Rheumatology Flashcards
(287 cards)
1
Q
Rheumatoid Arthritis is
A
A systemic auto-immune disease that manifests in the joints
Uncontrolled proliferation of synovial fluid
Synovitis, symmetrical, small joint
2
Q
Genetics of RA
A
Shared epitope confers genetic risk
HLA-DRB1*04
Strongly associated with ACPA (+)
3
Q
RA pathogenesis
A
ACPA
Anti-cutrullinated protein antibodies
Associated with RA
Anti-CCP
4
Q
Why people get RA
A
Genetic susceptability
Insult/trigger
5
Q
Criteria for RA
A
Morning stiffness
Arthritis in 3 joint areas
Arthritis in hand
Symmetrical arthritis
Rheumatoid nodules
Serum rheumatoid factors
Radiographic changes
must be present for more than 6 weeks
6
Q
RA is
A
Small joint
Synovitis
Symmetrical
7
Q
Small joint
Synovitis
Symmetric
A
Rheumatoid arthritis
8
Q
RA deformity
A
Boutoniere Deformity
Swan neck
Ulnar subluxation
9
Q
RA labs
A
Markers of inflammation
Anemia of chronic disease on labs
Thrombocytosis
Decreased albumin
***CCP
10
Q
Rheumatoid factor
A
Autoantibody against Fc portion of IgG (IgM, IgG, IgA)
Higher titer= worse disease
Indolent infections (endocarditis, Hepatitis B and C virus)
B cell lymphomas
Mixed cryoglobulinemia (Hepatitis C virus)
11
Q
RA X ray erosions
A
Located at articular surface
12
Q
RA joint space
A
Symmetric joint space narrowing
13
Q
C1-C2 subluxation
A
Significant risk for RA patients
Associated with RF and ACPA
14
Q
RA nodules associated with
A
high titer RF
15
Q
Other organ systems involved with RA
A
Caused by systemic inflammation
Lungs: nodules
Heart: pericardial effusions
RA: corneal melt
Skin: pyoderma gangrenosum
16
Q
RA treatment
A
Many different treatment options
NSAIDS (bandaid, should not be used as monotherapy)
DMARDS
Early diagnosis and treatment to target is the GOAL
Use combinations to achieve disease remission when needed (synergistic at lower doses and result in less toxicity)
17
Q
Methotrexate
A
ANCHOR drug for RA
Dose ONCE WEEKLY to avoid liver toxicity
Contraindicated in Renal Disease (kills bone marrow and will kill patient)
18
Q
What must you screen for before giving a biologic
A
Tuberculosis and Hepatitis B
19
Q
Triple therapy for RA
A
MTX + SSZ + HCQ
20
Q
Raynaud’s Phenomenon (RP)
A
Exaggerated vascular response to cold temperature, emothional stree, vibration
tri-phasic color change with clear demarcation (white, blue, red)
Primary: younger, symmetric
Secondary: older, assymetric (may develop ulcers and secondary necrosis- autoamputation)
21
Q
Raynaud’s phenomenon treatment
A
core body warming
22
Q
Sjogrens Syndrome
A
systemic autoimmune disease associated with lymphocytic infiltration of exocrine glands
can be primary or secondary
23
Q
Sjogrens Syndrome clinical presentation
A
Xerophthalmia (dry eyes)
Xerostomia (dry mouth)
Raynaunds phenomenon
vasculitis
Non-errosive inflammatory arthritis
Interstitial Lung disease
Type I renal tubular acidosis
Marked increase in risk on non-Hodgkin’s Lymphoma (heralded by decline in RF titer)
CT Scan of splenomegaly with lymphoma in SjS
24
Q
Sjogrens Syndrome Serology
A
ANA
Anti-SSA/SSB (Ro/La)
Rheumatoid factor
25
Sjogrens Syndrome
Shirmer’s test: test for dry eye
Salivary Gland biopsy
26
Systemic Lupus Erythematosus
Prototypic immune complex deposition disease (low self tolerance)
Universally ANA Positive
27
For SLE you need how many criteria
4/11 positive
28
Photosensitive malar rash
Lupus
29
Lupus
Oral ulcers
photosensitivity
Lupus rash
alopecia
Discoud lupus
subacute cutaneous lupus
Jaccoud’s Arthropathy: non erosive inflammatory arthritis
pericarditis and valvular lesions
pleural effusions
pneumonitis (diffuse alveolar)
shrinking lung syndrome
30
SLE nephritis
look for active urinary sefiment on the urinalysis (blood and protein)
anti-dsDNA
low compliment
kidney biopsy: light miscroscapy, immunoflouresence, electron microscopy
31
SLE serology
Positive ANA
Suberologies:
anti-smith (most specific)
anti-dsDNA
renal disease
anti-phospholipid Abs
clotting risk
32
SLE Hematologic disease
lymphocytopenia
33
Drug induced lupus
Serology: ANA and anti-histone (in isolation)
Renal disease is very rare
34
Neonatal Lupus
autoimmune disease from passive transfer of autoantiboides from mother to fetus resulting fetal and neonatal disease
Anti-Ro (SSA) and Anti-La (SSB)
Rash and heart block
35
Anti-phospholipid antibody syndrome
pregnancy loss, vascular thrombosis, circulating anti-phospholipid antibodies
Antiphospholipid Abs include:
Lupus anticoagulent (clotting test with mix)
PTT or dRVVT (will be increased)
mix with normal serum
factor defiency (PTT corrects)
inhibitor (PTT won’t correct)
Anti-cardiolipin Ab
B2-Glycoprotein-I Ab
36
Clinical findings APS
DVT
recurrent fetal loss or other OB complications
livedo reticularis
Avascular necrosis
CAPS
37
Avascular necrosis
cellular death in bone due to the loss of blood supply
cause by glucocorticoid use
inflammatory/autoimmune disease
38
Inflammatory Myopathies
indifious, progressive and usually painless proximal muscle weakness
immune mediated myopathies
polymyositis (PM) and dermatomyositis (DM)
noted to coexist with cancer especially DM
39
Dermatomyositis
Heliotrope rash on eye lids
gottrons papules on knuckles
Rash can occur at any time
V sign or shawl sign around the neck
periungual erythema
mechanics hand
40
Clinical findings of PM and DM
dysphagia
dyspnea from interstitial lung disease (ILD)
Antisynthetase syndrome (subset of PM with rapidly progressive ILD and mechanics hands)
Anti-Jo-1
mechanics hands
41
PM and DM labratories
Elevated markers of muscle damage: CPK, aldolase, LDH
LTFs may also be up (AST and ALT are released by muscle)
Anti-Jo-1 is an antisynthetase Ab
42
DM and PM diagnosis
MRI or MRI STIR
Muscle biopsy
cancer testing
43
left-Polymyositis: perifascular inflammation
right- dermatomyositis: perivascular inflammation
44
Progressive Systemic Sclerosis (Scleroderma or PSS)
multisystem disease resulting in fibrosis of the skin, internal organs, and other connective tissues
Abnormal pathways:
vasculopathy
Inflammation: cell mediated and humoral
Fibrosis: fibroblast undergo permanent changes
45
Scleroderma clinical findings
RP, GERD, arthralgia/myalgias
puffy painful skin
sclerodactyly
telangiectasia
end stage leads to contraction and ulcers
RP is nearly universal
fibrosis or pulmonary hypertension
Gastrointestional: reflux, GAVE leading to bleeding
malignant hypertension
Scleroderma renal crisis which is treated with ACE inhibiots (life saving)
onion skin appearancw of renal blood vessels
46
Scleroderma diagnosis
positive ANA
Diffuse:
Centromere: CREST syndrome
Limited:
Scl-70: systemic sclerosis (scleroderma or PSS)
RNA Polymerase III (anti-POL III): rapidly progressive scleroderma
47
Spondyloarthritis (SpA)
Ankylosing spondylitis (AS)
Reactive Arthritis (ReA)- formerly Reiter’s syndrome
Psoriatic arthritis (PsA)
Arthritis associated with inflammatory bowel disease
48
SpA has a strong association with
HLA-B27
49
SpA: etiology and Pathogenesis
higher prevelance in males
50
Reactive arthritis is tiggered by
Salmonella, Shigella, Campylobacter, Yersinia, chlamydia
51
SpA common features
inflammatory low back pain
insidious onset
duration greater than 3 months
significant morning stiffness and improvement with exercise
SIJ involvement
Associated findings: Ocular (Uveitis), Dactylitis (sasuage digit), Enthesopathy
52
Dactylitis
flexor tenosynovitis
SpA common feature
53
Ankylosing Spondylitis
spinal fusion
SIJ inflammation and fusion
Inflammatory arthritis (large joint below the belt)
Head to wall test, bamboo spine
54
SpA: reactive arthritis
Classic Triad: Conjunctivitis, urethritis, and arthritis (cant see, pee, or climb a tree)
asymmetric arthritis
enthesopathy and achilles tendonitis (lovers heel)
circinate balanitis/keratoderma blennorrhagica (rash)
oral ulcers
55
Psoriatic Arthritis characteristics
enthesopathy
dactylitis
exuberant syndesmophytes
nail pitting and onycholysis
DIP joint affected
pseudorheumatoid
oligoarticular
arthritis mutilans (pencil in cup erosions
oil spots and nail pitting
56
IBD arthritis
sacroiliitis
enthesitis
uveitis
stomatitis
pyoderma gangrenosum
erythema nodosum
panniculitis on LLimb
57
Vasculitis
inflammation in blood vessels
always try to biopsy for diagnosis
biopsy tissues fed by artery when vessel can’t be biopsied
58
Giant cell (temporal) arteritis
headaches, vision loss, jaw claudication, constitutional symptoms (fever, weight loss, malaise)
Lab: Elevated ESR/CRP
Diagnosis: biopsy temporal artery
ultrasound arteries (non-compressible)
59
Anti-neutrophil cytoplasmic antibodies (ANCA)
C=cytoplasmic
P=perinuclear
C3PO
C-ANCA PR3
P-ANCA MPO
small to meduim sized blood vessels
GPAl MPA, EGPA
pulmonary renal involvement
must biopsy for disgnosis (lung is ideal)
60
GPA
sinusitis
tracheitis
ocular involvement
C-ANCA (PR3)
61
MPA
P-ANCA (MPO)
62
EGPA
Asthma
Eosinophilia
P-ANCA (MPO)
63
Mixed cryoglobulinemia
Cyroglobulins (IgGs that precipitate below 37 degrees and dissolve upon rewarming
HCV
B cell cancers
64
Polyarteritis Nodosa (PAN)
systemic vasculitis affecting medium sized muscular ateries
can be triggered by Hepatitis B
65
Takayasu’s Arteritis (TA)
systemic vasculitis of the aorta and its branches
extremity claudication “pulseless disease”
66
Rheumatoid factor
RA
SjS
HCV
Cryo
B-cell malignancy
67
Anti-Pol III
scleroderma
68
Anti-centromere
CREST
69
C-ANCA (PR3)
GPA
70
HLA B27
Seronegative SpA
71
Anti-CCP
RA
72
Anti-Smith
SLE
73
LAC
APS
74
P-ANCA (MPO)
MPA
EGPA
75
Anti-Scl 70
Scleroderma
76
Anti-DS DNA
SLE nephritis
77
Anti-histone (in isolation)
Drug induced lupus
78
In inflammatory arthritis joints are
Warm, swollen, erythematous
AM stiffness
Gelling phenomenon (pain better with activity)
79
Non-inflammatory arthritis joints are
Cool
Bony
Pain is worse with activity and better with rest
80
Key questions to determine if arthritis is inflammatory or non-inflammatory
What time of day is the pain the worst?
Is your pain better or worse with activity?
81
Arthritis lab evaluation
Urinalysis to look for blood or protein
Inflammatory marjers
82
Markers of inflammation
IL-6
Albumin should decrease
83
Westergren Sedimentation rate (ESR)
Blood placed in verticle tube and measure distance RBC fall over an hour
-fibrinogen
-immunoglobulin
84
Rheumatic factor
Autoantibody against Fc portion of IgG
Prognostic of RA
85
Anti-CCP antibody
High specificity for RA
86
Anti-Nuclear antibody
NOT A SCREENING TEST
Flourescent ANA test is the gold standary
87
SLE
dsDNA= renal disease
Smith= specific for SLE
Ro/SSAand La/SSB= cutaneous SLE and neonatal SLE
histone alone= drug induced SLE
88
Scleroderma
centromere= CREST
Scl-70 (topo-isomerase) & RNA pol III
89
Myositis
Jo-1 (tRNA synthetase)
90
Myositis
Jo-1 (tRNA synthetase)
91
Sjogren’s Syndrome
Ro/SSA & La/SSB
92
Anti-Phospholipid Ab Syndrome
lupus anti-coagulant
anti-cardiolipin
93
ANCA (anti-nuclear cytoplasmic antibody)
antibodies directed to cytoplasmic enzymes
P-ANCA and myeloperoxidase (AGPA and MPA)
C-ANCA and serine protease 3 (GPA)
94
Arthrocentesis
fluid obtained and sent for gram stain, cell count, crystal analysis, PCR
low risk of infection
95
Normal synovial fluid is
see through
96
MSU
97
CPPD
98
RA radiologic findings
Marginal (joint line) erosions
99
OA radio graphic findings
Osteophytes
100
EOA radio graphic findings
Gull-wing central erosions
101
Gout radiographic findings
Juxta-articular erosions with sclerotic and over-hanging edges
102
Pseudogout radiographic findings
Punctuate or linear densities in fibro or hyaline cartilage
Hooking on radial side of MCP
103
SLE radiographic findings
Jaccoud’s arthropathy
104
Psoriatic Arthritis
Pencil in a cup body erosion
105
Ankylosing spondylitis radiographic findings
symmetric sacroiliitis (iliac side, distal 1/3 of joint)= fusion
Syndesmophytes
106
RA vs OA
RA affects any joint other than DIP
OA affects hips, knees, spinal, all hand joints, and the MTP joint of big toe
107
Acute Monoarticular arthritis
Septic or crystalline arthritis
108
Signs and symptoms of septic arthritis
Fever
Rigor
Heart murmur
Cellulitis
Instrumentation
Guarding ROM
109
Syngs and Symptoms of crystalline Arthritis
Tophi
110
Labs and imaging for acute monoarticular arthritis
CBC
ESR, CRP
Blood, urine cultures
Serum creatine and urin acid
Imagining (yield is generally disappointing)
111
What is central to the evaluation of an inflamed joint
Synovial fluid analysis
Cell counts with diff, gram stain, culture, crystal analysis
112
Synovial fluid WBC
Normal <200 WBC <25% PMN
Non-inflammatory <2000 WBC <25% PMN
Inflammatory 1,000 to 75K WBC >50% PMN
Septic 50K to 100K WBC >85% PMN
113
Light microscopy
WBC
fibrillation materials= rice bodies
Phagocytized WBCs
Crystals
114
Yellow parallel crystal
Gout
mono sodium rate mono hydrate
Negative birefringence
115
Blue arrow
CPPD
Positive birefringence
116
Gout
M-males, monosodium urate crystals, monoarticular
E-episodic
N-negative birefringent
117
Uris acid is
End produce of purine degradation
118
Hyperuricemia
Serum urate concentration in excess or urate solubility
Gout= deposition of monosodium urate crystals in tissues
119
_ catalyzes the final conversions to urine acid
Can think oxidases
120
What is the most common inflammatory arthritis
Gout
121
Gout diagnosis
Joint tapping
A clinical diagnosis can be made without joint tapping based on typical features and no evidence of alternative diagnosis
Uris acid level (normal 1/3 of time)
X-rays are rarely helpful
122
Gout flare
Occurs in bursa, tendon, joints
First MTP joint most common initial site
rapid onset and escalation
123
Gout flare treatments
Colchicine
Prednisone NSAIDS
124
Characteristics of Advanced gout
Tophi
125
Advanced gout imaging
X ray: rat bite
Dual energy CT: detect uric acid deposition
Ultrasound of 1st MTP joint: double contour sign
126
Uric acid can be increased by
Diuretics: increased uric acid reabsorption
Low dose aspirin
Pyrazinamide, ethambutol, niacin
Cyclosporine and tacrolimus
127
Urate lowering drugs
DO NOT start during an acute attack
1st line: Xanthine Oxidase inhibitors
2nd line: Uricosuric drugs
Serum uric acid goal:<6 mg/dL
128
Caveats to Allopurinol (xanthine oxidase inhibitors)
Allopurinol hypersensitivity syndrome (HLA-5801 is a risk factor)
129
Peloticase
Uric are enxyme
For treatment failure gout
Highly immunogenicity
Increased rate of CV events
130
CPPD Deposition disease (Pseudogout)
131
Pseudogout (CPPD deposition disease)
High calcium and low phosphorus
Chondrocalcinosis (radiographic finding)
Most common affects wrists and knees
Risk factors: older age. hemochromatosis, hyperparathyroidism, hypothyroidism, hypomagnesemia
treatment: NSAIDS, steroids, colchicine
132
Disseminated gonorrhea infection
Low yield synovial fluid
Culture is critical
Treatment: antibiotics
133
Viral arthritis
Parvovirus: Fifth’s disease in children, arthritis and rash in adults
Increased IgM anti-Parvovirus
Can also happen with HIV, influenza, Hep B/C
134
Chikingunya virus
Transmitted through mosquito bite
Fever and arthralgia
1/3 have chronic rheumatic manifestations
elevated ESR
135
Tick borne illness (Borrelia burgdorferi)
Northern United States
Testing: ELISA and western blot
Treatment: antibiotics
136
Traveling joint pain and swelling following Strep infection
Maculopapular rash with central clearing
murmur
Acute rheumatic fever
carditis
Rash
Management: antibiotics
137
Jones criteria
for patients with preceding Group A strept infection
2 major or 1 major (carditis, arthritis, chorea, erythema marginatum, subcutaneous nodules) and 2 minor (arthralgia, fever, elevated ESR/CRP, prolonged PR interval)
138
There is an increase in _ in patients with autoimmune diseases
Levels of autoantibodies
Mostly IgM antibodies (aids in clearance, stops activation of phagocytes through DAMPs, and inhibits binding of antigens to sIgs resulting in no B cell responses
139
T cell help causes
Isotope switches
Memory
Increased concentration of antibodies
140
Usually the determinant of autoimmune disease is unknown because
Patients present to clinic years after symptoms start
Initiating agent is gone
141
Autoantibodies are good biomarkers that are helpful in diagnosing and monitoring disease (epiphenomenon)
ANA
Rheumatoid factor
ACPA
Other disease specific antibodies (ex. Pemphigus vulgaris)
Epiphenomenon: secondary effect or byproduct that arises from but does not causally influence a process
142
Sedimentation rate
High- indication of high levels of inflammation
Can be used to determine how well treatment is working
143
CRP
Acute phase protein
Made by liver in response to IL-6
CRP levels increase as a result of inflammation
144
Rheumatoid factor
Autoantibodies that bind to Fc portion of Ig
Enhances phagocytosis/opsonization
Immune complexes deposited in kidney
145
How are CRP and RF measured
Nephelometry (looks at light deflection)
Form immune complexes (cause light deflection)
Measures how fast complexes form and the amount present
146
Antinuclear antibodies
Not specific for any one disease but indicate autoimmune disease
Are epiphenomenon (not damaging)
147
ANAs are measured by
Indirect immunoflouresence assay (used for viral serology, rapid viral diagnosis, rental pathology, and the detection of antibodies)
148
Antibody detection is performed using an
Indirect ELISA
149
ANA is run on
Hep-2 cells to determine ANA positivity
Dilute serum and add to slide
Wash using fluorescently labeled anti-Human IgG
Wash off antibody
Last dilution of serum that gives a positive result is the dilution reported (is significant when greater that 1:160)
150
Rim patterm
anti-dsDNA (SLE)
151
Homogenous pattern
Anti-histone antibody
drug induced SLE
152
Speckled pattern
Anti-Sm and/or anti-
Ro/SSA and/or anti-
La/SSB and/or anti-
UIRNP
Sjögren’s syndrome
153
Nucleolar pattern
Anti-Th or anti-
fibrillarin/anti-U3RNP
or anti-U17RNP
May think of Scleroderma or polymocysitis
154
Centromere pattern
CREST/pulmonary hypertension
155
Anti-dsDNA
Used when SLE is suspected (test using Crithidia)
156
When looking at an ANA are the proteins abnormal
No, normal proteins inside the cell
157
CCP is diagnostic for
RA
158
Test immunomarkers using
ELISA
159
Yellow parallel
Gout
160
Blue crystals
Pseudogout
161
Primary bone tumors
Common before 30 years old
Most common around knee/humerus
162
Common tumors metastasizing to bone in adults
P T Barnum Loves Kids
Prostate
Thyroid
Breast
Lung
Kidney
163
Symptoms of bone tumors
Pain: deep, constant, worse at knight
Most benign tumors found incidentally
164
Trivial trauma causes
Pathological fracture
165
Evaluation of bone tumor
Plain X ray (first)
MRI for staging or surgery planning
Biopsy may not be necessary
166
Ultimate diagnosis of bone tumors requires
Radiologic histologic correlation
167
Benign primary bone tumors are often
Incidental
168
Osteochondroma
Most common benign bone tumor
Mushroom shaped
Start at epiphyseal line
Excision only if symptomatic
169
Osteoid osteoma
Hound patients
INTRAcortical tumor (diaphysis of long bones)
Sudden onset of pain (worse at night) that is very sensitive to NSAIDS (PGE2 release)
Conservative treatment (radio frequency ablasion)
170
Chondroma
Arises in childhood junction of diaphysis and metaphysics in long bones or short tubular bones of hand and foot
171
Fibrous dysplasia
Weaker bone susceptible to deformities or fractures
GNAS mutation
Proximal femur, jaw, and ribs
Ground glass appearance
McCune-Albright syndrome
Chinese letter trabrculae
Treatment: conservative
172
Non-ossifying fibroma
Common-most incidental
Seen in children
Error in remodeling metaphysical cortices in growth
Eccentric lesion in metaphysical cortex of tibial and fibula
Round or oval defects in sclerotic rim of metaphysis
Swirling of bland spindle cells with admired osteoclasts
Treatment: reassurance
173
Osteosarcoma
Tumor of osteoblasts
May be primary in kids or secondary in adults (caused by chemo)
Reported in most bones
In metaphysis
20% metastatic at presentation
Presentation: pain, soft tissue mass, pathological fracture
Aggressive radiodense lesion on x ray
Periosteal reaction: Codman’s triangle, Sunburst pattern (seen in rapidly growing lesions that can’t lay down new bone fast enough)
Atypical spindle cells
Diagnosis: XR, chemotherapy, neuadjuvant chemotherapy
174
Chondrosarcoma
Malignant tumor of chondrocytes
Stipples/popcorn appearance on X ray
Grade important for prognosis
Treatment: surgery
175
Ewing sarcoma/primitive neuroectodermal tumor
2nd or 3rd decade of lime
Any bone (Askin tumor ES/PNET involving rib)
More likely in diaphysis than other bone tumors
Pseudorosettes
CD99
Recurrent chromosomal translocation t(11;22)
Treatment: neoadjuvanct chemotherapy and surgery
176
Periosteal reaction
Bad sign suggestive of malignant bone tumor
Most commonly osteosarcoma
177
Benign soft tissue tumors
Outweigh malignant
178
Things causing soft tissue abnormalities
Abscess/folliculitis
Cysts
Hematomas
Fibrosis
Tumors
179
Connective tissue accounts for
70% of body mass
Tumors are rare
1,600 malignant tumors per year
5-10 benign for every malignant
Begin a spindle cell lesions
180
Divisions of soft tissue tumors
Benign: Do not reoccur after excision
Intermediate: high risk of recurrence or rare ability to metastasize
Malignant: substantial risk of metastasis
181
Malignant is different from epithelial tumors
Invasion not as important
Many are malignant by definition
Metastasis almost always a good indicator
182
Histologic markers of aggressive behavior
Mitotic activity
Nuclear pleomorphism
Necrosis
Infiltrative growth
183
CD31
Vascular
184
SMA
Smooth muscle
185
S100
Nerve/fat
Melanoma
186
Myogenin
Skeletal muscle
187
Keratin
Synovial sarcoma
188
_ is very important in the diagnosis of soft tissue tumors
Cytogenetics
189
What does tumor grade refer to
How dysplastic cells are
190
For malignant tumors, histologic grade
Is as important or more important than subtype (based on differentiation, mitoses, necrosis
191
-sarcoma
Malignant soft tissue tumor
Spreads hematogenously
VERY RARE for benign tumor to transform
Expansile growth with pseudocapsule (can look well encapsulated and still be very bad)
A/w prior radiotherapy
192
Soft tissue tumors are usually
Painless
193
Rumor, dollar, calor, fever
Abscess
194
Fluctuate/compressible
Cyst
195
Transluminates
Cyst
196
Direct history or trauma/anti coagulation with overlying ecchymosis
Hematoma
197
Key features of soft tissue neoplasms
Size
Depth
Mobility
Growth
198
<5 cm, mobile, superficial
Benign
199
>5 cm, fixed, deep
Higher risk of malignant
200
Growth may be a marker of
Malignancy
201
Soft tissue tumors are common components of
Tumor syndromes
neurofibromatosis, tuberous sclerosis, Li fraumeni, FAP
Key features: multiple tumors, strong family history, young age, skin manifestations
202
Lipoma
Most common benign soft tissue tumor
Made of mature adipocytes
Variants: angiolipoma, spindle cell/pleomorphic, intramuscular
203
Liposarcoma
Malignant tumor of adipocytes
Deep extremities are common site
Fatty tumor with admixed atypical cells with or without lip oblasts
Variants: well differentiated/atypical. Lipomatous tumor, myxoid/round cell, pleomorphic
204
WDLS/ALT
WDLS: complete excision not possible (retroperitoneum)
ALT: sites where wide excision is easy (extremity)
Genetics: MDM2 amplification (chromosome 12)
205
Myxoid/round cell liposarcoma
Chicken fire vasculature
T(12;16) FUS-DDIT3 fusion
206
Hemangioma
Benign vascular tumor
Appear around 1 month
3 phases: rapid growth, slow growth, involution
Treatment: none
30% resolve by 3 years
80-90% by 9 years
Laser therapy if impinging vital structure, ulceration, bleeding
207
Kaposi sarcoma
Vascular tumor of intermediate malignant potential
Endemic- only Mediterranean men, leg lesions
Immunodefiency associated
Any musosal surface, lymph node, or visceral organ
HHV-8 driven
Slit like vessels
208
Angiosarcoma
Malignant tumor of endothelium
Two types:
Cutaneous: sun exposed skin of elderly or irradiated skin
Long-standing lymphedema (Stewart-Treves syndrome)
Visceral
Pleomorphic endothelial cells with high mitotic rate
Poor prognosis
209
Neurofibroma
Benign peripheral nerve sheath tumor that arises from Schwann cells
Associated with Neurofibromatosis
Shredded carrots with buckled nuclei
S100-positive
210
Schwannoma
Benign peripheral nerve sheath tumor
Arise from Schwann cells
Associated with large nerve trunks
Most sporadic, seen in ND-2
S100-positive spindle cells
Antoni A (cellular), Antoni B (hypocellular) zones
Verrocay bodies
211
Malignant peripheral nerve sheath tumor
Bulky, deep seated
Strong association with NF-1 and prior radiation
212
Dermatofibroma
Benign fibrous histiocytoma
Thought to be associated with trauma
In women 20-50
Cellular dermal lesions surround adjacent thick collagen bundles
213
Dermatofibrosarcoma protuberans
Intermediate malignant potential
Older men
subcutaneous tissues of back and upper extremities
Cart wheeling CD34-positive spindle cells, infiltrate underlaying dat
Need wide local excision
t(17;22) PDGFR-COL1A1
214
Synovial sarcoma
Unknown histogenesis
Malignant soft tissue tumor affecting young adults
Arises in deep soft tissue around joint such as knee or ankle
monomorphic spindle cells
Biphasic- epithelial like structures
Monophonic- no epithelial like structures
t(x;18) SYT:SSX1 fusion
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High grade pleomorphic sarcoma
Malignant fibrous histocytoma
Most common type of malignant soft tissue tumor
Located in deep soft tissue/retroperitoneum of adults
High mitotic rate, marked pleomorphism
Negative for all IPX markers- no identifiable differentiation
Cytogenetically heterogeneous
Diagnosis of Exclusion
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Management of soft tissue lesions
Biopsy
MRI
Surgery:
benign- excise with negative margins
Intermediate- wide local excision (rim of normal tissue)
Malignant- neoadjuvant
Allows limb salvage
Done by specialists
Chemo only for some subtypes
Most people with sarcoma die of metastatic disease (found within 2-3 years of diagnosis in 80%)
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Skin Abscess
caused by S aureus
incision and drainage
sometimes antibiotics
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Diabetic foot ulcers
polymicrobial
adequate surgical debreadment and wound care is vey imporatant
Oral antibiotics for infection (culture guides therapy)
Duration of antibiotic therapy depends on osteomyelitis and debreadment
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Pyomyositis
pus within muscle groups
S. aureus
tropical pyomyositis (more common in temperate climates)
localized pain in muscle groups
surgical excision and drainage necessary
antibiotics depending on culture results
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Necrotizing fasciitis
Polymicrobial (mixed aerobic and anaerobic)- type I
Occurs commonly after surcial procedures in patients with diabetes and perephrial vascular disease
Monomicrobial- (group A streptococcus-S pyogens, S aureus)-Type II
can occur in any age group in patients without underlying medical conditions
Unexplained pain increasing rapidly overtime
Erythemia
Leukocytosis, elevated CK, elevated creatine
Woody feeling to subcutaneous tissues, blister, fever, hypotension and tachycardia, leukocytosis
Need blood cultures
Imaging not necessary unless questioning diagnosis
Surgical debridement
Blood cultures and emperic therapy
Adjunct therapy: hyperbaric oxygen therapy, IVIG for systemic infections to neutralize toxins
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Gas Gangrene
Clostritium
Severe penetrating wounds
Sever pain, gas in tissues, sepsis, treatment same as necrotizing fascitis
Water exposure: need to obtain history
Freshwater: vibrio vulnificus
Saltwater Aeromonas hyrophilia
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Infections following bites
Dog and cat: Pasteurella, S aureus, Bacteroides, Fusobacterium, Capnocytophaga
Human: Eikenella
Treatment: antibotic prophylaxis (oral or IV)
Sometimes surgical intervention is required
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Acute Bacterial Arthritis (Septic arthritis)
Typically Staph infection
Hematogenously aquired
Direct innoculation
Spread from adjacent tissues
Risk factors: immunosuppression, diabetes, malignancy, chronic renal failure, IV drug abuse, HIV, joint disease, sickle cell disease, surgery, penetrating injury, prosthetic joint
Monoarticular
Pain, loss of function, decreased ROM, fever
Focal joint tendernedd, inflammation, joint effusion
Arthrocentesis
Labs based on epidemiologic history
Radiology not necessary for diagnoisis
Needle aspiration, arthroscopic drainage, arthrotomy
Antibiotic therapy delayed until arthrocentesis
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Gonococcal arthritis
Disseminated N gonorrheae infection (sexually active people)
Dermatitis, tenosynovitis, migratory polyarthralgia or polyarthritis, fever, malaise
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Osteomyelitis
Spread from adjacent soft tissue, hematogenous, direct innoculation
S. Aureus, coagulase - staph
Pain at site of infection, fevers, chills, erythemia
Chronic condition evolving over months to years, non-specific pain, sinus tract
Fevers, erythema, swelling not common
Diagnosis: inflammatory markers, histopathology, radiologic studies
MRI is standard of care for diagnosis
Bone biopsy, needle aspiration, culture
surgical debridement and antimicrobial therapy
withhold antibiotics until cultures have been obtained (6-8 weeks)
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Vertebal Osteomyelitis, Spondylodiskitis, Epidural Abscess
infection of intervertebral disk and adjacent vertebrae
pain and tenderness in spine
S aureus, coagulase - staphylococci
MRI standard of care for diagnosis
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Prosthetic joint infections
significant mortality risk
Risks: males, prior surgery at same time, rheumatoid arthritis, immunosuppression, diabetes, cancer, poor nutritional status, obesity, smoking, chronic medical problems
Can be early onset (S aureus): aquired during implantation
Delayed onset (3-24 months): less virulent organisms (coagulase - Staph) aquired during implantation
Late onset (>24 months after surgery: hematogenous spread (dental source, pyogenic skin infections, GI and GU infections), line sepsis
Usually caused by Staph
Indolent: progressive pain, chronic draining sinus,
NO fever, swelling, systemic toxicity
Acute: fever, joint pain, swelling, erythemia
Lab: ESR, CRP, synovial fluid anaysis
Intraoperative cultures
X ray not needed
Debridement and implant retention
stage 1 revision: extract joint and immediately reimplant
stage 2 revision: extract joint and treat with antibiotics for a while before now implant
Perminent resection arthroplasy for non-ambulatory
Ambutation
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What do you need for healing a fracture?
adequate blood supply and adequate mechanical stability
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Fractures stimulate the release of
growth factors that promote angiogenesis and vasodilation
increases blood supply to fracture site
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Intramembranous ossification
direct bone formation
requires rigid fixation, opposition of bone fragments
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Endochondrial ossification
cartilage (soft callus) transitions to bone (hard callus) tolerant of appropriate motioin, fracture hematoma critical for healing
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What stimulates callus formation and mineralization
molecular and mechanical factors
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Wolff’s law
bone is restructured in response to stress and strain
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Adavced Trama Life Support (ATLAS) Trama Evaluation
A establish an AIRWAY
B Breathe for the patient if they arent
C assess and restore Circulation
D assess neurologic Disability
E EXPOSE entire patient
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Inspection
Look
Feel
Move
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X rays should be
orthogonal
incude joint above and below fracture
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Fractures are classified by
bone involved, fracture pattern, displacement,
angulation (defined by position of distal fragment relative to proximal fragment)
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Angulation
distal fragment relative to proximal
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Naming a fracture
bone
location
fracture pattern
displacement
angulation
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Emergent skeletal issues
Unstable pelvic fractures (hemmorage control)
open fractures (hemmorage control)
realigning
compartment syndrome
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What is especially important for open fractures
ANTIBIOTICS
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Compartment syndrome
increased pressure in fixed compartments squeezing contents (compress/damages vessels and nerves)
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Muscle survival
3-4 hours- reversible
8 hours- irreversible
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Nerve survival
2 hours- lose nerve conduction
4 hours- neurapraxia
8 hours- irreversible changes
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When is a compartment pressure measure used?
only in an obtunded patient
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Compartment syndrome
****pain out of proportion
rigid compartment with shiny skin
Pulselessness is not a characteristic of compartment syndrome
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treatment of compartment syndrome
fasciotomy
DO not close wounds
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degenerative disk diasease
cervical and lumbar spine
diagnois: physical exam and X ray
treated conservitavely (try to avoid narcotics
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cervical stenosis
narrowing of space avaliable for spinal cord
myelopathy and radiculopathy
exam and imaging
surgical and non surgical treatment
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Cervical Myelopathy
symptoms and phyical exam finding consistent with spinal cord compression
(balance problems, gait instability, hand dysfunction, neurogenic pain)
upper motor neuron findings: hyperreflexia, pathologic reflexes, coordination deficits
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Cervical Disc Herniation
presentation: radiculopathy (compression of nerve root)
unilateral arm symptoms
typically non-surgical treatment but sometimes surgical treatment is necessary
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Most common reason you see spin patients in clinic
Low back pain (most commonly due to degenative disc disease)
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Lumbar Degenerative Disc Disease
osteoarthritis of the lumbar spine
treated conservatively (try to avoid narcotics)
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Lumbar spine stenosis
narrowing of space avaliable for nerves commonly caused by arthritic changes
neurogenic claudication
conservative treatment
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Claudication
activity related leg pain that improves with rest
caused by perephrial vascular disease and lumbar spinal stenosis
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Neurogenic claudication (lumbar spinal stenosis)
pain bad when walking/standing
gets better with sitting/leaning forward
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Vascular claudication (periphreal vascular disease)
pain with walking that improves with standing
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Lumbar disk herniation
radiculopathy with or without paresthsia or weakness
conservative or surgical treatment
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Lumbar Spondylolisthesis
malalignment of the cephalad vertebrae in relation to the caudal vertebrae
presents as stenosis symptoms (neurologic claudication or radicular pain)
conservative or surgical treatment
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Common conditions of the thoracic spine
compression fractures
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Compresson fractures of thoracic spine
mid back pain after low energy trauma
symptoms of metabolic bone disease
conservative treament
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Number one reason people get joint replacements
Osteoarthritis
Must have pain, disability and functional limitations, failure of non-surgical treatment (must try conservative therapy before joint replacement
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When to use cementless vs cemented
cementless- younger age
cemented- femoral component in elderly
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Macrophages
eat up plastic particles from plastic wear causing bone lysis making it difficult to reconstruct
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Most common cause of revision after total knee replacement
Infection
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Subtypes of Cutaneous Lupus
Acute: butterfly rash
Subacute: photodistributed annular plaques
Chronic: discoid lupus erythematosus
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Differences between types of cutaneous lupus erthematosus rash
all have same histological features, but clinical presentation is different
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Lupus or Dermatomyositis
Vacuoles
Increased dermal mucin
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All people with _ have active systemic lupus erythematosus
Malar butterfly rash (acute cutaneous lupus erythematous)
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Lupus rash spares
Knuckles
Nasolabial fold
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Subacute cutaneous lupus
Photo distribution (annular)
Positive SS-A and SS-B
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Neonatal lupus
SS-A and SS-B antibodies
Annular rash on scalp and face
Congenital heart block, permanent
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Discoid lupus erythematous
Chronic
Scalp, face, and ears
Scarring with hyper-pigmented rim
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Dermatomyositis
photosensitive rash
Internal organ involvement
Histology (biopsy findings are identical to lupus)
Rely on clinical presentation
Diffuse scalp involvement, heliotrope rash, erythema in nasolabial folds, gottrons papules, gottrons sign on elbows and knees, V neck erythema, shawl sign, holster sign, periungual erythema, dialated capillaries
Mechanics hands (positive Jo-1 antibody)
Calcinosis
Ulceration (regions where gottrons papules would be present)
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Scleroderma categories
Diffuse- proximal to knees/elbows
Limited- distal to knees/elbows
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Sclerosis
Scarring
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Systemic sclerosis
Puffy hands
Sclerodactyly
Mask face, decreased oral aperture, radial perioral folds
Raynaud’s phenomenon, Digital ulcer, pitted scars, nail fold capillary changes, calcinosis (around hands)
Telangiectasia (matted or squared off)
Pigment changes (leukoderma of scleroderma, salt and pepper sign)
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Morphea
Localized scleroderma of the skin
Circumscribed, generalized, or linear variant (limb length variant)
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Systemic sclerosis vs Morphea
Head and neck and symmetric distal extremities
More than 90% have raynaud’s
Lacks distal involvement, no raynaud’s, no capillary changes, no systemic involvement, no autoantibodies
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Small Vessel Vasculitis
Purpuric papules on dependent areas or in areas of pressure or trauma
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Vasculitis diagnosis
Biopsy important
H&E
Direct immunoflouresence
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Hemlock Schonlein Purpura
IgA Vasculitis
Gastrointestinal
Joint
Renal involvement (IgA nephropathy)
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Granulomatosis with polyangiitis
C-ANCA (PR3)
Affects mixed medium and small vessels
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Microscopic polygangiitis
Eosinophilic granulomatosis with polyangiitis
P-ANCA (MPO)
Mixed small and medium vessels affected
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Medium vessel vasculitis
Livedo rash
Digital necrosis
SubQ nodules
Acrocyanosis
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Erythema nodosum
Panniculitis
Erythematous, painful nodules
Associated with streptococcal infection
Sarcoidosis
IBD
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Pyoderma gangrenosum
Pathergy
Ulcerations begin as violaceous or gray with undetermined borders
Criss-cross pattern (cribiform scarring)
Can be associated with disease about 50% of the time
