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107 Cards in this Set

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what type of ossification is not affected in achondroplasia?
membranous - skull, facial bones, and axial skeleton are normal
Heberden's nodes (DIP) and Bouchard's nodes (PIP)
osteoarthritis
pain in weight-bearing joints after use that improves with rest
osteoarthritis
destruction of articular cartilage, subchondral bone formation, sclerosis, osteophytes, and eburnation are associated with what?
osteoarthritis
what are joint mice? what are they associated with?
osteophytes that fracture and float into synovial fluid along with fragments of separated cartilage - seen in osteoarthritis
pannus formation in joints, especially MCP, PIP
rheumatoid arthritis
morning stiffness improving with use; symmetric joint involvement, and systemic symptoms (fever, fatigue, pleuritis, pericarditis)
rheumatoid arthritis
Rx for severe cases of osteoporosis?
bisphosphonates or pulsatile PTH
type I osteoporosis?
postmenopausal - increased bone resorption due to decreased estrogen levels
type II osteoporosis?
senile osteoporosis - affects men and women over 70
what types of fractures are associated with type I osteoporosis?
vertebral crush fractures - acute back pain, loss of height, kyphosis
what type of fractures are associated with type II osteoporosis?
distal radius (Colles') fractures, vertebral wedge fractures
in this disease there is a failure of normal bone resorption that leads to thickened, dense bones
osteopetrosis (marble bone disease)
in this disease osteoclasts function abnormally
osteopetrosis
what level of alk phos is osteopetrosis associated with?
normal
what causes osteitis fibrosa cystica?
hyperparathyroidism
this disease is characterized by 'brown tumors', high serum calcium, low serum phosphorus, and high alk phos
osteitis fibrosa cystica
these are cystic spaces lined by osteoclasts, filled with fibrous stroma and sometimes blood
brown tumors (seen in osteitis fibrosa cystica)
in this disease there is abnormal bone architecture caused by an increase in both osteoblastic and osteoclastic activity
Paget's disease (osteitis deformans)
what level of alk phos is Paget's disease associated with?
increased (normal serum calcium, phosphorus, adn PTH)
in this disease, bone is replaced by fibroblasts, collagen, and irregular bony trabeculae
polyostotic fibrous dysplasia
Albright's syndrome is a form of what?
polyostotic fibrous dysplasia
multiple unilateral bone lesions, unilateral ppigmented skin lesions, precocious puberty
Albright's syndrome
pain and stiffness in shoulders and hips, often with fever, malaise and weight loss; doesn't cause muscular weakness
polymyalgia rheumatica
what is polymyalgia rheumatica associated with?
temporal (giant cell) arteritis; occurs in patients over 50
is ESR increased or decreased in polymyalgia rheumatica?
increased
progressive proximal muscle weakness caused by CD8+ T cell-induced injury to myofibers
polymyositis
muscle biopsy with evidence of inflammation is diagnostic for what?
polymyositis
increased CK, increased aldolase, and positive ANA, anti-Jo-1
polymyositis/dermatomyositis
Raynaud's, arthralgias, myalgias, fatigue, esophageal hypomotility
mixed CT disease
antibodies to U1RNP?
mixed connective tissue disease
what is the classic triad of Sjogren's syndrome?
xerophthalmia, xerostomia, arthritis
patients with Sjogren's syndrome are at increased risk for what?
B cell lymphoma
what is enlarged in Sjogren's syndrome?
parotids
what patients does Sjogren's primarily affect?
females between 40-60
autoantibodies to ribonucleoprotein antigens, SS-A (Ro), SS-B (La)
Sjogren's
fever, fatigue, weight loss, nonbacterial verrucous endocarditis, hilar adenopathy, and Raynaud's
SLE
what are the kidney findings in SLE?
wire loop lesions in kidney with immune complex deposition (with nephrotic syndrome)
what part of the SI does celiac sprue tend to affect?
jejunem
what skin finding is celiac sprue associated with?
dermatitis herpetiformis
what malignancy is celiac sprue most strongly affiliated with?
T cell lymphoma
blunting of villi and lymphocytes in the lamina propria are suggestive of what?
celiac sprue
what type of crystals precipitate in gout?
monosodium urate
what type of diuretics is gout associated with?
thiazide
needle shaped negatively birefringent
gout
with what type of crystals is pseudogout associated?
calcium pyrophosphate
rhomboid crystals that are weakly positively birefringent
pseudogout
what type of joints does pseudogout usually affect?
large joints, especially the knee
what is the treatment for pseudogout?
none
pulmonary hemorrhages leading to hemoptysis, renal lesions leading to hematuria, anemia, crescentic glomerulonephritis
Goodpasture's syndrome
what type of hypersensitivity reaction does Goodpasture's represent?
type II
anti-glomerular basement membranes produce what type of staining on immunofluorescence?
linear
what population does Goodpasture's usually affect?
men 20-40
what level is elevated in sarcoidosis?
ACE
immune-mediated, widespread noncaseating granulomas common in black females
sarcoidosis
what do the epithelial granulomas contain in sarcoidosis?
microscopic Schaumann and asteriod bodies
why is hypercalcemia associated with sarcoidosis?
elevated conversion of vitamin D to its active form in epitheliod macrophages
restrictive lung disease, bilateral hilar lymphadenopathy, erythema nodosum, Bell's palsy are associated with what?
sarcoidosis
patients with what disorder have decreased sensitivity and often anergy to skin test antigens?
sarcoidosis
chronic inflammatory disease of spine and SI joints -> ankylosis, uveitis, and aortic regurgitation
ankylosing spondylitis
this disorder is characterized by excessive fibrosis and collagen deposition throughout the body
scleroderma (progressive systemic sclerosis)
widespread skin involvement, rapid progression, early visceral involvement
diffuse scleroderma
CREST syndrome
calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, telangiectasia
idiopathic, segmental, thrombosing vasculitis of intermediate and small peripheral arteries and veins; seen in heavy smokers
Buerger's disease
intermittent claudication, superficial nodular phlebitis, and cold sensitivity, severe pain in affected part (may lead to gangrene) are characteristic of what?
Buerger's disease
what is the treatment for Buerger's disease?
quit smoking
what type of arteries does Takayasu's arteritis affect?
medium and large arteries
granulomatous thickening of aortic arch and/or proximal great vessels; elevated ESR
Takayasu's arteritis ("pulseless disease")
who does Takayasu's arteritis primarily affect?
Asian females under 40
what are the symptoms of Takayasu's arteritis?
fever, arthritis, night sweats, myalgia, skin nodules, ocular disturbances, weak pulses in upper extremities:
FAN MY SKIN On Wednesday
this is the most common vasculitis that affects medium and small arteries, usually branches of the carotid
temporal/giant cell arteritis
unilateral headache, jaw claudication, impaired vision (occlusion of ophthalmic artery, which can lead to blindness)
temporal/giant cell arteritis
half of patients with temporal arteritis have what?
systemic involvement and polymyalgia rheumatica (proximal muscle pain, periarticular pain)
what is elevated in temporal arteritis? what is the treatment?
elevated ESR; steroids are Rx
necrotizing immune complex inflammation of medium-sized muscular arteries, typically involving renal and visceral vessels
polyarteritis nodosa
approximately 30% of patients with polyarteritis nodosa are seropositive for what?
Hep B
multiple aneurysms and constrictions are seen on angiogram in what disease?
polyarteritis nodosa
what is the treatment for polyarteritis nodosa?
corticosteroids, cylophosphamide
what is the triad associated with Wegener's granulomatosis?
necrotizing vasculitis, necrotizing granulomas in the lung and upper airway, and necrotizing glomerulonephritis
perforation of nasal septum, chronic sinusitis, otitis media, mastoiditis, cough, dyspnea, hemoptysis, and hematuria are associated with what disease?
Wegener's granulomatosis
what is a strong marker of Wegener's granulomatosis?
C-ANCA
what might be seen on chest x ray in Wegener's?
large nodular densities
what is the treatment for Wegener's?
cyclophosphamide and corticosteroids
this vasculitide is like Wegener's but lacks granulomas and is associated with P- or C-ANCA
microscopic polyangitis
this is a vasculitis limited to the kidney
primary pauci-immune crescentic glomerulonephritis
granulomatous vasculitis with eosinophilia that involves lungs, heart, skin, kidneys, nervous system; often seen in atopic patients
Churg-Strauss syndrome
this is an acute, self-limiting disease of infants/kids; acute necrotizing vasculitis of small/medium sized vessels; fever, congested conjunctiva, changes in lips/oral mucosa, lymphadenitis
Kawasaki disease/ mucocutaneous lymph node syndrome
what potentially serious outcome can result from Kawakaki disease?
coronary aneurysms
what is the most common form of childhood systemic vasculitis?
Henoch-Schonlein purpura
skin rash, arthralgia, intestinal hemorrhage, abdominal pain, and melena in kid?
Henoch-Schonlein purpura
where do giant cell bone tumors most commonly occur? in what age group?
epiphyseal end of long bones - 20-40 years old
this is a locally aggressive benign tumor often around the distal femur, proximal tibial region
giant cell tumor
what type of bone tumor has a 'soap bubble' or 'double bubble' appearance on x-ray?
giant cell tumor
spindle-shaped cells with multinucleated giant cells found in what type of benign bone tumor?
giant cell tumor
what is the most common benign bone tumor?
osteochondroma (exostosis)
this type of bone tumor is usually found in men <25 and commonly originates from long metaphysis
osteochondroma
this is a benign cartilaginous neoplasm found in intramedullary bone, usually in the distal extremities
endochondroma
what is the most common malignant tumor of bone?
osteosarcoma
this malignant bone tumor's peak incidence is in men 10-20 years old and is commonly found in the metaphysis of long bones
osteosarcoma
name some predisposing factors for osteosarcoma
Paget's disease of bone, bone infarcts, radiation, and familial retinoblastoma
Codman's traingle or sunburst pattern on x-ray
osteosarcoma
this is an anaplastic small blue cell tumor of bone most common in boys <15
Ewing's sarcoma
what is the characteristic appearance of Ewing's sarcoma?
onion skin
this malignant bone tumor is aggressive with early mets but is responsive to chemo; commonly appears in diaphysis of long bones, pelvis, scapula, and ribs
Ewing's sarcoma
what is the chromosomal translocation associated with Ewing's sarcoma?
11; 22
this is a malignant cartilaginous tumor most common in men 30-60
chondrosarcoma
where are chondrosarcomas usually located? what can they originate from?
pelvis, spine, scapula, humerus, tibia, or femur; may be primary or from osteochondroma (rare)