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
Features of adult Stills disease
``` 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)
26
Management of myasthenic crisis
Plasmapheresis or IV immunoglobulin Plasma is quicker but more expensive equipment
27
Most common target of pANCA
MPO | Myeloperoxidase
28
Most common target of pANCA
MPO | Myeloperoxidase
29
Most common target of cANCA
PR3 | Proteinase 3
30
What antibody is usually found in Myasthenia gravis
(nicotinic) Acetylcholinesterase receptor antibodies in 90%
31
Patient with MS, on Natalizumab, acutely confused, with hemiparesis
That drug can reactivate JC virus causing progressive multifocal leukoencephalopathy PML
32
Compliment deficiency that predisposes to SLE
Deficiency in C1q, C1rs, C2 and C4 predisposes to immune complex disease such as SLE
33
Patient with dermatomyositis... What is most important first investigation?
Exclude malignancy. ~25% diagnosed with dermatomyositis have an underlying malignancy (typically ovarian, breast and lung cancer), this prevalence increases in the elderly.
34
Poor prognostic factors related to RA
Rh F positive Anti CCP autoantibodies HLA DR4 Early erosions and extra articular features like nodules
35
How does Rheumatoid factor correspond with severity / progression in RA
High levels of Rh F are assoicated with severe progressive disease, but are not a marker of disease progression
36
What type of HLA allele is most associated with Rheumatoid arthritis?
HLA DR4 | RA - R4
37
Myalgia and fatigue, mononeuritis, glomerulonephritis... Diagnosis and antibody
Microscopic polyangiitis | pANCA
38
Most common complications of diffuse cutaneous systemic sclerosis
Most common cause of death is resp (80%) ILD and pulmonary arterial hypertension
39
HLA memory aid
``` 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
Features of Adult onset Stills disease
Arthralgia, fever, salmon Mac pap rash, lymphadenopathy RF and ANA negative
41
Management of Reynaud's....
Secondary care First line CCB like nifedipine Second line IV prostacycline like epoprostenol
42
What type of collagen is primarily affected by Ehlers Danlos Syndrome?
Collagen type 3 3hlers-danlos
43
SLE - antibodies associated with congenital heart block
Anti Ro Road...block
44
Poor prognostic features of RA
``` RF +ve Anti CCP antibodies Slow onset HLA DR4 XR early erosions Extra articular features like nodules ``` Possibly female
45
Anti-GQ1b antibodies
Present in 90% of Miller Fischer syndrome
46
Drugs that exacerbate MG
Beta blockers Gentamicin Lithium Phenytoin
47
SLE - antibodies associated with congenital heart block
Anti Ro Ro ro ro your boat
48
Bony lump at DIP
Heberden's OA
49
Bony lumps at PIP
bouchards RA
50
wegners / granulomatosis with polyangiitis features
Ent features like sinusitis
51
Goodpastures features
Pulmonary haemorrhage
52
anti-smooth muscle antibodies (SMA)
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
RA scleritis Vs episcleritis
Scleritis is Sore | ePiscLEritis is PainLess
54
Which immunoglobulin are most common?
GAMDE
55
Management of SLE
Hydroxychloroquine
56
Management of MG
pyridostigmine
57
Diagnosis of ankylosing spondylitis
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
Which antibiotics should you avoid when on methotrexate
Trimethoprim and co-trimoxazole They all work by inhibiting the enzyme dihydrofolate reductase, so can cause myelosuppression
59
Which cytokine is most important in pathophys of RA
Tumour necrosis factor
60
Related cramps and myoglobinuria after exercise
McArdle's disease Auto R type V glycogen storage disease
61
Etanercept TNF-alpha adverse effect
Reactivation of TB
62
Symps and antibodies of mixed connective tissue disease
Anti RNP Reynaud's N - no synovitis but swollen hands Pain in muscles and joints
63
mixed connective tissue disease prognosis
1/3 long-term remission Chronic symps Develop severe systemic involvement and premature death
64
What feature is isn't commonly seen in drug induced lupus
Glomerulonephritis
65
Medium vessel vasculitis assx w hep B
Polyarteritis nodosa No Autoantibodies likely to be positive
66
Fever malaise foot drop, livedo reticularis , background of hep b
Polyarteritis nodosa No antibody likely positive, raised ESR
67
Clotting bloods of patient with anti phospholipid syndrome
Paradoxically, prolonged APTT
68
Clotting bloods of patient with anti phospholipid syndrome
Paradoxically, prolonged APTT | Low platelets
69
Cubital tunnel syndrome
cUbital tUnnel syndrome - Ulnar nerve - fUnny bone. Tingling in 4th and 5th NO pain or tenderness over medial epicondyle (that's medial epicondylitis)
70
Lower back pain - prolapsed disc L4
on alL 4s - sensory loss anterior of knee And reduced knee reflex (seen in L3 and l4)
71
Lower back pain - prolapsed disc L3
Sensory loss over anterior thigh | Reduced knee reflex (seen in L3 and l4)
72
Polymyositis and dermatomyositis Autoantibodies
Jo and ANA