MuscSkCT - FA Patho p451 - 464 Flashcards
(132 cards)
1
Q
explain why estrogen is bone protective
A
estrogen blocks apoptosis in bone forming osteoblast and induces apoptosis in bone-resorbing osteoclast
2
Q
what bone cell secretes acid and collagenases?
A
osteoclast
3
Q
what cell is multinucleated and is derived from monocyte?
A
osteoclast
4
Q
what bone path takes place in epiphysis?
A
giant cell tumors
5
Q
what bone paths take place in metaphysis?
A
osteosarcoma (often around the knee), osteochondroma
6
Q
what bone paths take place in diaphysis?
A
Ewing sarcoma, myeloma, osteoid osteoma, fibrous dysplasia
7
Q
which bone path is associated with nighttime pain, central nidus, and pain released by aspirin?
A
osteoid osteoma
8
Q
3 characteristics of Ewing sarcoma?
A
- anaplastic small blue cell malignant tumor 2. extremely aggressive with early metastases, but responsive to chemotherapy 3. “onion skin” periosteal reaction in bone
9
Q
what population is most affected by Ewing sarcoma?
A
boys < 15 years old
10
Q
what is the 2nd most common primary malignant tumor after multiple myeloma?
A
osteosarcoma (osteogenic sarcoma)
11
Q
name 5 predisposing factors for osteosarcoma (osteogenic sarcoma)
A
- paget dz of bone 2. bone infarcts 3. radiation 4. familial retinoblastoma 5. Li Fraumeni syndrome (germline p53 mutation)
12
Q
what is the age distribution on osteosarcoma?
A
biomodal distribution (10-20 yr old and more than 65)
13
Q
what other malignancy has also bimodal distribution?
A
hodgkin (young adulthood and more than 55)
14
Q
on x-ray, how will osteosarcoma look like?
A
codman triangle (from elevation of periosteum) or sunburst pattern on x-ray
15
Q
what is the most common benign tumor?
A
osteochondroma
16
Q
does osteochondroma transform to chondrosarcoma?
A
rarely yes
17
Q
what is the characteristic of osteochondroma?
A
mature bone with cartilagionus (chondroid) cap
18
Q
what bone tumor is characterized as soap bubble appearance on x-ray with multinucleated giant cells
A
giant cell tumor
19
Q
what is the most common site of giant cell tumor?
A
epiphyseal end of long bone around knee (distal femur, prox tibia) - only 1 in this age group (20-40 yrs)
20
Q
in paget dz of bone, hearing loss is due to
A
auditory foramen narrowing
21
Q
name the 4 stages of paget dz of bone
A
- lytic (osteoclast) 2. mixed (osteoclast + osteoblast) 3. sclerotic (osteoblast) 4. quiescent (minimal osteoclast/osteoblast)
22
Q
explain the pathophysio of the paget dz of bone
A
common, localized disorder of bone remodeling cause by inc in both osteoblastic and osteoclastic activity
23
Q
what bone path is associated with mosaic pattern of woven and lamellar bone?
A
paget dz of bone
24
Q
what is the most common site of osteonecrosis (avascular necrosis) and why?
A
femoral head (due to insufficiency of medial circumflex femoral artery)
25
name 7 causes for osteonecrosis
ASEPTIC 1. **a**lcoholism 2. **s**ickle cell 3. **s**torage dz (Guacher) 4. **e**xogenous steroid 5. Legg - Calve -**P**erthes 6. **t**rauma 7. **i**diopathic 8. **c**aisson (the bend)
26
what is another name for idiopathic cause of osteonecrosis?
Legg Calve Perthes disease
27
what is the pathophysio of achondroplasia?
(gain of function), constitutive activation of fibroblast growth factor receptor (FGFR3) inhibiting chondrocyte proliferation
28
mode of inheritance of achondroplasia?
autosomal dominant with full penetrance (homozygosity is lethal)
29
how is Laron syndrome diff from achondroplasia?
small genitalia, dec linear growth, everything small due to defective growth hormone receptor
30
treatment drugs for type 2 (senile) osteoporosis?
bisphosphonate, PTH analog (teriparatide), SERMs, calcitonin, denosumab
31
what is denosumab?
monoclonal antibody against RANKL
32
what bone path is associated with bone filling marrow space leading to pancytopenia and extramedullary hematopoiesis?
osteopetrosis (marble bone dz)
33
what is defective in osteopetrosis?
failure of normal bone resorption due to defective osteoclast leading to thickened, dense bones that are prone to fracture
34
what mutation is responsible for osteopetrosis?
mutations in carbonic anhydrase II impairing the ability of osteoclast to generate acidic environment necessary for bone resorption
35
what renal path will also have impaired carbonic anhydrase?
proximal (type2) urine pH
36
what is the treatment for osteopetrosis?
bone marrow transplant is curative as osteoclast is from monocytes
37
what path is associated with "bone-in-bone" appearance on x-ray?
osteopetrosis
38
what bone cell secretes acid and collagenases?
osteoclast
39
what cell is multinucleated and is derived from monocyte?
osteoclast
40
what is the diff btw osteoporosis and osteomalacia in terms of bone mineralization?
normal bone mineralization in osteoporosis abnormal bone minerailization in osteomalacia (rickets)
41
4 Rx causes for primary osteoporosis?
1. long term steroid use 2. anticonvulsant 3. anticoagulants 4. thyroid replacement therapy
42
what symptoms/fractures can you get from osteoporosis?
Most commonly fractures from falls with fragile bones - hip, vertebra.
1. vertebral compression fractures associated with acute back pain, loss of height, kyphosis 2. fractures of femoral neck 3. fractures of distal radius (Colles fractures)
43
2 bone paths with normal lab values?
1. osteoporosis 2. osteopetrosis
44
what two bone paths will have the opposite lab values (Ca2+, PO43-, vs PTH/ALP?
osteomalacia/rickets and hypervitaminosis Osteomalacia - dec Ca2+, PO4, inc ALP,PTH Hypervitaminosis - inc Ca2+, PO4, dec ALP, PTH
45
2 causes for hypervitaminosis?
1. oversupplementation 2. granulomatous dz (sarcoidosis)
46
explain how the lab values will be diff btw 1' hyperparathyroidism vs 2'
1': high PTH --\> high Ca2+ --\> low PO43-, high ALP 2': high PO43- --\> low Ca2+ --\> high PTH, high ALP
47
In 2' hyperparathyroidism, what causes high PO43- conc?
usually due to dec PO43- from ESRD
48
in 2' hyperparathyroidism, what causes low serum Ca2+?
due to low activated vit D due to ESRD
49
MCP spared a. osteoarthris b. RA
a
50
RA which joint spared
DIP
51
pain improved with rest OA or RA
OA
52
TNF alpha inhibitors for RA List 3
etanercept (Enbrel), adalimumab (Humira), infliximab (Remicade), and certolizumab pegol (Cimzia)
53
what type of glucocorticoids would you give to osteoarthritis?
intra-articular
54
list two DMARDS for RA
Methotrexate Sulfasalzine hydroxychloroquine, leflunomide
55
most common fractures in osteoporosis type I?
femoral neck fracture, distal radius fracture
56
marker of osteoclast activity?
urinary OH-proline - breakdown product of collagen
57
Complications of Paget's disease
inc blood flow from AV shunts --\> high output heart failure and inc risk of ostogenic sarcoma
58
Paget's assoc w. with what childhood infection?
Paramyxo virus
59
Which marker is abnormal in Paget's
only an inc in ALP
60
Ewing sarcoma derived from what type of cells?
neuroectoderm poorly differentiated cells
61
RA association which HLA
HLA-DR4
62
what type of hypersensitivity is RA
type III IV
63
Heberden nodes in OA
He dips DIP
64
Bouchard nodes in OA
PIP and CMC
65
Pathology of rheumatoid nodules
fibrinoid necrosis with palisading histiocytes
66
most Specific marker for RA
anti-cyclic citrullinated peptide antibody
67
what is RF
IgM antibody against Fc portion of IgG
68
antibodies of sjogren
SSA SSB
69
what are SS-A and SS-B
ribonucleoproteins
70
Patient with Sjogren suddenly have unilateral enlargement of parotid gland
B cell lymphoma (MALT lymphoma)
71
Patient with acute onset monoarthritis, episodic pain
gout
72
what crystals 1. gout 2. pseudogout
gout: monosodium urate pseudogout: calcium pyrophoshate (rhomboid)
73
what type of joints do pseudogout affect
large joints, like the knee
74
what type of diuretics can cause gout
Loops and Thiazides - hyperuricemia
75
glycogen storage disease that cause gout
von gierke
76
negative birefringent what color
yellow parallel (BLUE parallel in pseudo) blue perpendicular
77
diseases associated with pseudogout
* hemachromatosis
* hyperparathyroid
* joint trauma
78
infectious arthritis in sexually active
n. gonorrheae
79
Infective Arthritis in older kids and adults
s. aureus
80
Gonococal arthrtis presentation
migratory arthritis with asymmetric pattern STD synovitis tenosynovitis dermatitis
81
Seronegative spondyloarthropathies what HLA
HLA-B27
82
sausage fingers pencil in a cup deformities on a Xray
psoriatic arthritis
83
vertebral body fusion
ankylosing spondylitis
84
see Aortic Regurg in which type of Seronegative arthritis
ankylosing spondylitis
85
conjunctivitis, urethritis, arthritis
Reactive arthritis reiter
86
cause of reactive arthritis
Post GI: shigella, salmonella, Yersinia, Campylo Chlymadia
87
Circinate balanitis
annular dermatitis of glan penis seen in reiter
88
keratoderma blennorrhagicum
hyperkeratotic vesicles in palms and soles reiter
89
SLE die with what type of kidney disease
nephritic DPGN
90
most sensitive SLE test
ANA
91
most specific SLE test
dsDNA, anti-smith
92
what is anti-Smith antibodies
Anti-snRNPs for splicing
93
drug induced SLE what antibodies
anti-histone
94
cause of drug-induced SLE
Methyldopa, Minocycline, Hydralazine, Isoniazid, Phenytoin, Sulfa drugs, Etanercept, Procainamide
95
antibodies in antiphospholipid syndrome
lupus anticog anticardiolipin anti-B2 glycoprotein
96
false positive VDRL
Viral infection (eg, EBV, hepatitis) Drugs (IV) Rheumatic fever Lupus and leprosy. Pregnancy
97
lupus anticog produce paradoxical prolongation of PT or aPTT
PTT
98
List the joint findings in osteoarthritis (6)
1. Osteophytes (bone spurs) 2. joint space narrowing 3. subchondral sclerosis and cysts 4. eburnation (polished ivory like appearance of bone) 5. Heberden nodes 6. Bouchard nodes Synovial fluid non-inflammatory (WBC
99
this marker in SLE indicate poor prognosis
anti-dsDNA
100
MCP spared a. osteoarthris b. RA
osteoarthritis
101
RA + pneumoconiosis
Caplan syndrome
102
A 40 yo female comes in with a Hx of dyspnea especially after climbing stairs. HR and pulse are normal. After doing pulmonary function test, results show an elevated FEV1/FVC ratio. She also has a slight facial droop that has been worsening over the months. What pathology is associated with these symptoms, and what findings are prominent on imaging?
The pt has Sarcoidosis and hilar nodes are prominent on CXR known as potato sac lymph adenopathy bilaterally. The facial droop is due to Bell's palsy.
103
A physician orders a biopsy for an African descent patient with positive HX for Sarcoidosis. What will be found in the biopsy (appearance and inclusions)?
Epithelioid granulomas containing Asteroid and Schuamann bodies
104
A physican orders lab test for a 40 yo female with interstitial fibrosis suspected of Sarcoidosis. What metabolites will be elevated and the mechanism behind the pathology
Hypercalcemia. Sarcoidosis causes increased activation 1 alpha hydroxylase in the kidney. This mediates Vitamin D activation and leads to increased Ca reabsorption
105
A 60 yo female comes into the ER complaining of severe pain in her hips. She previously stated that she has been having intermittent pain in her temples What disease is she affected by at the moment? Which lab results will be found?
Polymyalgia Rheumatica --\> Assoc with GCA. Lab reults will show elevated ESR, CRP.
106
A 40 yo female comes to her family physician complaining of pain and stiffness in her muscles. She also feels lethargic during the day, and is having trouble sleeping at night. What Dx do the physician make and what does he prescribe?
Fibromyalgia TX with SNRI/TCA and anti-convulsants and regular exercise
107
A patients come in a malar rash and redness on her hands. The physician also notices that the patient's hands look roughed up as if she has been working outside with her hands. What is the Dx and TX?
Dermatomyositis with perimysial inflammation from CD4 Th cells. Tx with corticosteroids + MTX
108
Immunopathologically how does Polymyositis differ from dermatomyositis?
Polymyositis- Cd8 +CTL induced endomysial inflammation Dermatomyositis - CD4+ Th cells induced permysial inflammation
109
A 40 yo female comes in with a Hx of dyspnea especially after climbing stairs, and has lost 10 lbs of weight in the last 3 months. HR and pulse are normal. She also feels tired easily and feels weakness in washing dishes in the morning and prefers to do them at night time. What pathology is associated with these symptoms, and what underlying mechanism is associated with it?
Patient has Lambert Eaton syndrome where the Abs are destroying her presynaptic Ca Ch and decrease ACh release in the NMJ. LE syndrome is associated with Small CC of the lung which explains the dyspnea and cachexia
110
How does Myasthenia Gravis differ from LE with regards to muscle movement? Why?
Muscle weakness worsens as the day progresses b/c Abs are against the AcH receptor. As the day ends the ACh receptors decrease which causes increased weakness later.
111
A 45 yo female comes into her physician and complains of increased tightness and puffiness in her skin. She also shows that fingertips around her nails feel rougher than usual. What is the physician worried about after confirming his DX with these lab results to be positive?
The patient is positive for anti topoisomerase I Ab (SCl-70) as and has Diffuse systemic Scleroderma. These patients have a high mortality rate with renal and pulmonary sclerosis.
112
Name the findings in Limited Scleroderma (5 S/S) and the Ab associated with the path?
Limited Scleroderma is associated with anti centromere AB CREST syndrome 1) Calcinosis 2) Raynaud's phenomenon 3) Esophageal dysmotility 4) Sclerodactyly 5) Telengectasia
113
Genetic issue with Ewing sarcoma?
t(11;12) translocation causing fusion protein EWS-FLI 1
114
Rx associated with Osteoporotic fractures?
* anticonvulsants that (+) p450 (phenobarbital, phenytoin, carbamazepine) - inc Vit D catabolism
* Aromatase (-)'rs/Hydroxyprogesterone - Dec estrogen
* GnRH (+)'r - dec testosterone/estrogens
* PPI - dec Ca absorption
* Glucocorticoids,
* Unfractionated heparin,
* Thiazolidindiones - dec bone formation
115
bony mass with radiolucent core, nighttime pain, cental nidus
osteoid osteoma
116
Causes of uric acid overproduction
Lesch Nyhan, PRPP excess, inc cell turnover (tumor lysis syndrome), von Gierke disease, dec G6Pase
117
which gout rx works by (-) microtubule polymerization?
colchicine
118
locations of tophus formation?
external ear, olecranon bursa, achilles tendon
119
Rx that inc uric acid formation
Pyrazinamide, Thiazides, Furosemide, Niacin, Cyclosporin
120
rhomboid crystals that are birefrigent?
calcium pyrophosphate deposition disease (pseudo gout)
121
invasion of exocrine glands by lymphocytes?
sjogrens (why has dry eyes and mouth)
122
migratory arthritis seen with infectious arthritis and what other illness?
Lyme disease
123
Decrease of what immune components with SLE?
C3, C4, CH50 - due to immune complex formation
124
associated with anti U1 RNP antibodies
mixed CT disease (speckled ANA)
125
increased serum ACE levels and CD4+/CD\*+ ration in bronchoalveolar lavage fluid?
Sarcoidosis
126
why is there hypercalcemia with sarcoidosis/.
due to inc alpha hydroxylase mediated vitamin D (+)'n in macrophages
127
why can polymyalgia rheumatica present with pain in shoulders and hips, as well as temporal pain?
associated with temporal arteritis (giant cell)
128
markers with polymyositis/dermatomyosititis?
Inc CK, pos for ANA, anti-Jo1, anti-SRP, and anti-Mi2
129
red, sometimes scaly, papules that erupt on any of the finger joints, made worse by sunlight exposure?
Gottron papules of Dermatomyositis
130
What is Raynaud's?
dec blood flow to the skin due to arteriolar vasospasm in response to cold or stress, often in fingers and toes
131
How to treat Raynaud's?
CCB
132
triad of autoimmunity + noninflammatory vasculopathy + collagen deposition with fibrosis?
Scleroderma
