sjogren's?

Answer 27

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

Question 28

What are clinical features of Sjogren's?

Answer 28

``` Dry eyes Dry mouth Rash Vasculitis Renal tubule acidosis Interstitial lung disease Gluten sensitivity Lymphoma risk ```

Question 29

What would you US in sjogrens?

Answer 29

Salivery glands

Question 30

How do you manage sjogrens?

Answer 30

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

Question 31

What is polymyositis?

Answer 31

Chronic autoimmune muscle inflammation

Question 32

What is dermatomyositis?

Answer 32

Chronic autoimmune inflammation of skin and muscles

Question 33

What is diagnostic for polymyositis and dermato?

Answer 33

``` CK (Creatine Kinase) Other causes of raised CK: Rhabdomyositis AKI MI Statins Exercise ```

Question 34

Polymyositis and Dermato are paraneoplastic to which cancer?

Answer 34

Lung Breast Ovarian Gastric

Question 35

How does polymyositis present?

Answer 35

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

Question 36

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

Answer 36

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

Question 37

What autoabs are presetn in polymyositis?

Answer 37

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

Question 38

How is polymyositis diagnosed?

Answer 38

``` Clinlcally Elevated CK Autoabs EMG Muscle biopsy definitive ```

Question 39

How is polymyositis managed?

Answer 39

``` PET scan OT and physio Corticosteroids Immunosupression IVIg Biologicals -Infliximab ```

Question 40

What is SOAP BRAIN MD for SLE?

Answer 40

``` 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 ```