RANDOM Flashcards
1
Q
Inflammatory myopathy often overlap with other CTDs typically
A
MCTD
SLE
Sjogren
SSc
2
Q
Predominant bacteria that cause infectious myopathy in children
A
Staph
Strep
3
Q
MC implicated viral agent of viral myositis
A
Influenza B
4
Q
viral myositis: onset of symptoms and resolution
A
3 days after onset of fever and respi symptoms; up to 1 month
5
Q
Gene affected in muscular dystrophies
A
Dystrophin gene
6
Q
MC age of muscular dystrophies
A
2-3y
7
Q
Pseudohypertrophy of calves is seen in
A
muscular dystrophies
8
Q
Painless prox muscle weakness is seen in
A
Hyperthyroid myopathy
9
Q
3 MC malig associated with IIM
A
Leukemia
CNS tumors
Lymphoma
10
Q
Classic histopath of skin lesions in JDM
A
Vacuolar interface dermatitis with dermal mucin deposition
11
Q
Mainstay of tx of JDM
A
High dose CS + steroid-sparing agents
12
Q
Most widely used treatment for JDM
A
CS
13
Q
Most widely used steroid-sparing agent for JDM
A
MTX
14
Q
MTX dose/route for JDM
A
15-20mg/bsa/wk, SC
15
Q
Treatment for steroid resistant JDM
A
IVIg
16
Q
Options for JDM with persistent skin rash despite resolution of muscle disease (2)
A
IVIg, MMF
17
Q
Option for JDM intolerant to MTX
A
Cyclosporine, 3-5mkday
18
Q
Option for severe or refractory JDM
A
CYC
19
Q
Option for JDM with skin-predominant disease without significant muscle involvement
A
HCQ 5mkday max 400/day
20
Q
T/F JDM patients should be encouraged to exercise even with active disease
A
T
21
Q
Cure for JDM lipodistrophy
A
None
22
Q
JDM: Reliable test to monitor disease activity
A
None
23
Q
Muscle strength tests for JDM
A
MMT
CMAS
MMT8
24
Q
Frequency of disease damage assessment in JDM
A
At least annual
25
Course of JDM
1/3 monocyclic
3-30% polycyclic
30-60% chronic
26
Predicts chronic course in JDM
Higher disease activity score at diagnosis
27
Skin vs MSK: More rapid improvement
MSK
28
JDM Predictors of longer time to remission
Rash 3 months after dx
Abn nailfold capillary and rash at 6m
29
Causes of death in JDM
GI, pulmonary, unresponsive myositis, CV, infection
30
MC damaged organ system in JDM
Skin
31
HRQoL domain that is most impaired in JDM
Physical well-being
32
Median onset of lipodystrophy in JDM
4y after dx
33
T/F skin biopsy of locaclized and systemic sclerosis are indistinguishable
T
34
MC affected organs in JSSc
Vascular
Cutaneous
GI
Pulmo
MSK
35
JSSc gender predilection
F
36
A diagnosis of JSSc should be suspected in the ff
1) Very young child with RP
2) Older child with moderate to severe RP with associated nailfold cap abnormalities, digital ulcers, and Ab profile of SSc
37
2007 Classification criteria for JSSc
Major (Required): Skin induration/thickening proximal to the MCP or MTP joints
Minor (2 required): Scleroderma-specific organ involvement
*Age of onset must be <16y
Page 381, Table 27.1
38
ACR/EULAR classification criteria for SSc (Adult)
Page 381, Table 27.2
Definite SSc: Total score of ≥9
39
ACR/EULAR classification criterion for SSc (Adult) with automatic score of 9
Skin thickening of both hands extending proximal to the MCPs
40
Clinical subtypes of SSc
Diffuse cutaneous
Limited cutaneous
Overlap syndrome
41
Antibody associations: Diffuse cutaneous SSc
Anti Scl-70
42
Antibody associations: Limited cutaneous SSc
Anti centromere
43
Antibody associations: Overlap syndrome SSc
Anti PM-Scl
44
Clinical subtype of SSc: Rapidly progressive skin thickening
Diffuse
45
Clinical subtypes of SSc: Early visceral disease
Diffuse
46
Clinical subtypes of SSc: Restricted, nonprogressive skin thickening
Limited
47
Clinical subtypes of SSc: Late visceral disease
Limited
48
Clinical subtypes of SSc: CREST syndrome
Limited
49
CREST syndrome
Calcinosis, RP, esophageal dysmotility, sclerodactyly, telangiectasia
50
Clinical subtypes of SSc: Features of SSc + another CTD
Overlap
51
Commonly overlaps with JSSc
JDM
52
Adult vs Juvenile SSc: Overlap
JSSc
53
MC clinical feature of SSc
RP
54
Adult vs Juvenile SSc: Better survival rates
Juvenile
55
3 phases of Scleroderma skin lesions
Edematous
Sclerosis (or induration)
Atrophy
56
Predilection of JSSc edematous phase
Distal extremities
57
Onset of sclerosis phase of JSSc
Several weeks to months after edematous phase
58
Fibrotic phase of JSSc is paticularly noticeable where
Dorsum of fingers and hands (acrosclerosis)
59
TYpical scleroderma facies due to sclerotic skin changes
Pinched nose
Thin pursed lips
Small mouth with diminished aperture
Prominent teeth
Expressionless appearance
60
mRSS scores how many areas of the body
17
61
mRSS scores areas of the body as
0-3, none, mild, mod, severe
62
Calculated from mRSS and assist in predicting internal organ involvement in those with dcSSc
Skin thickness progression rate (STPR)
63
Telangiectasia of SSc is most prominent in these areas
Face and upper ext
64
Color changes of RP
pallor-cyanosis-erythema
65
Findings in NFC of SSc
Dilated (>2x normal width) and tortuous THEN dropout (absence of NFC) and arborization of capillaries
66
Normal finger-to-palm measurement
0 (able to touch fingertip to palmar crease)
67
FTP is a component of
Medsger severity scale of SSc
68
Typical myopathy in SSc affects what muscle group
Proximal and typically symmetrical
69
How to measure FTP
Place a pen marking at the distal palmar crease at the base of the middle finger; ask patient to fully flex the middle finger; measure distance from distal palmar crease to tip of patient's finger in full flexion
70
Children with dcSSC and this organ system involvement are at particular risk for severe cardiac involvement
Myositis
71
T/F GI manifestation may precede skin involvement in JSSc
T, in 42-74%
72
T/F SSc typically progreses through the GIT from top to bottom
T
73
PFTs in JSSc that are particularly concerning
Decreased FVC
Decreased DLCO
74
Major cause of Scleroderma related mortality in children with SSc
Cardiac involvement
75
Major cause of Scleroderma related mortality in adults with SSc
Lung involvement
76
PAthognomonic pathological finding in the heart of patients with SSc
Mosaic patchy distribution of myocardial fibrosis
77
Recommended baseline cardiac exams for JSSc
ECG and echo
78
Mild renal dysfxn commonly occurs in JSSC due to
Vasculopathy rather than GN
79
ADult vs juvenile SSc: SCleroderma renal crisis
Adult
80
Medical emergency in SSc characterized by accelerated arterial htn, renal insufficiency, microangiopathic hemolytic anemia, and thrombocytopenia
SRC
81
Baseline renal test recommended for SSC patients
UA
Spot UPCr
BP
82
T/F Neuro involvement is rare in SSc
T
83
Subtype of localized scleroderma that is most likely to resemble JSSc
Pansclerotic morphea, most sever type of LS
84
Important diff between JSSc and pansclerotic morphea
1) In pansclerotic morphea, although fingers and hands may be swollen from venous congestion from surrounding sclerosis, thickness of the skin and skin changes are rarely found distal to the MCPs and PIPs. In contrast, JSSc usually starts with skin changes and thickness of the distal ext and proceeds proximally.
2) RP, NFC changes, and digital ulcerations are not significant in pansclerotic morphea
85
Characteristic findings of the skin in eosiniphilic fasciitis
1) Peau d'orange
2) Groove sign due to deep facial involvement
86
Treatment for scleroderma renal crisis
ACEis (catopril or enalapril) given immediately
87
PRevention of SRC
Immediate lowering of BP in SSc and malignant htn
Avoid any sudden changes in plasma volume, particularly marked reductions
88
Organ specific treatment for Systemic Sclerosis
Table 27.8, Page 395
89
SSc, first-line treatment: RP
CCB (nifedipine, amlodipine)
90
SSc, first-line treatment: Digital ulcers
PDE-5 inhibitors (sildenafil, tadalafil)
91
SSc, first-line treatment: Pulmonary htn
PDE-5 inhibitors, endothelin receptor antagonists
92
SSc, first-line treatment: ILD
CYC, MMF, CS
93
SSc, first-line treatment: Skin
MTX, MMF, CS
94
SSc, first-line treatment: MSK
NSAID, HCQ, MTX, CS
95
SSc, treatment for very severe disease: RP
Prostacyclins (iloprost)
Sympathectomy (botulinum toxin)
Fat grafting
96
SSc, treatment for very severe disease: Digital ulcers
Prostacyclins
97
SSc, treatment for very severe disease: ILD
Hematopoietic stem cell transplantation
98
SSc, treatment for very severe disease: Skin
CYC, rituximab
99
SSc, treatment of GI manifestations: Tooth decay
Fluoride tx every 3 months
100
SSc, treatment of GI manifestations: Dysphagia
Soft foods, small portions, adeq fluids, low sugar, gingival mucosa grafting
101
SSc, treatment of GI manifestations: Esophageal dysmotility, reflux, constriction
Metoclopramide, erythromycin 3-5mkday
Wt loss
Small freq meals
Avoid eating 3h prior to bed
Raise had of bed 6 inches
Avoid tight clothes, heavy lifting, bending
PPI, H2 blockers
102
SSc, treatment of GI manifestations: Watermelon stomach (GAVE), fructose intolerance
Metoclopramide, erythromycin, ocreotide
103
SSc, treatment of GI manifestations: Small int bacterial overgrowth
FODMAP diet
104
Adult vs Juvenile SSc: More favorable prognosis
Juvenile
105
MC cause of death
Cardiac (including PAH) and respi failure
