Rheumatology Flashcards

1
Q

X-ray findings of rheumatoid arthritis

A

Loss of joint space, periarticular osteoporosis, swelling

Late: periarticular erosions, subluxation

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2
Q

Associations with myotonic dystrophy

A

Distal weakness initially
Diabetes
auto Dominant
Dyarthria

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3
Q

Features of myotonic dystrophy

A
20-30yrs... Myotonic facies "haggard"
Distal weakness
Frontal balding
Bilateral ptosis
Dysarthria
Mild mental impairment
Testicular atrophy
Heart block, cardiomyopathy
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4
Q

Diagnosis of vitamin d resistant rickets

A

Increased urine phosphate, in context of normal calcium and cupped metaphyses on XR

X linked dominant

Replace vit d

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5
Q

Symmetrical proximal muscle weakness, Reynauds, no rash diagnosis

A

Polymyositis
(Anti Jo)

Rash indicated dermatomyositis

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6
Q

Investigations for proximal symmetrical muscle weakness with Reynauds and no rash

A

Polymyositis

Elevated CK, and other elevated muscle enzymes (LD, AST, ALT)
Anti Jo1 (seen in pattern of disease with lung involvement)
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7
Q

What does CREST stand for

A

Calcinosis
Reynauds
Eosophageal dysmotility and malabsorption
Sclerodactyly
Telangiectasia (small dilated blood vessels

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8
Q

Features of myasthenia gravis

A

Diplopia
Muscle fatigue
Ptosis
Dysphagia

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9
Q

Management of myasthenia gravis

A

Long acting anticholesterase inhibitor, pyridostigmine
Immunosuppression with steroids
Thymectomy

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10
Q

Lambert Eaton syndrome management

A

Treat underlying cancer!

Immunosuppression with pred, aza

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11
Q

Genetic link with multiple sclerosis

A

HLA DR2…
Monozygotic twin concordance of 30%
Dizygotic 2%

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12
Q

Environmental factors of multiple sclerosis

A

Higher latitudes

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13
Q

Neurological triad of signs for MS

A

Charcot’s triad - Dysarthria, nystagmus, intention tremor

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14
Q

Two specific signs for MS

A

L’hermittes sign -electric shock down spine when head forwards
Urthoffs phenomenon - worse vision when body temp increases

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15
Q

Associations with pANCA and cANCA

A

pANCA granulomatosis with polyangitis (wegeners)
cANCA churg Strauss syndrome

pANCA also associated with IBS (UC>C), Connections tissue disease and autoimmune hepatitis

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16
Q

Which immunodeficiency predisposes to neisseria meningitidis

A

C5-9 deficiency

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17
Q

What antibody is suggestive of RA

A

Anti cyclic citrullinated peptide antibody can be detectable 10 years before! Sensitivity of 70 (similar to RF) but specificity of 90-95%

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18
Q

Investigations for proximal symmetrical muscle weakness with vesicular rash on back and shoulders and heliotrope rash around periorbital region

A

Dermatomyositis

ANA positive, and 25% anti Mi-2 positive

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19
Q

Immunoglobulins reaction of Rheumatoid Factor

A

Rheumatoid factor is an IgM antibody against IgG

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20
Q

Autoantibody associated with dermatomyositis

A

ANA

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21
Q

Autoantibody associated with rheumatoid arthritis

A

Anti CCP (cyclic citrullinated peptide) antibody is most specific (90%)
also a poor prognostic factor
may be detectable up to 10 years before the development

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22
Q

Autoantibody associated with SLE

A

Anti dsDNA antibodies…non-specific symptoms such as, fatigue, fever, oral ulcers, joint pain and a rash

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23
Q

Autoantibody associated with diffuse systemic sclerosis

A

Anti scl 70

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24
Q

Autoantibody associated with primary biliary cirrhosis

A

AMA…
Often asymp but fatigue, pruritus and jaundice

IgM

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25
Q

Features of adult Stills disease

A
Bimodal presentation 15-25 35-45
Arthralgia 
elevated ferritin
Salmon pink maculopapular rash
LN
fever
RF and ANA neg

Treat with methotrexate then Anakinra (competitively inhibits the action of IL-1)

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26
Q

Management of myasthenic crisis

A

Plasmapheresis or IV immunoglobulin

Plasma is quicker but more expensive equipment

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27
Q

Most common target of pANCA

A

MPO

Myeloperoxidase

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28
Q

Most common target of pANCA

A

MPO

Myeloperoxidase

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29
Q

Most common target of cANCA

A

PR3

Proteinase 3

30
Q

What antibody is usually found in Myasthenia gravis

A

(nicotinic) Acetylcholinesterase receptor antibodies in 90%

31
Q

Patient with MS, on Natalizumab, acutely confused, with hemiparesis

A

That drug can reactivate JC virus causing progressive multifocal leukoencephalopathy PML

32
Q

Compliment deficiency that predisposes to SLE

A

Deficiency in C1q, C1rs, C2 and C4 predisposes to immune complex disease such as SLE

33
Q

Patient with dermatomyositis… What is most important first investigation?

A

Exclude malignancy.
~25% diagnosed with dermatomyositis have an underlying malignancy (typically ovarian, breast and lung cancer), this prevalence increases in the elderly.

34
Q

Poor prognostic factors related to RA

A

Rh F positive
Anti CCP autoantibodies
HLA DR4
Early erosions and extra articular features like nodules

35
Q

How does Rheumatoid factor correspond with severity / progression in RA

A

High levels of Rh F are assoicated with severe progressive disease, but are not a marker of disease progression

36
Q

What type of HLA allele is most associated with Rheumatoid arthritis?

A

HLA DR4

RA - R4

37
Q

Myalgia and fatigue, mononeuritis, glomerulonephritis… Diagnosis and antibody

A

Microscopic polyangiitis

pANCA

38
Q

Most common complications of diffuse cutaneous systemic sclerosis

A

Most common cause of death is resp (80%)

ILD and pulmonary arterial hypertension

39
Q

HLA memory aid

A
SjogR3n's DR3
RA DR4 (also T1DM)
hA3mochomatosis A3
B5hcet's disease B51
CeiliaQ DQ2/DQ8
naRcolepsy / goodpastuRes DR2
Reiters ... 27yr sleeping around b27

B27 also ank spond (presents agreed 20-30)
And acute anterior uveitis because that’s also assx

40
Q

Features of Adult onset Stills disease

A

Arthralgia, fever, salmon Mac pap rash, lymphadenopathy

RF and ANA negative

41
Q

Management of Reynaud’s….

A

Secondary care
First line CCB like nifedipine
Second line IV prostacycline like epoprostenol

42
Q

What type of collagen is primarily affected by Ehlers Danlos Syndrome?

A

Collagen type 3

3hlers-danlos

43
Q

SLE - antibodies associated with congenital heart block

A

Anti Ro

Road…block

44
Q

Poor prognostic features of RA

A
RF +ve
Anti CCP antibodies
Slow onset
HLA DR4
XR early erosions
Extra articular features like nodules

Possibly female

45
Q

Anti-GQ1b antibodies

A

Present in 90% of Miller Fischer syndrome

46
Q

Drugs that exacerbate MG

A

Beta blockers
Gentamicin
Lithium
Phenytoin

47
Q

SLE - antibodies associated with congenital heart block

A

Anti Ro

Ro ro ro your boat

48
Q

Bony lump at DIP

A

Heberden’s OA

49
Q

Bony lumps at PIP

A

bouchards RA

50
Q

wegners / granulomatosis with polyangiitis features

A

Ent features like sinusitis

51
Q

Goodpastures features

A

Pulmonary haemorrhage

52
Q

anti-smooth muscle antibodies (SMA)

A

Type 1 autoimmune hepatitis

Treat with steroids, / immunosup e.g. azathioprine
liver transplantation

CAN get smooth muscle ab in primary biliary cholangitis but more assx with AMA and IgM

53
Q

RA scleritis Vs episcleritis

A

Scleritis is Sore

ePiscLEritis is PainLess

54
Q

Which immunoglobulin are most common?

A

GAMDE

55
Q

Management of SLE

A

Hydroxychloroquine

56
Q

Management of MG

A

pyridostigmine

57
Q

Diagnosis of ankylosing spondylitis

A

x-ray of the sacroiliac joints - look for sclerosis,or subchondral cysts
Or MRI if still unclear
Looking for inflam of sacroiliac joints (bone marrow oedema)

58
Q

Which antibiotics should you avoid when on methotrexate

A

Trimethoprim and co-trimoxazole

They all work by inhibiting the enzyme dihydrofolate reductase, so can cause myelosuppression

59
Q

Which cytokine is most important in pathophys of RA

A

Tumour necrosis factor

60
Q

Related cramps and myoglobinuria after exercise

A

McArdle’s disease

Auto R type V glycogen storage disease

61
Q

Etanercept TNF-alpha adverse effect

A

Reactivation of TB

62
Q

Symps and antibodies of mixed connective tissue disease

A

Anti RNP
Reynaud’s
N - no synovitis but swollen hands
Pain in muscles and joints

63
Q

mixed connective tissue disease prognosis

A

1/3 long-term remission
Chronic symps
Develop severe systemic involvement and premature death

64
Q

What feature is isn’t commonly seen in drug induced lupus

A

Glomerulonephritis

65
Q

Medium vessel vasculitis assx w hep B

A

Polyarteritis nodosa

No Autoantibodies likely to be positive

66
Q

Fever malaise foot drop, livedo reticularis , background of hep b

A

Polyarteritis nodosa

No antibody likely positive, raised ESR

67
Q

Clotting bloods of patient with anti phospholipid syndrome

A

Paradoxically, prolonged APTT

68
Q

Clotting bloods of patient with anti phospholipid syndrome

A

Paradoxically, prolonged APTT

Low platelets

69
Q

Cubital tunnel syndrome

A

cUbital tUnnel syndrome - Ulnar nerve - fUnny bone. Tingling in 4th and 5th

NO pain or tenderness over medial epicondyle (that’s medial epicondylitis)

70
Q

Lower back pain - prolapsed disc L4

A

on alL 4s - sensory loss anterior of knee

And reduced knee reflex (seen in L3 and l4)

71
Q

Lower back pain - prolapsed disc L3

A

Sensory loss over anterior thigh

Reduced knee reflex (seen in L3 and l4)

72
Q

Polymyositis and dermatomyositis Autoantibodies

A

Jo and ANA