Rheumatology Step Up Flashcards

1
Q

associated findings in ANA-negative lupus

A
  • arthritis, Raynaud’s phenomenon, subacute cutaneous lupus

- risk of neonatal lupus in offspring

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2
Q

serology in ANA-negative lupus

A

Ro (antiSS-A) positive

ANA negative

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3
Q

CF in neonatal lupus

A

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

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4
Q

criteria for dx. SLE (11)

A
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
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5
Q

presence of what ab’s is diagnostic of SLE?

A

anti-dsDNA

anti-Smith

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6
Q

how do you diagnose a flare of SLE?

A

rise in anti-dsDNA

fall in C3/C4 levels (CH50 is more sensitive)

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7
Q

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

A

ANA

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8
Q

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

A
neonatal lupus
subacute cutaneous lupus
sjogren's syndrome
complement deficiency
ANA-negative lupus
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9
Q

major use of ESR

A

diagnose/rule out inflammatory processes

monitor the course of inflammatory conditions

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10
Q

major use of CRP

A

mainly infection - much more sensitive and specific for ESR

- if > 15, bacterial infection is present

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11
Q

best tx. for SLE pts with acute flare

A

steroids

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12
Q

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

A

hydroxychloroquine

NSAIDs for less severe symptoms

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13
Q

what should you recommend for pts on hydroxychloroquine tx for SLE

A

yearly eye examination bc of retinal toxicity

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14
Q

Tx. of active lupus nephritis

A

cyclophosphamide

azathioprine

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15
Q

MCC of death in SLE

A

renal failure

infections

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16
Q

drugs that may cause lupus-like syndrome

A

hydralazine
procainamide
isoniazid
quinidine

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17
Q

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

A

CNS and renal

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

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18
Q

pathophysiology of scleroderma

A

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

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19
Q

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

A

Raynaud’s

cutaneous fibrosis - thickening of skin of face and extremities

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20
Q

how do you dx. Raynauds?

A

nail-fold capillaroscopy - look for evidence of vessel damage

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21
Q

GI findings in scleroderma

A

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

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22
Q

MCC of death in scleroderma

A

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

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23
Q

renal involvement in scleroderma

A

in diffuse form only

- renal crisis w/ rapid malignant HTN

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24
Q

ab specific for limited form of scleroderma

A

anti-centromere ab

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25
Q

ab specific for diffuse form of scleroderma

A

anti-topoisomerase I (anti-Scl70)

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26
Q

findings in Barium swallow in scleroderma

A

absence of peristaltic waves in lower 1/3 of esophagus

decreased LES tone

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27
Q

what predicts prognosis in scleroderma?

A

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

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28
Q

CREST syndrome

A
Calcinosis of digits
Raynaud's phenomenon
Esophageal dysmotility
Sclerodactyly
Telengiectasias
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29
Q

Tx. of scleroderma

A

symptomatic

  • MSK = NSAIDs
  • Raynauds = CCB
  • skin findings = D-penicillamine
  • renal = ACEi
  • GI = PPIs/H2 blockers
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30
Q

20% of pts with scleroderma have…

A

Sjogrens syndrome

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31
Q

typical findings in anti-phospholipid syndrome

A

recurrent venous/arterial thrombosis
recurrent pregnancy loss
thrombocytopenia
livedo reticularis

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32
Q

lab findings in antiphospholipid syndrome

A

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

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33
Q

Tx of antiphospholipid syndrome

A

anticoagulation - INR 2.5-3.5

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34
Q

primary Sjogren’s syndrome

A

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

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35
Q

secondary Sjogren’s syndrome

A

dry eyes and dry mouth along with a connective tissue disease

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36
Q

MCC of death in Sjogren’s

A

malignancy

- increased risk of non-Hodgkin’s lymphoma

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37
Q

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

A

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

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38
Q

most accurate test for dx Sjogren’s

A

salivary gland biopsy (lip or parotid)

- not needed for diagnosis

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39
Q

Tx. for Sjogrens

A
  1. pilocarpine or Cevimeline
  2. artificial tears
  3. NSAIDs/ steroids - arthritis
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40
Q

ab found in mixed CT disease

A

anti-U1-RNP ab

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41
Q

predom. cells implicated in RA

A

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

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42
Q

which joints are characteristically NOT involved in RA?

A

DIP joints

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43
Q

what symptom is present in all patients?

A

morning stiffness

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44
Q

cutaneous finding that is pathognomic for RA

A

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

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45
Q

pulmonary findings in RA

A

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

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46
Q

cardiac findings in RA

A

rheumatoid nodules - conduction block

pericarditis

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47
Q

eye findings in RA

A

scleritis
scleromalacia - softening of sclera
dry eyes and mucous mbs

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48
Q

nervous system findings in RA

A

mononeuritic multiplex - damage to one or more peripheral nerves

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49
Q

Felty syndrome

A

triad of RA, splenomegaly and neutropenia

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50
Q

associated clinical findings in Felty’s syndrome

A

anemia, thrombocytopenia
LAD
recurrent infections
high titres of RF

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51
Q

type of anemia seen in RA

A

normocytic, normochromic anemia

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52
Q

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

A

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

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53
Q

poor prognostic indicators in RA

A

high RF titres
subcutaneous nodules
autoantibodies to RF
erosive arthritis

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54
Q

lab findings in RF

A

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

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55
Q

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

A

erosive joint damage

- early tx. with DMARDs indicated

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56
Q

criteria for dx. of RA

A
  1. inflammatory arthritis > 3 joints
  2. sx. lasting 6 weeks
  3. high ESR/CRP
  4. serum RF or ACPA positive
  5. radiographic changes
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57
Q

general tx. approach to RA

A

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

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58
Q

first-line DMARD to start in RA

A

Methotrexate/Folate

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59
Q

side effects of Methotrexate

A
GI upset, oral ulcers
mild alopecia
bone marrow suppression
hepatocellular injury
pulmonary fibrosis
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60
Q

what should the patient have monitored while on Methotrexate therapy?

A

CBC every 2-3 months
LFTs
renal function

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61
Q

alternate first-line DMARD agents

A

leflunomide - same efficacy
hydroxychloroquine
sulfasalazine

62
Q

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

A

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

63
Q

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

A

joint replacement surgery

64
Q

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

A

normal

65
Q

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

A

noninflammatory - OA/trauma

66
Q

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

A

inflammatory arthritis

67
Q

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

A

septic arthritis

68
Q

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

A

PMN

69
Q

radiographic changes in gout

A

punched out erosions with overhanging rim of cortical bone

- if no Hx of trauma, do not need XR for diagnosis

70
Q

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

A

nothing really…it lasts 7-10 days and resolves

71
Q

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?

A

gout!!!

72
Q

what is a tophi?

A

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

73
Q

common locations of tophi

A

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

74
Q

definitive diagnostic test in gout

A

joint aspiration and synovial fluid analysis

75
Q

tx. of choice in acute gout

A

NSAIDs - esp. indomethacin

- avoid aspirin and acetaminophen

76
Q

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

A

colchicine

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

77
Q

colchicine is contraindicated in..

A

renal insufficiency

cytopenia

78
Q

when do you use oral steroids for acute gout?

A

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

79
Q

what can probenicid or sulfinpyrazone be used for?

A

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

80
Q

what are the uricosuric drugs contraindicated in?

A

if pt has history of renal stones

81
Q

preferred drug for prophylaxis of gout

A

allopurinol - once daily dosing, 300 mg

82
Q

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

A

Steven Johnson syndrome / rash

83
Q

who can Febuxostat be Rx. to?

A

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

84
Q

what are serum uric acid levels useful for?

A

not used for diagnosis

- can monitor response to chronic gout tx.

85
Q

RF of pseudogout (6)

A
age
osteoarthritis
hypothyroidism
hemochromatosis
hyperparathyroidism
Bartter's syndrome
86
Q

what can you see on radiograph in pseudogout?

A

chondrocalcinosis - linear radiodense deposits in dark joint space on XR

87
Q

tx. of pseudogout

A

symptomatic tx. - same as gout

88
Q

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

A

inclusion body myositis

89
Q

characteristic clinical feature of polymyositis/dermatomyositis (4)

A

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

90
Q

which muscles are never involved in polymyositis/dermatomyositis?

A

ocular muscles

- ddx. w/ myasthenia and Lambert-Eaton

91
Q

what features are unique to dermatomyositis?

A

Heliotrope rash
Gottron’s papules
V sign/Shawl sign

92
Q

associated findings in both dermatomyositis and polymyositis

A

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

93
Q

associated findings in dermatomyositis only

A

vasculitis - in children

increased incidence of malignancy

94
Q

what additional measures should you take with a dermatomyositis patient?

A

ensure patient undergoes age appropriate cancer screening

95
Q

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

A

check aldolase and CPK levels - will be elevated

96
Q

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

A

CK level

97
Q

nonspecific markers of cell injury in myositis

A

aldolase, LDH, AST, ALT

98
Q

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

A

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

99
Q

when do you do an EMG study in inflammatory myopathy?

A

when biopsy is inconclusive

- will show short duration and low amplitude

100
Q

definitive diagnosis of inflammatory myopathy

A

muscle biopsy

101
Q

muscle biopsy showing perivascular and perimysial inflammation and fibrosis

A

dermatomyositis

102
Q

muscle biopsy showing endomysial inflammation and fibrosis

A

polymyositis

103
Q

earliest and severely affected muscle groups in poly/dermatomyositis

A

neck flexors
shoulder girdle
pelvic girdle

104
Q

initial tx. in inflammatory myopathy

A

corticosteroids - monitor with CPK levels; continue until symptoms improve

105
Q

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

A

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

106
Q

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?

A

polymylagia rheumatica

107
Q

what other disease is polymyalgia rheumatica associated with?

A

temporal arteritis

108
Q

how can you diagnose polymyalgia rheumatica?

A

clinical diagnosis

109
Q

what lab finding correlates with disease activity in polymyalgia rheumatica?

A

ESR - usually > 100

110
Q

tx. of polymyalgia rheumatica

A

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

111
Q

CF in fibromyalgia

A

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

112
Q

diagnostic criteria of fibromyalgia

A
  1. widespread pain including axial pain for atleast 3 months

2. pain in atleast 11/18 possible tender point sites

113
Q

what drugs have shown beneficial in fibromyalgia?

A

SSRIs/TCAs

114
Q

what is a prerequisite for the diagnosis of ankylosing spondylitis?

A

bilateral sacroilitis

115
Q

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?

A

ankylosing spondylitis

116
Q

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

A

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

117
Q

Schober test

A

measures spinal flexion - positive in ankylosing spondylitis

118
Q

what is diagnostic of ankylosing spondylitis?

A

imaging showing sacroilitis or bamboo spine

  • ESR may be elevated
  • HLAb27 not needed
119
Q

Tx. of ankylosing spondylitis (3)

A

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

120
Q

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

A

reactive arthritis

121
Q

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

A

Reactive arthritis

122
Q

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

A

Reiters syndrome

123
Q

undifferentiated spondyloarthropathy

A

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

124
Q

diagnosis of reactive arthritis

A

synovial fluid analysis

125
Q

first line therapy for reactive arthritis

A

NSAIDs

126
Q

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

A

try sulfasalazine or immunosuppressives (azathioprine)

127
Q

should you treat patient with reactive arthritis with antibiotics?

A

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

128
Q

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

A

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

129
Q

finding on biopsy of temporal artery in GCA

A

giant cells; mononuclear cell infiltration or granulomatous inflammation

130
Q

first line tx. of temporal arteritis

A

high dose steroids

  • IV if visual loss is notes
  • oral if no visual symptoms
131
Q

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

A

Takayasu arteritis

132
Q

what is Takayasu arteritis?

A

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

133
Q

how do you diagnose Takayasu arteritis?

A

arteriogram

134
Q

Tx. of takayasu arteritis

A

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

135
Q

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

A

Churg Strauss

136
Q

definitive diagnosis of Churg Strauss

A

biopsy of lung/skin - prominence of eosinophils

positive p-ANCA

137
Q

Tx. of Churg Strauss

A

steroids - prednisone

138
Q

lab findings in Wegener’s granulomatosis

A
elevated ESR
normochromic normocytic anemia
hematuria
positive C-ANCA
thrombocytopenia
139
Q

how can you confirm diagnosis of Wegeners?

A

open lung biopsy

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

140
Q

what Tx. is used to induce remission in Wegeners?

A

cyclophosphamide + corticosteroids

141
Q

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

A

rituximab or IVIG AND

plasmaphoresis

142
Q

CF in PAN

A
fever, weakness
weight loss
myalgias/arthralgias
abdominal pain - bowel angina
HTN
mononeuritic multiplex - foot/wrist drop
livedo reticularis
143
Q

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

A

PAN

144
Q

which organ system is spared in PAN?

A

lungs

145
Q

Dx. of PAN

A

nerve biopsy

mesenteric angiography

146
Q

what can PAN be associated with?

A

hep B
HIV
drug reactions

147
Q

tx. of PAN

A

start with steroids

  • if severe, add cyclophosphamide
  • tx. underlying HepB
148
Q

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

A

Behcet’s syndrome

149
Q

Tx. of Behcet’s syndrome

A

steroids

150
Q

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

A

Buergers disease

151
Q

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

A

Henoch-Schonlein purpura

152
Q

Tx of Henoch-Schonlein purpura

A

usually self limited

- give steroids if severe