Rheumatology

Question 1

Churg-Strauss Syndrome (eosinophilic granulomatosis with polyangiitis) is associated with which antibodies?

Answer 1

MPO/P-ANCA

Question 2

How does Churg-Strauss tend to present?

Answer 2

Nasal congestion, adult-onset asthma, peripheral neuropathy, and glomerulonephritis.

Question 3

Which antibodies are associated with granulomatosis with polyangiitis (Wegner’s granulomatosis)?

Answer 3

cANCA

Question 4

How does granulomatosis with polyangiitis present?

Answer 4

URT involvement (recurrent sinusitis, epistaxis, saddle nose deformity), LRT involvement (cough, haemoptysis, pleuritis), pauci-immune glomerulonephritis (haematuria, proteinuria).
Often presents with a decline in renal function alongside recurrent epistaxis.

Question 5

How is granulomatosis with polyangiitis managed?

Answer 5

Steroids, biologics (rituximab)

Question 6

What is the HLA association for Bechet’s Disease?

Answer 6

HLA-B51

Question 7

What are the manifestations of Behcet’s disease?

Answer 7

Recurrent oral and genital ulcers, erythema nodosum, uveitis, acute pericarditis, acneiform lesions, superficial thrombophlebitis at venepuncture sites, arthritis, etc.

Question 8

How do you investigate for and manage Behcet’s disease?

Answer 8

Inx: Pathergy test (intradermal skin prick: development of papule or pustule is a +ve result as it represents exaggerated inflammatory response)
Mx: steroids around flares, may require systemic therapy long term (methotrexate, biologics)

Question 9

What is Polyarteritis nodosa?

Answer 9

Segmental transmural necrotising, small-medium muscular vessel vasculitis.
Systemic sx: skin (nodular and ulcerating lesions), heart, kidneys, GIT, and nervous system (mononeuritis multiplex).

Question 10

Which hepatitis is polyarteritis associated with?

Answer 10

Hep B

Question 11

Which vessels are NOT affected in polyarteritis nodosa?

Answer 11

Pulmonary vessels

Question 12

Who does polyarteritis nodosa primarily affect?

Answer 12

Men 40-60 y/o

Question 13

How is polyarteritis nodosa managed?

Answer 13

Mild: oral corticosteroids
Moderate: IV corticosteroids
Severe: Immunosuppressive therapy (e.g., cyclophosphamide)

Question 14

What are the manifestations of Giant Cell Arteritis?

Answer 14

Headache, unilateral vision loss (curtain closing), scalp tenderness, jaw claudication, etc.

Question 15

Patients with GCA are 17 times more likely to experience what aortic complication?

Answer 15

Aortic dissection.

Question 16

Which other rheumatological condition is associated with GCA?

Answer 16

PMR (about 50% GCA pts have a background of PMR)

Question 17

What type of optic neuropathy can develop in GCA? How does the optic disc look on examination?

Answer 17

Anterior ischaemic optic neuropathy: infarct of the short ciliary arteries which supply the head of the optic nerve.
Swollen, white, chalky optic disc on examination.

Question 18

What is an enteropathic arthropathy? What are the skin manifestations?

Answer 18

Joint involvement before, during, or after IBD diagnosis.
Associated with skin manifestations (pyoderma gangrenosum, erythema nodosum).

Question 19

What are the most common manifestations of psoriatic arthritis?

Answer 19

• Family/personal hx of psoriatic rash
• Occult rash (scalp, ears, umbilicus, natal cleft)
• Nail changes (pitting, oncolysis, ridges),
• Enthesopathy (Achilles tendonitis)
• Dactylitis
• Pencil-in-cup deformity on XR
• Asymmetrical oligoarthritis (large joint swelling)

Question 20

What makes psoriatic arthritis seronegative?

Answer 20

No associated autoantibodies

Question 21

What is the triad of reactive arthritis?

Answer 21

Conjunctivitis, urethritis, arthritis.

Question 22

What most commonly triggers reactive arthritis?

Answer 22

Genitourinary or gastrointestinal illness, most commonly chlamydia trachomatis.

Question 23

What is Caplan’s Syndrome?

Answer 23

Intrapulmonary nodules alongside rheumatoid arthritis.
‘Rheumatoid Pneumoconiosis’ associated with exposure to dust in industry (silica, asbestos, etc).

Question 24

What are the features of polymyalgia Rheumetica?

Answer 24

Shoulder hip and girdle pain/stiffness, often in the morning for <1 hour.
Systemic features: weight loss, loss of appetite, malaise, low grade fever.

Question 25

Who does PMR most commonly affect?

Answer 25

Women >50

Question 26

How can you investigate for PMR?

Answer 26

Generic bloods, tend to find that ESR is markedly raised >60.

Question 27

How is PMR managed?

Answer 27

Corticosteroids: sx relived within 72 hours.

Question 28

What markers can be measured when diagnosing rheumatoid arthritis?

Answer 28

Rheumatoid factor and anti-CCP

Question 29

What are the most commonly involved joints in rheumatoid arthritis?

Answer 29

PIPs and MCPs

Question 30

What are the signs of rheumatoid arthritis in the hands on XR?

Answer 30

- Periarticular osteopenia - Joint erosions - Periarticular soft tissue swelling - Joint space narrowing

Question 31

What score can be used to measure disease severity of rheumatoid arthritis? At what point might you start a DMARD?

Answer 31

DAS28, >5.0 = active disease - commence therapies.

Question 32

Below what DAS28 score indicates well controlled rheumatoid arthritis disease?

Answer 32

<3.2

Question 33

What medications are safer for patients with RA to be on if they wish to start family planning?

Answer 33

Sulfasalazine or hydroxychloroquine AND FOLIC ACID. NOT methotrexate!!

Question 34

What is Felty's syndrome?

Answer 34

Longstanding (tends to be erosive) RA, splenomegaly, neutropenia.

Question 35

What is meant by the term 'Seronegative Spondyloarthropathy'?

Answer 35

Axial and peripheral joint pain, enthesitis, tend to be HLA-B27 +ve but are -ve for rheumatoid factor.

Question 36

What signs do you see on XR with ankylosing spondylitis?

Answer 36

Lumbar spinal fusion (especially SI joints) know as bamboo spine, and syndesmophytes (bony outgrowths from spinal ligaments as they attach to adjacent vertebral bodies).

Question 37

What type of respiratory failure can occur in AS?

Answer 37

T2 RF due to poor mechanical ventilation = hypoxia and hypercapnia.

Question 38

Which HLA type is AS associated with?

Answer 38

HLA-B27

Question 39

What are some extra-articular features of AS?

Answer 39

Anterior uveitis, aortic insufficiency, apical pulmonary fibrosis

Question 40

How is AS managed?

Answer 40

NSAIDs, DMARDs, steroid injections, and if these are unsuccessful then try biologics (anti-TNF).

Question 41

What are the manifestations of inflammatory arthritis?

Answer 41

Morning stiffness, loss of function, fatigue, generally unwell with flu-like sx, and swelling, erythema, and warmth around joints.

Question 42

What is deposited in joints in pseudogout, how is this seen on microscopy?

Answer 42

Calcium pyrophosphate dihydrate crystals = positively birefringent rhomboids.

Question 43

What might you see on XR of pseudogout?

Answer 43

Calcification of articular cartilage known as chondrocalcinosis.

Question 44

True or False: Pts with hyperparathyroidism and haemochromatosis are more at risk of Pseudogout?

Answer 44

TRUE

Question 45

What type of crystal is deposited in gout? How do these appear on microscopy?

Answer 45

Monosodium urate monohydrate crystals: negatively birefringent needles.

Question 46

What is gout in the big toe known as?

Answer 46

Podagra

Question 47

What are the signs/sx of Gout?

Answer 47

Sudden, Intense pain, hot joint, limited range of movement due to pain.

Question 48

What are the RFs for gout?

Answer 48

- Male>55 - increased alcohol intake - high protein diet - episodes of dehydration

Question 49

What is the acute and long term management of gout?

Answer 49

Acute: colchicine, NSAIDs Long Term: allopurinol or febuxostat to reduce urate levels

Question 50

How does Adult Onset Still's Disease present?

Answer 50

Inflammatory disorder, presents with pyrexia, arthralgia, fine, non-purpuric salmon pink rash.

Question 51

What markers are most specific in SLE?

Answer 51

- Anti-dsDNA - Anti-smith

Question 52

What happens to C3 and 4 in SLE?

Answer 52

Low

Question 53

Which antibodies are found in drug-induced lupus? How does it present?

Answer 53

- Anti-histone abs. - Rash and increased sensitivity to sunlight

Question 54

What are the signs/sx of SLE?

Answer 54

- Butterfly malar rash - Joint pain (Jaccoud arthropathy, where deformities are reducible in extension) - Fatigues - Lupus nephritis (declining kidney function) - Cardiorespiratory issues

Question 55

How do you manage hypertension in SLE?

Answer 55

ACEIs or ARBs to reduce cardiovascular risk and help to decrease proteinuria.

Question 56

How do you manage the rash associated with Lupus if they have no other constitutional sx?

Answer 56

Hydroxychloroquine

Question 57

How do you manage the rash associated with Lupus alongside other constitutional sx?

Answer 57

Hydroxychloroquine + prednisolone

Question 58

How can we manage lupus nephritis?

Answer 58

Steroids and cytotoxic drugs such as cyclophosphamide.

Question 59

Which hypersensitivity reaction is Sjogren's? What is the pathophysiology?

Answer 59

Type IV - lymphocytic infiltration of glands causing decreased lacrimal and salivary secretion.

Question 60

What are the manifestations of Sjogren's?

Answer 60

- Dry eyes, mouth - Joint pain - Vaginal dryness = dyspareunia

Question 61

Which antibodies develop in Sjogren's?

Answer 61

Anti Ro and anti La

Question 62

How is Sjogren's managed?

Answer 62

Hypromellose: artificial tears, artificial saliva,

Question 63

Sjogren's increases the risk of which lymphoma? How does it present?

Answer 63

MALT low grade B-cell NHL: bilateral parotid swelling and cervical lymphadenopathy.

Question 64

Cutaneous Sclerosis (Scleroderma) can be split into which two types?

Answer 64

Limited: CREST syndrome (calcinosis, Raynaud's, oesophageal dysmotility, sclerodactyly, telangiectasia), anti-centromere antibodies Diffuse: anti-scl 70 antibodies, spreads further and involves mulitple organ systems

Question 65

What are the pulmonary complications of the two types of cutaneous sclerosis?

Answer 65

Limited: pulmonary HeTN Diffuse: pulmonary fibrosis

Question 66

What is Polymyositis? What antibodies are associated with it?

Answer 66

Inflammation and degeneration of skeletal muscle (inflammatory myopathy). Anti-Jo-1 antibodies.

Question 67

What is the main presentation of Polymyositis?

Answer 67

Bilaterally painless proximal muscle weakness due to chronic muscle inflammation. This causes problems such as standing up from sitting.

Question 68

Do you get muscle wasting in Polymyositis?

Answer 68

Much later on in the disease

Question 69

What type of pts most commonly present with Polymyositis?

Answer 69

40-60 y/o women.

Question 70

How can you investigate for Polymyositis?

Answer 70

- CK (will be elevated) - EMG (to confirm myopathic disorder) - Antibody testing

Question 71

What is Dermatomyositis?

Answer 71

Polymyositis with added manifestations on the skin e.g., heliotrope rash and shawl sign.

Question 72

Is Dermatomyositis associated with antibodies?

Answer 72

Yes, anti-Jo-1 antibodies like polymyositis BUT much less so than polymyositis. Anti-Mi2 antibodies are more specific.

Question 73

Which is more associated with malignancies of the lung: Polymyositis or Dermatomyositis?

Answer 73

Dermatomyositis

Question 74

What is complex regional pain syndrome?

Answer 74

Severe pain, swelling, and increased sensitivity to touch which tends to be triggered by a minor injury. May also cause skin colour changes.

Question 75

What is 1st line for medical management of Raynaud's?

Answer 75

Dihydropyridine CCB (nifedipine)

Question 76

What is Pes Planus?

Answer 76

Flat footed, congenital or acquired loss of longitudinal foot arch.

Question 77

What is acute Sarcoidosis called? How does it present? Mx?

Answer 77

Lofgren syndrome: fever, polyarthralgia, erythema nodosum, bilateral hilar lymphadenopathy. Mx: NSAIDs and rest.

Question 78

What are the systems affected my chronic sarcoidosis? Mx?

Answer 78

Pulmonary, neurological, ocular, cardiac, abdominal, and dermatological. Mx: steroids oral or IV.

Question 79

Which electrolyte abnormality is associated with Sarcoidosis?

Answer 79

Hypercalcaemia: granulomas contain macrophages which release excess 1,25-hydroxyvitamin D3 (active vitamin D aka calcitriol).

Question 80

Which serum markers can be raised in dermatomyositis and polymyositis?

Answer 80

They turn your muscles into CLAAA: - creatinine kinase - lactate dehydrogenase - aldolase - AST - ALT

Question 81

Which serotype is associated with both Rheumatoid arthritis and Felty's Syndrome?

Answer 81

HLA-DR4

Question 82

What WCC abnormality is associated with SLE?

Answer 82

Lymphopenia