224 RA

Question 0

What is the cause of RA?

Answer 0

Persistent synovitis leading to poly arthritis and systemic inflammation. Trigger antigen unknown

Question 1

What is rheumatoid factor?

Answer 1

The Fc portion of IgG which is recognised by antibodies as non self

Question 2

What is the prevalence of RA in the UK?

Answer 2

0.5-1%

Question 3

At what age is the typical age of onset of RA?

Answer 3

30 - 50 mostly in women

Question 4

Which cytokine is overexpressed in RA?

Answer 4

TNF-α

Question 5

What causes the overproduction and over expression of TNF-α in RA?

Answer 5

Interaction between macrophages and T and B lymphocytes

Question 6

What infiltrates into the synovial fluid in RA?

Answer 6

Inflammatory cells- incl polymorphs, lymphocytes and plasma cells

Question 7

What other pyrogenic cytokine is stimulated by TNF-α?

Answer 7

IL-6

Question 8

Which cells produce a large amount of adhesion molecule?

Answer 8

Synovial fibroblasts

Question 9

What happens to the surface layer of lining cells in the synovium in RA?

Answer 9

Becomes hyperplastic and thickened

Question 10

What is the cause of joint effusions in RA?

Answer 10

Vascular infiltration and increased permeability of vessels here

Question 11

What is the mechanism of cartilage damage in RA?

Answer 11

Directly by cytokines and chondrocytes

Indirectly via blockage of normal nutrients to cartilage due to hyperplastic lining of synovium.

Question 12

What is the mechanism of injury to the epiphyseal bone cavity in RA?

Answer 12

Fibroblasts grow along the vascular proliferation of synovium into the bone and cause damage

Question 13

Which class of Ig is most commonly detected in investigations for RA?

Answer 13

IgM

Question 14

What are ACPA’s in RA?

Answer 14

Anti-citrullinated peptide antibodies –> detected in Pts with RA

Question 15

Name 2 complications of RA

Answer 15

Septic arthritis

Amyloidosis

Question 16

Which bacteria is most common in septic arthritis?

Answer 16

Staph aureus

Question 17

What is amyloidosis?

Answer 17

Disorder of protein metabolism with extracellular deposition of pathological insoluble proteins in organs and tissues

Question 18

What is Boutonniere’s deformity?

Answer 18

Fixed Flexion of PIP joint

Question 19

What can swelling and subluxation of ulnar styloid in RA lead to?

Answer 19

Rupture of extensor tendons - causing sudden onset finger drop in ring and little finger

Question 20

What is the outcome of forward migration of the fibrofatty pad underneath the prox metacarpal joint in RA?

Answer 20

Ulcers forming under prox MT joint

Question 21

Which bacteria is most likely to cause septic arthritis?

Answer 21

Staph A

Question 22

Which 2 genes are thought to play a part in the genetic component of RA?

Answer 22

HLA-DR4

PTPN22

Question 23

What are the 2 major cell types in the pannus of a rheumatic joint?

Answer 23

T-lymphocytes and macrophages

Question 24

Name 2 complications of RA

Answer 24

Septic arthritis | Amyloidosis

Question 25

What is amyloidosis?

Answer 25

Disorder of protein metabolism where amyloid proteins get deposited in tissues - β-pleated sheets which are resistant to proteolysis

Question 26

What is Caplans syndrome? (occurs in RA rarely)

Answer 26

RA + pneumoconiosis

Question 27

What type of pneumonia can occur in an RA patient?

Answer 27

BOOP (bronchiolitis obliterans organising pneumonia)

Question 28

What are the S&S of rheumatoid vasculitis? | 5 listed

Answer 28

Nail fold infarcts Cutaneous vasculitis and necrosis of skin Mononeuritis multiplex Necrosing arteritis of mesenteric vessels Leg ulcers

Question 29

What are the possible neuro complications of RA? | 4 listed

Answer 29

Carpal tunnel Tarsal tunnel Peripheral sensory neuropathy Cord compression

Question 30

Name 3 ocular manifestations of RA

Answer 30

Sicca syndrome Scleritis/episcleritis Scleromalacia perforans

Question 31

What is Felty's syndrome? (In RA)

Answer 31

Splenomegaly and neutropenia (increased risk of infection here)

Question 32

What should be prescribed with MTX for RA?

Answer 32

Folic acid

Question 33

What is the drug therapy for new RA diagnosis?

Answer 33

Analgesia NSAID's Corticosteroids 1 DMARD

Question 34

Name 2 DMARDs | 4 listed

Answer 34

MTX Sulphasalazine Leflunomide Hydroxychloroquine

Question 35

Name 2 antiTNF drugs for RA

Answer 35

Infliximab Adalimumab Certolimab Rituximab

Question 36

Which cytokines does TNF-α activate in RA?

Answer 36

IL-1, IL-6, IL-8

Question 37

What do the autoantibodies target in SLE?

Answer 37

Nuclear antigens

Question 38

Which virus is associate with SLE?

Answer 38

Epstein Barr

Question 39

What causes the rashes in SLE?

Answer 39

Immune complexes causing microvascular occlusions

Question 40

What characteristic rashes are seen in SLE?

Answer 40

Malar rash (butterfly which spares nasal creases) Discoid rash Photosensitivity rash

Question 41

Which ABs are commonly found in serology of SLE? | 4 listed

Answer 41

ANA (100%) dsDNA (50%) antiSM (15%) anti-Ro/anti-La (20%)

Question 42

What is the treatment for mild SLE?

Answer 42

NSAIDs Hydroxychloroquine Steroids

Question 43

What DMARD is used to treat severe SLE?

Answer 43

Cyclophosphamide (alkylating agent which adds alkyl to guanine base of DNA - used as chemo)

Question 44

Which cells are involved with destruction of muscle fibres in polymyositis?

Answer 44

Cytotoxic T cells

Question 45

What's the pathophysiology of dermatomyositis?

Answer 45

Auto antibodies causing activation of the complement system - damages muscle capillaries

Question 46

What are Gottron's papules a sign of?

Answer 46

Dermatomyositis

Question 47

Where is the macular erythematous rash most commonly found on a pt with dermatomyositis?

Answer 47

Face and chest

Question 48

What investigation is performed to confirm a diagnosis of dermatomyositis?

Answer 48

Muscle Bx

Question 49

What are the possible serious complications which can arise in a pt with dermatomyositis? 2 listed

Answer 49

Aspiration due to swallowing difficulty | Lung fibrosis due to chest wall weakness

Question 50

Which AB is involved in limited systemic sclerosis?

Answer 50

Anti-centromere AB

Question 51

Which antibody is seen in diffuse systemic sclerosis?

Answer 51

Scl-l70 AB

Question 52

What are the clinical features of systemic sclerosis? | CREST

Answer 52

``` Calcinosis Raynauds Esophageal and GIT dysmotility Sclerodactyly Telangectasia ```

Question 53

What are the possible complications of diffuse systemic sclerosis? (2 listed)

Answer 53

Pulmonary HTN leading to L sided HF | Renal crisis leading to accelerated malignant HTN

Question 54

Name 2 drugs which can be used to treat systemic sclerosis and scleroderma

Answer 54

Mycophenolate mofetil | Cyclophosphamide

Question 55

What is the pathophysiology of Sjögren syndrome?

Answer 55

Lymphocytic infiltration to exocrine organs

Question 56

Which antibodies are present in Sjögren syndrome?

Answer 56

Anti-Ro and anti-La | Also RF and ANA

Question 57

What are the S&S of Sjögren syndrome? | 3 listed

Answer 57

Dry eyes and mouth Salivary gland enlargement Purpura

Question 58

Which condition is linked to giant cell arteritis?

Answer 58

Polymyalgia rheumatica

Question 59

Name one complication of GCA

Answer 59

Blindness due to arteritis of ophthalmic artery

Question 60

Name 2 S&S of GCA | 4 listed

Answer 60

Jaw claudication Localised HA (scalp tenderness) Temporal artery enlargement and tenderness + constitutional symptoms

Question 61

What is vasculitis?

Answer 61

Inflammation within the walls of vessels causing impairment to blood flow and damage to vessel integrity - leads to haemorrhage and infarction of organs

Question 62

What investigation is required in GCA?

Answer 62

Temporal artery biopsy

Question 63

Is GCA more common in men or women?

Answer 63

Women

Question 64

What is the immediate treatment of GCA?

Answer 64

High dose steroids within 24 hours

Question 65

What is Takayasu's arteritis?

Answer 65

Granulomatous inflammation of aortic arch and branches

Question 66

Name 2 S&S of Takayasu's arteritis | 4 listed

Answer 66

Constitutional Claudication/pulslessness of upper limbs (+/- lower limbs) HTN due to renal artery stenosis Neuro symptoms

Question 67

Name 2 investigations to diagnose Takayasu arteritis

Answer 67

Arteriography | Echo

Question 68

What is the treatment of Takayasu's arteritis

Answer 68

High dose steroids

Question 69

What is the pathophysiology of granulomatous polyangiitis?

Answer 69

Ig's against ANCAs, especially PR3 which causes increased neutrophil of activation and granuloma formation

Question 70

Which structures does granulomatous polyangiitis normally affect?

Answer 70

Midline - eyes, nose, trachea, lungs | + kidneys

Question 71

Name 2 ENT S&S of granulomatous polyangiitis | 3 listed

Answer 71

Saddle nose Epistaxis Sinusitis

Question 72

Name 2 pulmonary S&S of granulomatous polyangiitis | 3 listed

Answer 72

Pulmonary infiltrates Cough Haemoptysis

Question 73

Name 2 renal S&S of granulomatous polyangiitis | 3 listed

Answer 73

Microscopic haematuria Casts Renal failure

Question 74

What is the treatment of mild/moderate granulomatous polyangiitis?

Answer 74

MTX/azothioprine + steroids

Question 75

Which drug is used for the treatment of severe granulomatous polyangiitis?

Answer 75

Cyclophosphamide

Question 76

What is polyarteritis nodosa?

Answer 76

Necrotising inflammationof small/medium sized arteries causing lesions in bifurcation which can lead to micro aneurysms, haemorrhage and thrombosis

Question 77

Which sign is diagnostic of polyarteritis nodosa?

Answer 77

Rosary sign in arteries

Question 78

Which investigations are useful to diagnose polyarteritis nodosa?

Answer 78

Inflammatory markers in blood | Artery biopsy

Question 79

What is the treatment of polyarteritis nodosa?

Answer 79

Steroids and Immunosuppression

Question 80

Which virus is a trigger in 30% of pts with polyarteritis nodosa?

Answer 80

HepB

Question 81

What is Churg-Strauss syndrome?

Answer 81

Rare complication of asthma (moderate yo severe) leading to vascular necrosis which starts in the lungs

Question 82

How does Churg-Strauss syndrome typically present?

Answer 82

Hypergammaglobinaemia

Question 83

Which markers are present in serology in Churg-Strauss syndrome?

Answer 83

IgE RF ANCA

Question 84

What are the 3 stages of Churg-Strauss syndrome?

Answer 84

1. Allergic rhinitis and asthma 2. Eosinic infiltration - cough, pneumonia 3. Widespread i.e. Peripheral neuropathy, renal, arthralgia, purpurin, diarrhoea, GIT bleeding, myocarditis/pericarditis

Question 85

What can be seen on CXR in a person with Churg-Strauss syndrome?

Answer 85

Bilateral, peripheral patchy parenchymal opacities