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71 Cards in this Set

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What is the most common form of arthritis?
Osteoarthritis
this problem Affects at least one joint in 80% of people over 70 years old
Osteoarthritis
Insidious onset of joint stiffness
Deep, aching joint pain, which worsens with repetitive motion
Decreased range of motion
Crepitus
Joint effusions and swelling
Osteophytes may cause nerve compression

this describes?
osteoarthritis
what is the major pathophysiology behind osteoarthritis?
Degeneration and loss of articular cartilage
where is OA commonly seen on the body?
Weight-bearing joints --> knees, hips, and spine
A systemic, chronic, inflammatory disease characterized by progressive arthritis, production of rheumatoid factor, and extra-articular manifestations

what is this?
RA
what causes RA?
an autoimmune reaction triggered by an infectious agent in a genetically susceptible individual.
what is the genetic predisposition seen in RA?
HLA-DR4 and DR1
patient comes in complaining of morning stiffness and symmetrical pain of the wrists. What does she likely have?
RA
what is Pannus formation and what is it seen in?
proliferation of the synovium and granulation tissue over the articular cartilage of the joint

RA
what is the major lab finding in RA?
Rheumatoid factor (RF)

Usually an IgM autoantibody against the Fc fragment of IgG
the titer of what lab finding correlates with the prognosis of the associated disease?
Rheumatoid Factor for RA
monosodium urate crystals in joints, resulting in recurrent bouts of acute arthritis describes?
GOUT
what leads to gout?
overproduction of uric acid
a 70 year old man comes in to your office with an exquisitely painful, inflamed big toe. What does he have?
gout
Deposition of calcium pyrophosphate crystals describes?
pseudogout
what are the crystals associated with pseudogout
Deposition of calcium pyrophosphate crystals
man comes in complaining of knee joint pain. You see a history of diabetes and hypothyroidism. You have ruled out osteoarthritis and RA, what is the likely cause? What lab finding would you see
pseudogout

Deposition of calcium pyrophosphate crystals
what is the most common dystrophy?
x-linked Duchenne muscular dystrophy
a boy is born with no seeming problems, yet he has become increasingly weak by age 5. By age 10 he is in a wheelchair. What is the problem?
x-linked Duchenne muscular dystrophy
Disease progresses relentlessly until death by the early twenties
Begins in the pelvic girdle muscles, then extends to the shoulder girdle
Heart and cognitive impairment appears to be a component of the disease

what is this?
x-linked Duchenne muscular dystrophy
Variation in fiber size
Increased numbers of internalized nuclei
Degeneration of muscle fibers
Regeneration of muscle fibers
Proliferation of enomysial connective tissue

are commonly seen in what?
Duchenne muscular dystrophy and Becker muscular dystrophy
where is the gene that is responsible for x-linked muscular dystrophy found?
Xp21
slowly progressive muscle weakness of the legs and pelvis, presents onset in adolescence. What is this?
Becker muscular dystrophy
what is the major difference btw Becker and Duchenne muscular dystrophy?
Becker's has later onset in life
a child is brought to the emergency department with a laceration that requires stitches. Upon administration of anesthesia, the patient starts to expeirence tachycardia, tachypnea, muscle spasms, and later hyperpyrexia. What does this patient have? What type of problem is this?
Malignant hyperpyrexia (malignant hyperthermia)

Ion Channel Myopathies
patient presents with muscle weakness that actually gets better with repeated contractions. What is this?
Lambert-Eaton sydrome
what is commonly associated with Lambert-Eaton syndrome?
small cell carcinoma of the lung
Your 68 y/o gent patient comes to you for evaluation of 25 pound weight loss (without intention) and proximal muscle weakness over a six month course. Physical exam shows that the weakness does not increase with repetitive motion. Most likely underlying mechanism for this is?



a. Diabetes mellitus

b. Becker muscle dystrophy

c. Toxic myopathy

d. Small cell lung carcinoma

e. Carcinoma of the thymus
D
pt is easily fatigued, exhibits ptosis, and gets more fatigued by the end of the day. What does this patient have? What is the mech?
Myasthenia Gravis

Antibodies to AChR
Achondroplasia is what?
Most common form of inherited dwarfism
what is the problem seen in Osteogenesis imperfecta
abnormal synthesis of type I collagen
a patient comes in with recurrent fractures. On exam you see a thin sclera with a blue hue. The patient is also hypermobile. What does this person have?
Osteogenesis imperfecta (OGI) (“brittle bone disease”)
a patient presents with deafness, small, fragile, and discolored teeth, and a thin dermis. The patient has a history of recurrent fractures. What do they have?
osteogenesis imperfecta
patient has excessive resorption of bone followed by disorganized bone replacement producing thickened but weak bone. What does this describe?
Pagets
a european man has an infection with paramyxovirus and now is having bone deformity and fracture. What does he have??
Pagets
if you see a haphazard arrangement of cement lines, creating a “mosaic pattern” of lamellar bone what does the patient have?
Pagets
Highly elevated serum alkaline phosphatase, Increased levels of urinary hydroxyproline, and xrays that show bone enlargement with lytic and sclerotic areas would be indicative of?
Pagets
decreased bone mass (osteopenia), resulting in thin, fragile bones that are susceptible to fracture

this defines?
Osteoporosis
this is the Most common bone disorder in the US that
Most commonly occurs in postmenopausal Caucasian women and the elderly
Osteoporosis
what are some of the primary causes of Osteoporosis
Estrogen deficiency (postmenopausal, Turner’s syndrome)
Genetic factors (low density of original bone)
Lack of exercise
Old age
Nutritional factors
benign neoplasm that frequently involves the skull and facial bones is what?
osteoma
what is commonly associated with Gardners syndrome?
osteoma
benign, painful growth of the diaphysis of a long bone, often the tibia or femur
Osteoid osteoma
you have a male age 22 comes in with pain along his femur that is worse at night, but is relieved with aspirin. You see a central radiolucency surrounded by a sclerotic rim on x-ray.

What does the pt have?
Osteoid osteoma
Most common primary malignant tumor of bone is?
Osteosarcoma
patients with familial retinoblastoma have a high risk for what?
Osteosarcoma
if you see Codman’s triangle (periosteal elevation)
“Sunburst” pattern
Bone destruction

on xray, what do you have?
Osteosarcoma
if you see anaplastic cells producing osteoid and bone
Osteosarcoma
Malignant neoplasm of undifferentiated cells arising within the marrow cavity is what?
Ewing sarcoma
What are the genetics associated with Ewing sarcoma?

****** MUST KNOW
classic translocation t(11;22), which produces the EWS-FLI1 fusion protein

Heed this: Basketball legend Patrick Ewing was #33, so 11+22=33
Sheets of undifferentiated small round blue cells resembling lymphocytes and
Homer Wright pseudorosettes are micro findings indicative of?
Ewing Sarcoma
there are 5 common sites of metastatic lesions. What are they?
Prostate (often osteoblastic)
Breast
Lung
Thyroid
Kidney
Mot common soft tissue tumor of adulthood?
Lipoma
Benign tumors of fat are what?
Lipomas
where is the characteristic chromosomal abnormality seen in a lipoma?
13q
where are the majority of lipomas found?
subcutaneous
you have a middle aged man who comes in with a lump under the skin on the trunk and upper extremity. The lumps are benign. What are they likely?
lipomas
are lipomas painless?

******
yes EXCEPT ANGIOLIPOMAS (on forearm)
One of the most common sarcomas of adulthood
Patients  in their 40’s – 60’s
Uncommon in children
Usually arise in deep soft tissues/proximal extremities and retroperitoneum
Often develop into large tumors

this describes?
LIPOSARCOMA
lipoblasts/cytoplasmic vacuoles --> scallop the nucleus

indicative of?
Liposarcoma
presence of metaplastic bone is seen in?

*****
Myositis ossificans
Usually develops in athletic adolescents/young adults
Follows an episode of trauma (<50% of cases)
Typically arises in musculature of proximal extremities

what is this?
Myositis ossificans
what distinguishes MYOSITIS OSSIFICANS from a neoplasm?
Bone matures peripherally
Whereas it is immature/or not formed at all – in the center
Called zonation effect
Indicates a reactive process
A neoplasm has an opposite zonation effect
· most mature tissue is located centrally
muscle becoming bone is?
MYOSITIS OSSIFICANS
Characterized by nodular of poorly defined broad fascicles
Mature-appearing fibroblasts surrounded by abundant dense collagen

seen in the palm, sole, and penis
FIBROMATOSIS
what is a common feature of FIBROMATOSIS?
that it RECURS
The most common type of soft tissue sarcoma?

****
MALIGNANT FIBROUS HISTIOCYTOMA
where does malignant fibrous histiocytoma (MFH) most commonly occur? In what type of patient?
Occur in the deep soft tissues of extremities in adults

Peak in the 7th decade (but have been recorded in children)
what do most malignant fibrous histiocytomas involve? 2
deep fascia or substance of a skeletal muscle

(sarcoma of the soft tissue; most common in adults)
do malignant fibrous histiocytomas tend to recur? Do they metastasize?
Tumor is prone to local recurrence

Has the capacity to metastasize – esp. to lungs and regional L.N.’s