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Flashcards in Rheumatology Step Up Deck (152):
1

associated findings in ANA-negative lupus

- arthritis, Raynaud's phenomenon, subacute cutaneous lupus
- risk of neonatal lupus in offspring

2

serology in ANA-negative lupus

Ro (antiSS-A) positive
ANA negative

3

CF in neonatal lupus

skin lesions
cardiac lesions - AV block, transposition
valvular and septal defects

4

criteria for dx. SLE (11)

malar rash
discoid lesions
photosensitivity
oral/nasal ulcers
arthritis
pericarditis/pleuritis
hematologic disease - cytopenias
renal disease - proteinuria
CNS - seizures/psychosis
ANAs
other ab - dsDNA, Smith, FP on VDRL

5

presence of what ab's is diagnostic of SLE?

anti-dsDNA
anti-Smith

6

how do you diagnose a flare of SLE?

rise in anti-dsDNA
fall in C3/C4 levels (CH50 is more sensitive)

7

which ab's are very SENSITIVE for SLE but not specific?

ANA

8

when Ro (SS-A) and Lo (SS-B) ab's are found in SLE, they are associated with...

neonatal lupus
subacute cutaneous lupus
sjogren's syndrome
complement deficiency
ANA-negative lupus

9

major use of ESR

diagnose/rule out inflammatory processes
monitor the course of inflammatory conditions

10

major use of CRP

mainly infection - much more sensitive and specific for ESR
- if > 15, bacterial infection is present

11

best tx. for SLE pts with acute flare

steroids

12

best long term tx. for constitutional, cutaneous and articular manifestations of SLE

hydroxychloroquine
NSAIDs for less severe symptoms

13

what should you recommend for pts on hydroxychloroquine tx for SLE

yearly eye examination bc of retinal toxicity

14

Tx. of active lupus nephritis

cyclophosphamide
azathioprine

15

MCC of death in SLE

renal failure
infections

16

drugs that may cause lupus-like syndrome

hydralazine
procainamide
isoniazid
quinidine

17

which two organ systems does drug-induced lupus NOT affect?

CNS and renal
- if these sx are present, it is NOT drug-induced

18

pathophysiology of scleroderma

cytokines stimulate fibroblasts to produce collagen; high quantity of collagen is responsible for symptoms

19

what symptom(s) is/are present in almost all pts with scleroderma?

Raynaud's
cutaneous fibrosis - thickening of skin of face and extremities

20

how do you dx. Raynauds?

nail-fold capillaroscopy - look for evidence of vessel damage

21

GI findings in scleroderma

dysphagia/reflux from esophageal immobility
delayed gastric emptying
abdominal distention
pseudo-obstruction

22

MCC of death in scleroderma

pulmonary involvement - pulmonary fibrosis or HTN (diffuse form only)

23

renal involvement in scleroderma

in diffuse form only
- renal crisis w/ rapid malignant HTN

24

ab specific for limited form of scleroderma

anti-centromere ab

25

ab specific for diffuse form of scleroderma

anti-topoisomerase I (anti-Scl70)

26

findings in Barium swallow in scleroderma

absence of peristaltic waves in lower 1/3 of esophagus
decreased LES tone

27

what predicts prognosis in scleroderma?

degree of skin involvement - difffuse form has worse prognosis than limited form

28

CREST syndrome

Calcinosis of digits
Raynaud's phenomenon
Esophageal dysmotility
Sclerodactyly
Telengiectasias

29

Tx. of scleroderma

symptomatic
- MSK = NSAIDs
- Raynauds = CCB
- skin findings = D-penicillamine
- renal = ACEi
- GI = PPIs/H2 blockers

30

20% of pts with scleroderma have...

Sjogrens syndrome

31

typical findings in anti-phospholipid syndrome

recurrent venous/arterial thrombosis
recurrent pregnancy loss
thrombocytopenia
livedo reticularis

32

lab findings in antiphospholipid syndrome

presence of lupus anticoagulant and/or anticardiolipin antibody
prolonged PTT or PT that is not corrected by adding plasma

33

Tx of antiphospholipid syndrome

anticoagulation - INR 2.5-3.5

34

primary Sjogren's syndrome

dry eyes and dry mouth, along w/ lymphocytic infiltration of minor salivary glands (histology)

35

secondary Sjogren's syndrome

dry eyes and dry mouth along with a connective tissue disease

36

MCC of death in Sjogren's

malignancy
- increased risk of non-Hodgkin's lymphoma

37

what test has high sensitivity and specificity for Sjogren's syndrome?

Schirmer test - filter paper inserted into eye to measure lacrimal gland output

38

most accurate test for dx Sjogren's

salivary gland biopsy (lip or parotid)
- not needed for diagnosis

39

Tx. for Sjogrens

1. pilocarpine or Cevimeline
2. artificial tears
3. NSAIDs/ steroids - arthritis

40

ab found in mixed CT disease

anti-U1-RNP ab

41

predom. cells implicated in RA

T helper cells - therefore, RA is not seen in pts with HIV/AIDs

42

which joints are characteristically NOT involved in RA?

DIP joints

43

what symptom is present in all patients?

morning stiffness

44

cutaneous finding that is pathognomic for RA

subcutaneous rheumatoid nodules (sacrum, elbows, occiput, achilles tendon)

45

pulmonary findings in RA

pleural effusions (low glucose)
pulmonary fibrosis
rheumatoid nodules in lung

46

cardiac findings in RA

rheumatoid nodules - conduction block
pericarditis

47

eye findings in RA

scleritis
scleromalacia - softening of sclera
dry eyes and mucous mbs

48

nervous system findings in RA

mononeuritic multiplex - damage to one or more peripheral nerves

49

Felty syndrome

triad of RA, splenomegaly and neutropenia

50

associated clinical findings in Felty's syndrome

anemia, thrombocytopenia
LAD
recurrent infections
high titres of RF

51

type of anemia seen in RA

normocytic, normochromic anemia

52

what must you screen for in every pt with RA prior to undergoing surgery or intubation?

cervical radiograph of spine to assess for evidence of C1-C2 subluxation/instability

53

poor prognostic indicators in RA

high RF titres
subcutaneous nodules
autoantibodies to RF
erosive arthritis

54

lab findings in RF

high titres of RF - more severe disease
anticitrullinated peptide (ACPA)
elevated ESR/CRP
normocytic, normochromic anemia

55

what are pts with positive RF and ACPA at risk for? how should you intervene?

erosive joint damage
- early tx. with DMARDs indicated

56

criteria for dx. of RA

1. inflammatory arthritis > 3 joints
2. sx. lasting 6 weeks
3. high ESR/CRP
4. serum RF or ACPA positive
5. radiographic changes

57

general tx. approach to RA

when pt presents with sx, start them on NSAIDs (steroids if NSAIDS not adequate relief); at the same time, start pt on DMARD therapy, taper NSAIDs/steroids after approx 6 weeks of tx and then use those PRN

58

first-line DMARD to start in RA

Methotrexate/Folate

59

side effects of Methotrexate

GI upset, oral ulcers
mild alopecia
bone marrow suppression
hepatocellular injury
pulmonary fibrosis

60

what should the patient have monitored while on Methotrexate therapy?

CBC every 2-3 months
LFTs
renal function

61

alternate first-line DMARD agents

leflunomide - same efficacy
hydroxychloroquine
sulfasalazine

62

when can you consider using anti-TNF inhibitors in RA?

if methotrexate cannot control disease - i.e. resistant RA

63

what can you do for a pt with severe RA with severe pain unresponsive to conservative measures?

joint replacement surgery

64

clear synovial fluid with < 200 WBCs and PMNs < 25%

normal

65

clear/yellow synovial fluid with < 2000 WBCs and < 25% PMNs

noninflammatory - OA/trauma

66

cloudy yellow synovial fluid with > 5000 cells and 50-70% PMNs

inflammatory arthritis

67

turbid, purulent synovial fluid with > 50 000 WBCs and > 75% PMNs

septic arthritis

68

what cell type plays key role in acute inflammation of gout?

PMN

69

radiographic changes in gout

punched out erosions with overhanging rim of cortical bone
- if no Hx of trauma, do not need XR for diagnosis

70

what happens if you do not treat an acute gouty attack?

nothing really...it lasts 7-10 days and resolves

71

pt comes in because of severe pain in his foot that caused him to awake from sleep; the joint appears erythematous, swollen and tender..what should you consider?

gout!!!

72

what is a tophi?

aggregations of urate crystals surrounded by giant cells in an inflammatory reaction; occurs after years of uncontrolled gout

73

common locations of tophi

extensor surfaces of forearms, elbows, knees
achilles tendon
pinna of ear

74

definitive diagnostic test in gout

joint aspiration and synovial fluid analysis

75

tx. of choice in acute gout

NSAIDs - esp. indomethacin
- avoid aspirin and acetaminophen

76

alternative drug in acute gout for pts who cannot take NSAIDs or did not respond to NSAIDs

colchicine
- 2nd line due to common GI side effects which result in low compliance

77

colchicine is contraindicated in..

renal insufficiency
cytopenia

78

when do you use oral steroids for acute gout?

if pt has no response to or cannot tolerate NSAIDs/colchicine
- 7-10 days of oral prednisone

79

what can probenicid or sulfinpyrazone be used for?

uricosuric drugs that increase renal excretion of uric acid; can use for gout prophylaxis in pts who are undersecretors

80

what are the uricosuric drugs contraindicated in?

if pt has history of renal stones

81

preferred drug for prophylaxis of gout

allopurinol - once daily dosing, 300 mg

82

what do you need to watch for when tx. pt with allopurinol?

Steven Johnson syndrome / rash

83

who can Febuxostat be Rx. to?

pts who are in need of gout prophylaxis but intolerant to allopurinol

84

what are serum uric acid levels useful for?

not used for diagnosis
- can monitor response to chronic gout tx.

85

RF of pseudogout (6)

age
osteoarthritis
hypothyroidism
hemochromatosis
hyperparathyroidism
Bartter's syndrome

86

what can you see on radiograph in pseudogout?

chondrocalcinosis - linear radiodense deposits in dark joint space on XR

87

tx. of pseudogout

symptomatic tx. - same as gout

88

slowly progressive weaknes and wasting of both distal and proximal muscles (arms/legs), MC in men and associated with absence of autoab's and low CK levels

inclusion body myositis

89

characteristic clinical feature of polymyositis/dermatomyositis (4)

symmetrical proximal muscle weakness
decreased muscle strength on testing
fine motor tasks usually preserved
myalgia and dysphagia common

90

which muscles are never involved in polymyositis/dermatomyositis?

ocular muscles
- ddx. w/ myasthenia and Lambert-Eaton

91

what features are unique to dermatomyositis?

Heliotrope rash
Gottron's papules
V sign/Shawl sign

92

associated findings in both dermatomyositis and polymyositis

conduction defects/ CHF
arthralgias
interstitial lung disease - one of leading causes of death

93

associated findings in dermatomyositis only

vasculitis - in children
increased incidence of malignancy

94

what additional measures should you take with a dermatomyositis patient?

ensure patient undergoes age appropriate cancer screening

95

what is the first step in diagnosis in suspected dermatomyositis/polymyositis?

check aldolase and CPK levels - will be elevated

96

what lab test can be used to monitor disease severity in myositis?

CK level

97

nonspecific markers of cell injury in myositis

aldolase, LDH, AST, ALT

98

what kinds of antibdodies are found in the inflammatory myopathies? (3)

anti-Jo1
anti-signal recognition particle
anti-Mi-2

99

when do you do an EMG study in inflammatory myopathy?

when biopsy is inconclusive
- will show short duration and low amplitude

100

definitive diagnosis of inflammatory myopathy

muscle biopsy

101

muscle biopsy showing perivascular and perimysial inflammation and fibrosis

dermatomyositis

102

muscle biopsy showing endomysial inflammation and fibrosis

polymyositis

103

earliest and severely affected muscle groups in poly/dermatomyositis

neck flexors
shoulder girdle
pelvic girdle

104

initial tx. in inflammatory myopathy

corticosteroids - monitor with CPK levels; continue until symptoms improve

105

what additional CF is found in inclusion body myositis that is not seen with dermato/polymyositis?

loss of deep tendon reflexes - nerves are not affected in the others

106

older female patient presents with stiffness in shoulder and hip regions after a period of inactivity; upon testing, you notice that muslce strength is normal - dx?

polymylagia rheumatica

107

what other disease is polymyalgia rheumatica associated with?

temporal arteritis

108

how can you diagnose polymyalgia rheumatica?

clinical diagnosis

109

what lab finding correlates with disease activity in polymyalgia rheumatica?

ESR - usually > 100

110

tx. of polymyalgia rheumatica

corticosteroids - response usually w/in 1-7 days; disease is generally self-limiting

111

CF in fibromyalgia

stiffness/body aches all over body
fatigue
disturbed sleep
anxiety/depression

112

diagnostic criteria of fibromyalgia

1. widespread pain including axial pain for atleast 3 months
2. pain in atleast 11/18 possible tender point sites

113

what drugs have shown beneficial in fibromyalgia?

SSRIs/TCAs

114

what is a prerequisite for the diagnosis of ankylosing spondylitis?

bilateral sacroilitis

115

young male comes in with low back pain and stiffness that is worse in the morning and gets better with activity; he also appears to have inflammation of the achilles tendon..what should you consider?

ankylosing spondylitis

116

aside from low back pain/stiffness, what else do pts with ankylosing spondylitis experience?

fractures with minimal trauma in late disease
chest pain and diminished chest expansion
constitutional symptoms
acute anterior uveitis/iridocyclitis

117

Schober test

measures spinal flexion - positive in ankylosing spondylitis

118

what is diagnostic of ankylosing spondylitis?

imaging showing sacroilitis or bamboo spine
- ESR may be elevated
- HLAb27 not needed

119

Tx. of ankylosing spondylitis (3)

NSAIDs - indomethacin (Sx)
Anti-TNF meds - for axial disease
Physical therapy

120

patient presents with asymmetric inflammatory oligoarthritis of LE 2 weeks after enteric or genitourinary infection

reactive arthritis

121

what should you consider if any patient has acute asymmetric arthritis that progresses sequentially from one joint to another

Reactive arthritis

122

patient presents with arthritis, urethritis and ocular inflammation - dx?

Reiters syndrome

123

undifferentiated spondyloarthropathy

when a patient has features of reactive arthritis but not evidence of previous infection

124

diagnosis of reactive arthritis

synovial fluid analysis

125

first line therapy for reactive arthritis

NSAIDs

126

what should you give a pt with reactive arthritis if NSAIDs do not provide relief?

try sulfasalazine or immunosuppressives (azathioprine)

127

should you treat patient with reactive arthritis with antibiotics?

usually not given - but may be beneficial in non-gonococcal urethritis (tetracycline)

128

pt presents with arthritis involving mainly the DIP joints; his nails also show pitting - what should you look for in this patient?

psoriasis - usually have skin disease for months-years before arthritis

129

finding on biopsy of temporal artery in GCA

giant cells; mononuclear cell infiltration or granulomatous inflammation

130

first line tx. of temporal arteritis

high dose steroids
- IV if visual loss is notes
- oral if no visual symptoms

131

young Asian woman presents to you with constitutional symptoms and pain/tenderness over vessels; on P/E you note decreased peripheral pulses, discrepancies of BP as well as arterial bruits

Takayasu arteritis

132

what is Takayasu arteritis?

granulomatous vasculitis of aortic arch and its major branches leading to stenosis or narrowing of vessels

133

how do you diagnose Takayasu arteritis?

arteriogram

134

Tx. of takayasu arteritis

steroids
treat HTN
surgery or angioplasty/bypass grafting - if severe

135

middle aged pt presents with new onset asthma and skin lesions (palpable purpura) - what should you consider?

Churg Strauss

136

definitive diagnosis of Churg Strauss

biopsy of lung/skin - prominence of eosinophils
positive p-ANCA

137

Tx. of Churg Strauss

steroids - prednisone

138

lab findings in Wegener's granulomatosis

elevated ESR
normochromic normocytic anemia
hematuria
positive C-ANCA
thrombocytopenia

139

how can you confirm diagnosis of Wegeners?

open lung biopsy
- often biopsy of nasal septum is done (less invasive)

140

what Tx. is used to induce remission in Wegeners?

cyclophosphamide + corticosteroids

141

what drugs can be used in systemic resistant Wegener's?

rituximab or IVIG AND
plasmaphoresis

142

CF in PAN

fever, weakness
weight loss
myalgias/arthralgias
abdominal pain - bowel angina
HTN
mononeuritic multiplex - foot/wrist drop
livedo reticularis

143

what should you consider in a diabetic with mononeuropathy (foot or wrist drop)?

PAN

144

which organ system is spared in PAN?

lungs

145

Dx. of PAN

nerve biopsy
mesenteric angiography

146

what can PAN be associated with?

hep B
HIV
drug reactions

147

tx. of PAN

start with steroids
- if severe, add cyclophosphamide
- tx. underlying HepB

148

pt comes in with recurrent oral and genital ulcerations, arthritis, uveitis, CNS involvement, fever and weight loss - dx?

Behcet's syndrome

149

Tx. of Behcet's syndrome

steroids

150

young smoker presents with cold, cyanotic distal extremities, paresthesias and noticeable ulcerations of digits - dx?

Buergers disease

151

young child develops palpable purpura on butt/legs, arthralgias and GI symptoms following an upper respiratory infection

Henoch-Schonlein purpura

152

Tx of Henoch-Schonlein purpura

usually self limited
- give steroids if severe