Rheumatology Flashcards

1
Q

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

A

MPO/P-ANCA

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2
Q

How does Churg-Strauss tend to present?

A

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

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3
Q

Which antibodies are associated with granulomatosis with polyangitis?

A

cANCA

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4
Q

How does granulomatosis with polyangiitis present?

A

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.

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5
Q

How is granulomatosis with polyangiitis managed?

A

Steroids, biologics (rituximab)

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6
Q

What is the H:A association for Bechet’s Disease?

A

HLA-B51

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7
Q

What are the manifestations of Behcet’s disease?

A

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

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8
Q

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

A

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)

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9
Q

What is Polyarteritis nodosa?

A

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

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10
Q

Which hepatitis is polyarteritis associated with?

A

Hep B

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11
Q

Which vessels are NOT affected in polyarteritis nodosa?

A

Pulmonary vessels

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12
Q

How does polyarteritis nodosa primarily affect?

A

Men 40-60 y/o

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13
Q

How is polyarteritis nodosa managed?

A

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

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14
Q

What are the manifestations of Giant Cell Arteritis?

A

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

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15
Q

Which other rheumatological condition is associated with GCA?

A

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

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16
Q

What type of optic neuropathy can develop in GCA?

A

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

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17
Q

Which patients get an enteropathy arthropathy?

A

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

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18
Q

What are the most common manifestations of psoriatic arthritis?

A
  • 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)
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19
Q

What makes psoriatic arthritis seronegative?

A

No associated autoantibodies

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20
Q

What is the triad of reactive arthritis?

A

Conjunctivitis, urethritis, arthritis.

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21
Q

What most commonly triggers reactive arthritis?

A

Genitourinary or gastrointestinal illness, most commonly chlamydia trachomatis.

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22
Q

What is Caplan’s Syndrome?

A

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

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23
Q

What are the features of polymyalgia Rheumetica?

A

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

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24
Q

Who does PMR most commonly affect?

A

Women >50

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25
Q

How can you investigate for PMR?

A

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

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26
Q

How is PMR managed?

A

Corticosteroids: sx relived within 72 hours.

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27
Q

What markers can be measured when diagnosing rheumatoid arthritis?

A

Rheumatoid factor and anti-CCP

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28
Q

What are the most commonly involved joints in rheumatoid arthritis?

A

PIPs and MCPs

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29
Q

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

A
  • Periarticular osteopenia
  • Joint erosions
  • Periarticular soft tissue swelling
  • Joint space narrowing
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30
Q

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

A

DAS28,
>5.0 = active disease - commence therapies.

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31
Q

Below what DAS28 score indicates well controlled rheumatoid arthritis disease?

A

<3.2

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32
Q

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

A

Sulfasalazine or hydroxychloroquine AND FOLIC ACID.
NOT methotrexate!!

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33
Q

What is Felty’s syndrome?

A

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

34
Q

What is meant by the term ‘Seronegative Spondyloarthropathy’?

A

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

35
Q

What signs do you see on XR with ankylosing spondylitis?

A

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

36
Q

What type of respiratory failure can occur in AS?

A

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

37
Q

Which HLA type is AS associated with?

A

HLA-B27

38
Q

What are some extra-articular features of AS?

A

Anterior uveitis, aortic insufficiency, apical pulmonary fibrosis

39
Q

How is AS managed?

A

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

40
Q

What are the manifestations of inflammatory arthritis?

A

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

41
Q

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

A

Calcium pyrophosphate dihydrate crystals = positively birefringent rhomboids.

42
Q

What might you see on XR of pseudogout?

A

Calcification of articular cartilage known as chondrocalcinosis.

43
Q

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

A

TRUE

44
Q

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

A

Monosodium urate monohydrate crystals: negatively birefringent needles.

45
Q

What is gout in the big toe known as?

A

Podagra

46
Q

What are the signs/sx of Gout?

A

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

47
Q

What are the RFs for gout?

A
  • Male>55
  • increased alcohol intake
  • high protein diet
  • episodes of dehydration
48
Q

What is the acute and long term management of gout?

A

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

49
Q

How does Adult Onset Still’s Disease present?

A

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

50
Q

What markers may test +ve in SLE?

A
  • Anti-dsDNA
  • Anti-smooth muscle
  • ANA
51
Q

What happens to C3 and 4 in SLE?

A

Low

52
Q

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

A
  • Anti-histone abs.
  • Rash and increased sensitivity to sunlight
53
Q

What are the signs/sx of SLE?

A
  • Butterfly malar rash
  • Joint pain (Jaccoud arthropathy, where deformities are reducible in extension)
  • Fatigues
  • Lupus nephritis (declining kidney function)
  • Cardiorespiratory issues
54
Q

How do you manage hypertension in SLE?

A

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

55
Q

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

A

Hydroxychloroquine

56
Q

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

A

Hydroxychloroquine + prednisolone

57
Q

How can we manage lupus nephritis?

A

Steroids and cytotoxic drugs such as cyclophosphamide.

58
Q

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

A

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

59
Q

What are the manifestations of Sjogren’s?

A
  • Dry eyes, mouth
  • Joint pain
  • Vaginal dryness = dyspareunia
60
Q

Which antibodies develop in Sjogren’s?

A

Anti Ro and anti La

61
Q

How is Sjogren’s managed?

A

Hypromellose: artificial tears, artificial saliva,

62
Q

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

A

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

63
Q

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

A

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

64
Q

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

A

Limited: pulmonary HeTN
Diffuse: pulmonary fibrosis

65
Q

What is Polymyositis? What antibodies are associated with it?

A

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

66
Q

What is the main presentation of Polymyositis?

A

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

67
Q

Do you get muscle wasting in Polymyositis?

A

Much later on in the disease

68
Q

What type of pts most commonly present with Polymyositis?

A

40-60 y/o women.

69
Q

How can you investigate for Polymyositis?

A
  • CK (will be elevated)
  • EMG (to confirm myopathic disorder)
  • Antibody testing
70
Q

What is Dermatomyositis?

A

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

71
Q

Is Dermatomyositis associated with antibodies?

A

Yes, anti-Jo-1 antibodies like polymyositis BUT much less so than polymyositis.

72
Q

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

A

Dermatomyositis

73
Q

What is complex regional pain syndrome?

A

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

74
Q

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

A

Dihydropyridine CCB (nifedipine)

75
Q

What is Pes Planus?

A

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

76
Q

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

A

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

77
Q

What are the systems affected my chronic sarcoidosis? Mx?

A

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

78
Q

Which electrolyte abnormality is associated with Sarcoidosis?

A

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

79
Q

Which serum markers can be raised in dermatomyositis and polymyositis?

A

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

80
Q

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

A

HLA-DR4

81
Q

What WCC abnormality is associated with SLE?

A

Lymphopenia

82
Q
A