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PPS - From Nelson's > Rheuma > Flashcards

Rheuma Flashcards

1
Q

Differentials fever

A

Systemic JIA, SLE, vasculitis, acute rheumatic fever, sarcoidosis, MCTD

Malignancies, infections and post-infectious syndromes, inflammatory bowel disease, periodic fever (autoinflammatory) syndromes, Kawasaki disease, HSP

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2
Q

Differentials arthralgia

A

JIA, SLE, rheumatic fever, JDM, vasculitis, scleroderma, sarcoidosis

Hypothyroidism, trauma, endocarditis, other infections, pain syndromes, growing pains, malignancies, overuse syndromes

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3
Q

Differentials weakness

A

JDM, myositis secondary to SLE, MCTD, and deep localized scleroderma

Muscular dystrophies, metabolic and other myopathies, hypothyroidism

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4
Q

Differential chest pain

A

Juvenile rheumatoid arthritis, SLE (with associated pericarditis or costochondritis)

Costochondritis (isolated), rib fracture, viral pericarditis, panic attack, hyperventilation

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5
Q

Differentials back pain

A

Enthesitis related arthritis, juvenile ankylosing spondylitis

Vertebral compression fracture, diskitis, intraspinal tumor, spondylolysis, spondylolisthesis, bone marrow–occupying malignancy, pain syndromes, osteomyelitis, muscle spasm, injury

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6
Q

Differentials fatigue

A

SLE, JDM, MCTD, vasculitis, JIA

Pain syndromes, chronic infections, chronic fatigue syndrome, depression

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7
Q

Differentials malar rash

A

SLE, JDM

Sunburn, parvovirus B19 (fifth disease), Kawasaki disease

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8
Q

Differentials oral ulcer

A

SLE, Behçet disease

HSV infection, PFAPA syndrome

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9
Q

Differentials Purpuric rash

A

Vasculitis, e.g., ANCA-associated vasculitis, HSP

Meningococcemia, thrombocytopenia, clotting disorders

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10
Q

Gottron papules

Heliotrope rash, periungual telangiectasias

A

JDM

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11
Q

Differential Arthritis

A

Juvenile idiopathic arthritis, SLE, vasculitis, HSP, MCTD, scleroderma, acute rheumatic fever, reactive arthritis

Postviral arthritis, reactive arthritis, trauma, infection, Lyme disease, Kawasaki disease, malignancy, overuse syndromes

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12
Q

Differential Gottron papules non rheuma

A

Psoriasis, eczema

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13
Q

Moa nsiads

A

Inhibit Cox cyclooxygenase which is critical in production of prostaglandin

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14
Q

Nsaid use skin reaction

Small hypopigmented depressed scars in areas of minor skin trauma

A

Pseudoporphyria

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15
Q

Side effect of hydroxychloroquine

A

Retinal toxicity

Irreversible color blindness or loss of central vision

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16
Q

Serious side effect of rituximab

A

Multifocal leukoencephalopathy

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17
Q

Most common rheumatic problem in kids

A

Jia

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18
Q

Criteria JIA

A

Age at onset: <16 yr
Arthritis (swelling or effusion, or the presence of 2 or more of the following signs: limitation of range of motion, tenderness or pain on motion, increased heat) in ≥1 joint
Duration of disease: ≥6 wk
Onset type defined by type of articular involvement in the 1st 6 mo after onset:
Polyarthritis: ≥5 inflamed joints
Oligoarthritis: ≤4 inflamed joints
Systemic-onset disease: arthritis with rash and a characteristic quotidian fever
Exclusion of other forms of juvenile arthritis

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19
Q

arthritis fever hepatosplenomegaly lymphadenopathy serositis

A

systemic JIA

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20
Q

cutaneous hypersensitivity to trauma in jia

A

koebner phenomenon

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21
Q

rare but fatal complication of systemic jia

A

macrophage activation syndrome
or hemophagocytic syndrome
or hemophagocytic lymphohistiocytosis

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22
Q 
acute profound anemia
thrombocytopenia
leukopenia
high fever
lymphadenopathy
hepatosplenomegaly
prolonged pt and ptt
A

macrophage activation syndrome
or hemophagocytic syndrome
or hemophagocytic lymphohistiocytosis

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23
Q

treatment of macrophage activation syndrome
or hemophagocytic syndrome
or hemophagocytic lymphohistiocytosis

A

mppt
cyclosporine
anikinra

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24
Q

quality of arthritis in ARF

A

exquisite joint pain

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25
hip pain due to idiopathic avascular necrosis of femoral head
Legg–Calvé–Perthes disease
26
ANA + in kids with JIA is associated with
chronic uveitis
27
complications of anterior uveitis in jia
posterior synechiae, cataracts, band keratopathy, blindness
28
predictors of severe disease in jia
``` young age at onset RF + rheumatoid nodules + anti CCP antibodies large number of affected joints wrist/hands affected ```
29
reactive arthritis occurs after
Salmonella sp., Shigella exneri, Yersinia enterocolitica, Campylobacter jejuni, Chlamydia trachomatis
30
arthritis uveitis urethritis
Reiter syndrome
31
rubella hepa B typically affect __
small joints
32
mumps varicella typically affect _
large joints
33
preceded by respiratory infection severe hip pain normal ESR, WBC in cbc on utz noted effusion/widening of joint space
transient synovitis | or toxic synovitis
34
charcs of biopsy of sle lesion
hyperkeratosis follicular plugging infiltration of mononuclear cells into the dermal/epidermal junction
35
immunofluorescence of both affected and not affected skin reveal disposition of immune complexes w/in dermal-epidermal junction
lupus band test
36
drugs associated with drug induced lupus
Minocycline, procainamide, hydralazine, isoniazid, penicillamine, diltiazem, interferon-α, methyldopa, chlorpromazine, etanercept, infliximab, adalimumab
37
how to diagnose sle
> or 4 (at least 1 clinical and 1 lab) OR biopsy proven lupus nephritis with positive ANA or anti DNA ``` clinical criteria: acute/chronic lupus oral ulcers alopecia arthritis serositis renal neuro hemolytic anemia leukopenia thrombocytopenia ``` ``` immuno critera: ANA anti DNA anti SM antiphospholipid antibody low complement (c3, c4, ch50) direct coombs ```
38
t or f | ana levels correlate with disease severity
false
39
what lab marker correlates with disease severity
anti dsDNA levels | particularly anti Smith antibody
40
most common complications/death
infection complication of glomerulonephritis neuropsychiatric disorders
41
side effects of cyclophosphamide
hemorrhagic cystitis premature gonadal failure malignancy
42
conduction abnormalities in neonatal lupus detected at. ____ aog
16wks aog
43
which maternal antobodies are transferred to fetus
``` anti Ro anti La (SSB) ```
44
__% recurrence with congenital heart block in next baby (if prev baby has heart block)
15%
45
proximal muscle weakness | heliotrope rash
juvenile dermatomyositis
46
rash in JDM over neck and chest due to photosensitivity
shawl sign
47
thickened erythematous scaly rash on palms anti Jo1 antibodies JDM
mechanic's hands
48
proximal muscle weakness symptoms
``` difficulty climbing combing hair getting out of bed postiive gowers sign head lag ```
49
associated with longstanding disease | JDM
lipodystrophy | calcinosis
50
what deposited in calcinosis | JDM
calcium phosphate hydroxyapatite fluoroapatite
51
findings EMG NCV in JDM
EMG myopathy and denervation with increased insertional activity, fibrillations, and sharp waves NCV normal
52
diagnosis of JDM
Classic rash: Heliotrope rash of the eyelids Gottron papules Plus 3 of the following: Weakness: Symmetric, Proximal Muscle enzyme elevation (≥1): CK, Aspartate aminotransferase, Lactate dehydrogenase, Aldolase Electromyographic changes: Short, small polyphasic motor unit potentials, Fibrillation, Positive sharp waves, Insertional irritability, Bizarre, high-frequency repetitive discharges Muscle biopsy: Necrosis, inflammation
53
what is given to reduce toxicity and side effects from folate inhibition in methotrexate use
folic acid
54
most common visceral manifestation of scleroderma
pulmonary
55
previously known as CREST syndrom
CREST - calcinosis cutis, raynaud, esophageal dysfxn, telangiectasia now systemic sclerosis
56
order of raynauds in cold/stress
blanching -> cyanosis -> erythema
57
how to diagnose juvenile systemic sclerosis | major criterion
``` MAJOR CRITERION (REQUIRED)* Proximal skin sclerosis/induration of the skin proximal to metacarpophalangeal or metatarsophalangeal joints ```
58
how to diagnose juvenile systemic sclerosis | minor criterion
AT LEAST 2 REQUIRED Cutaneous: sclerodactyly Peripheral vascular: Raynaud phenomenon, nailfold capillary abnormalities (telangiectasias), digital tip ulcers Gastrointestinal: dysphagia, gastroesophageal reflux Cardiac: Arrhythmias, heart failure Renal: Renal crisis, new-onset arterial hypertension Respiratory: pulmonary fibrosis (high-resolution computed tomography/radiography), decreased diffusing capacity for carbon monoxide, pulmonary arterial hypertension Neurologic: neuropathy, carpal tunnel syndrome Musculoskeletal: tendon friction rubs, arthritis, myositis Serologic: antinuclear antibodies—SSc-selective autoantibodies (anticentromere, antitopoisomerase I [Scl-70], anti brillarin, anti-PM/Scl, anti brillin or anti-RNA polymerase I or III
59
history of recurrent painful shallow that don't scar (if buccal, lips, tonge) or scar (genitals) uveitis erythema nodosum/pseudofolliculitis
behcet
60
autosomal recessive bried acute fever episodes polyserositis assoc with AA amyloidosis
familial mediterranean fever
61
progressive shrinkage and degeneration of the tissues beneath the skin, usually on only one side of the face (hemifacial atrophy) form of localized scleroderma
Parry–Romberg syndrome
62
``` autosomal recessive severe mental retardation ataxia myopathy cataracts failure to thrive decreased mevalonate kinase recurrent fever lasting 3-7days ab pain diarrhea nausea vomiting arthalgia ulcer elevated igD ```
dutch fever | or hyperimmunoglobulinemia D syndrome
63
``` autosomal dominant periodic fever 4-6 days severe ab pain nausea vomiting arthritis myalgia rash conjunctivitis unilateral periorbital edema TNF receptor AA amyloidosis develops in 25% ```
TRAPS | tumor necrosis factor receptor associated periodic syndrome
64
``` autosomal dominant CIAS1 gene -> cryopyrin periodic fever skin rash on biopsy noted perivascular infiltrates of PMN leukocytes polyarthralgia triggered by cold exposure ```
familial cold autoinflammatory syndrome
65
``` periodic fever aphthous stomatitis pharyngititis adenitis 2-5 yrs old ab pain arthralgia ```
marshall syndrome
66
autosomal dominant | granulomatous deposition in skin, eye,joint
blau syndrome
67
cardinal feature of sarcoidosis
noncaseating epitheloid granulomatous lesions
68
what are located in center of granulomatous lesions in sarcoidosis
macrophage epitheliod multinucleated giant cell cd4 t lymphocye
69
associated lab finding in sarcoidosis
hypercalcemia | vit D excess 1,25 OH VitD
70
other name for kawasaki
mucocutaneous lymph node syndrome | infantile polyarteritis nodosa
71
leading cause of acquired heart disease in developed countries
kawasaki
72
kawasaki affect __ sized arteries
medium
73
histopath exam of kawasaki
edema of endothelial and smooth muscle cells with intense inflammatory infiltration of the vascular wall initially by pmn then by macrophages, lymphocytes and plasma cells
74
how to diagnose kawasaki
``` FEVER PLUS 4: bilateral non exudative conjunctival injection with limbal sparing strawberry tongue (erythema of mucosa) dry cracked lips edema and erythema of hands and feet rash nonsuppurative LAD unilateral >1.5cm ```
75
giant coronary aneurysm __mm internal diameter
8mm | greatest risk of rupture
76
3 clinical phases of kawasaki
acute febrile. 1-2 weeks subacute 2 wk convalescent 6-8
77
which phase of kawaski has highest risk of death due to development of aneurysms
subacute
78
classification of aneurysm in kawasaki
small <5mm medium 508mm giant >8mm
79
when is 2d echo done in kawasaki
at diagnosis again after 2-3 weeks of illness 1 yr later (if normal 2decho) then f/u every 5 yr
80
MMR vaccine must be deferred __ mo after IVIG
11 months
81
most common vasculitis in kids
HSP
82
characteristic of HSP
leukocytoclastic vasculitis and igA deposition in the small vessels of skin, joints, gi, kidney
83
skin biopsy of HSP
vasculitis of the dermal capillaries and post capillary venules
84
diagnosis of HSP (american)
Two of the following criteria must be present: • Palpable purpura • Age at onset ≤20 yr • Bowel angina (postprandial abdominal pain, bloody diarrhea) • Biopsy demonstrating intramural granulocytes in small arterioles and/or venules
85
diagnosis HSP (european)
Palpable purpura (in absence of coagulopathy or thrombocytopenia) and 1 MORE: • Abdominal pain (acute, diffuse, colicky pain) • Arthritis or arthralgia • Biopsy of affected tissue demonstrating predominant immunoglobulin A deposition • Renal involvement (proteinuria >3 grams/24 hr), hematuria or red cell casts
86
age of onset takayasu
10 to 40yrs old
87
pulseless disease
takayasu arteritis
88
most commonly involed vessels in takayasu arteritis
subclaian renal carotid
89
proposed classification of pedia takayasu arteritis
Angiographic abnormalities (conventional, CT, or magnetic resonance angiography) of the aorta or its main branches and ONE OF FF: • Decreased peripheral artery pulse(s) and/or claudication of extremities • Blood pressure difference between arms or legs of >10 mm Hg • Bruits over the aorta and/or its major branches • Hypertension (de ned by childhood normative data) • Elevated acute phase reactant (erythrocyte sedimentation rate or C-reactive protein)
90
gold standard in diagnosis of takayasu
arteriography of aorta and major branches: carotid, subclavina, pulmo, renal, mesenteric
91
biopsy result in PAN
necrotizing vasculitis with granulocytes and monocytes infiltrating
92
gold standard imaging for PAN | finding?
arteriography | beads on a string
93
three forms of ANCA (antineutrophil circulating antibodies) asoociated vasculitis
wegener granulomatosis microscopic polyangiitis churg strauss syndrome
94
mean age diagnosis wegener | male or female predominance?
14 yrs | female
95
biopsy in wegener granulomatosis
necrotizing vasculitis kidney: cresentic GN with little to no immune complex deposition granulomatous inflammation
96
``` nasal ulceration -> saddle nose deformity epistaxis pulmo hemorrhage hematuria, proteinuria, hypertension conjunctivitis, uveitis, optic neuritis ```
wegener granulomatosis
97
treatment of ANCA associated vasculitis
corticosteroids oral cyclophosphamide (if kidney) methotrexate (if pulmo only)
98
peak age of growing pains
4-8 yrs old
99
diffuse musculoskeletal pain in 3 different areas in 3 months
juvenile primary fibromyalgia syndrome
100
ongoing burning limb that is subsequent to an injury, immobilization
complex regional pain syndrome | or previously Reflex sympathetic dystrophy
101
key features of complex regional pain syndrome
allodynia hyperalgesia autonomic dysfxn (cyanosis, mottling, hyperhidrosis)
102
age of onset complex regional pain syndrome | predominance
9-15yr | females 6:1
103
treatment for complex regional pain syndrome
amytriptylline
104
episodic cartilage inflammation causing destruction
relapsing polychondritis
105
diagnosis psoriatic arthritis
``` arthritis and psoriasis or arthritis and AT LEAST TWO of following: (1) dactylitis, (2) nail pitting or onycholysis, (3) psoriasis in a 1st-degree relative ```
106
diagnosis of Juvenile Ankylosing Spondylitis
xray evidence of sacroiliitis (sacroiliitis of grade 2 or greater bilaterally or at least grade 3 unilaterally) PLUS ONE of FF: inflammatory back pain limitation of motion in the lumbar spine limitation of chest expansion
107
erythema nodosum, pyoderma gangrenosum, fever, weight loss, or anorexia in a child with chronic arthritis
inflammatory Bowel Disease
108
Sclerosis typically starts on the __ side
iliac
109
Squaring of the corners of the vertebral bodies and syndesmophyte formation
bamboo spine | Ankylosing Spondylitis
110
AA amyloidosis has also been associated with
granulomatous dis- eases such as sarcoidosis, cystic brosis, Crohn disease, malignancies such as mesothelioma and Hodgkin diseases, intravenous drug abuse, and other infections, such as bronchiectasis and HIV.
111
hereditary amyloidosis genes involved
transthyretin and apolipoprotein A
112
fevers, myalgias, arthralgias, urticarial-like rash, and progressive sensorineural hearing loss
Muckle-Wells syndrome
113
homogeneous eosinophilic material that stains with Congo red dye and demonstrates the pathognomonic “apple-green birefringence” in polarized light
amyloid bodies
114
___ is the underlying cause of death in 40-60% of patients with amyloidosis
End-stage renal failure

PPS - From Nelson's (24 decks)

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