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MSK 2 > Robbins - Joints and ST Tumors (Ch. 26) > Flashcards

Robbins - Joints and ST Tumors (Ch. 26) Flashcards

1
Q

2 synovial cell types in synovial joints (w/ function)

A

Type A = phagocytic macrophages

Type B = hyaluronic acid producing fibroblasts

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2
Q

Why is hyaline cartilage unique?

A

No blood supply, no lymphatic drainage, no innervation

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3
Q

Composition of hyaline cartilage

A

Water (70%), Type 2 collagen (10%), proteoglycans (8%), chondrocytes

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4
Q

How is articular cartilage destroyed pathologically?

A

Increased IL-1 and TNF that triggers degradation – from chondrocytes, synoviocytes, fibroblasts, and inflammatory cells

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5
Q

50+, deep achy pain that worsens with use or early morning, crepitus, limited ROM. Muscle spasms, radicular pain, and neurologic deficits

Other name?

What is it?

A

Osteoarthritis

Degenerative joint disease (DJD)

Degeneration of cartilage and disordered repair that results in structural and functional failure of synovial joints

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6
Q

Most common cause of osteoarthritis

It is a disease in which chondrocytes respond to ___ and ___ stresses resulting in the breakdown of the ___

A

Old age

Biochemical, mechanical
Matrix

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7
Q

Men vs. women - most common osteoarthritis locations

A

Men - Hips

Women - Knees, Hands

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8
Q

Early vs. Late OA – what is happening microscopically?

A

Early – chondrocytes proliferate into clusters, water content increases, secrete mediators and proteases that cleave proteoglycans and collagen fibers

Late – repetitive injury causes chronic inflammation, chondrocyte death, cartilage loss, and subchondral changes

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9
Q

OA - see what macroscopically? (3)

A

Polished ivory surface (eburnation) w/ joint mice (as cartilage is sloughed off) and fibrous-walled cysts

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10
Q

Heberden nodes

Bouchard nodes

Seen in what?

A

Osteophytes at the distal IP joints

Bony outgrowths or cysts of the proximal IP joints

Osteoarthritis

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11
Q

Malaise, fatigue, generalized MSK pains. Joints are swollen, warm, painful, and stiff in morning or after inactivity. Joint enlargement and limited ROM.

Most common joints?

A

Rheumatoid arthritis

Hands and feet, then other extremity joints

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12
Q

Progression of RA in the joint?

Damage caused by what mediators?

A

Complete ankylosis – fusion of bones, thus near immobility

IL-1, **IL-17 (Th17), TNF, **IFN (Th1)

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13
Q

Characteristic exam/radiograph signs for RA (3)

A
  • Radial deviation of wrist
  • Ulnar deviation of fingers
  • Flexion-hyperextension of fingers (swan-neck)
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14
Q

Diagnosing RA (3)

A
  • Classic radiograph findings
  • Sterile, turbid synovial fluid w/ decreased viscosity, poor mucin clots, and inclusion-bearing neutrophils
  • Rheumatoid factor and anti-CCP antibodies
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15
Q

**Synovial cell hyperplasia, dense inflammatory infiltrates, increased vascularity, exudate on joint surfaces, osteoclastic activity

A

RA

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16
Q

**Mass of edematous synovium, germinal centers, inflammatory cells, granulation tissue, and fibroblasts growing over the articular cartilage, causing its erosion

A

Pannus - RA

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17
Q

RA can look like what other diseases? Why?

A

SLE or scleroderma

Lesions of skin, heart, BVs, and lungs are common as well

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18
Q

Molecular findings in RA (2)

A

Anti-CCP (citrullinated peptides) autoantibodies

Serum IgM or IgA against the Fc portion of serum IgG (Rheumatoid factor)

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19
Q

Genetic susceptibility for RA

Other findings in RA (other organs) (2)

A

HLA-DRB1

Nontender subQ nodules w/ fibrinoid necrosis, small/medium vasculitis

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20
Q

Child under 16, arthritis for 6+ weeks

Which joints most common? Few or many?

Molecular finding?
RF? Rh nodules?

Damage done by what?

A

Juvenile idiopathic arthritis

Large joints - few (oligo)

ANAs
NO RF or nodules

Cytokines (same as RA)

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21
Q

Seronegative spondyloarthropathies - what are they?

A

Immune-mediated diseases (T-cell response), in a susceptible person, to some environmental antigen, that cross reacts w/ native molecules of the MSK system and causes arthritis

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22
Q

Seronegative spondyloarthropathies - 4 diseases

A

Ankylosing spondylitis
Reactive arthritis
Enteritis associated arthritis
Psoriatic arthritis

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23
Q

4 commonalities w/ all the seronegative spondyloarthropathies

Why are they termed “seronegative”?

A
  • Pathology of ligamentous attachments of the joint, NOT the synovium
  • Sacroiliac joint involvement
  • NO RF
  • HLA-B27 association

No specific autoantibodies

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24
Q

Vertebrae + SI joints
10-20, young males - low back pain, spinal immobility
HLA-B27 (90%)

What is it?

A

Ankylosing spondyloarthritis

Destruction of articular cartilage and bony ankylosis (fusion)

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25
Q

Male, 20-30
Urethritis/cervicitis (non-gonococcal) or bloody diarrhea –> LATER arthritis (joint stiffness, low back pain) and conjunctivitis
HLA-B27 (80%)
Symptoms wax and wane for months

What is it?

Organisms?

Common specific joint findings? (2)

Other complications? (4)

A

Reactive arthritis (Reiter syndrome)

Autoimmune reaction initiated by the GU/GI infection

Chlamydia or bloody diarrhea GI bacteria (4)

Sausage finger/toe
Calcaneal spurs

Balanitis, conjunctivitis, cardiac arrhythmias, aortic regurgitation

26
Q

30-50, recurrent skin plaques w/ silver scab, joint pains in distal IP joints of hands and feet

Genetic susceptibility?

Common deformity in fingers/toes

How does this arthritis differ from RA?

A

Psoriatic arthritis

HLA-B27 and HLA-C

Pencil-in-cup deformity

RA = proximal IP joints

27
Q

Infectious arthritis - what is it?

4 types?

A

Class of arthritis diseases caused by various organisms and infections that seed the joint via hematogenous spread

  • Pyogenic
  • Mycobacterial (TB)
  • Lyme disease
  • Viral
28
Q

Sudden, acutely painful and swollen joint w/ restricted ROM. Fever, leukocytosis, and elevated sed rate. Purulent fluid on synovial fluid aspiration. Stains positive for an organism.

Most common organism in…

  • Kids
  • Adults
  • Sickle cell
  • Sexually active females
A

Suppurative (bacterial) arthritis

Kids = H. flu
Adults = staph. aureus
HbSS = Salmonella
Females = Gonococcus
29
Q

Gradually increasing joint (LE) pain, confluent granulomas w/ central caseous necrosis, lung mass or osteomyelitis

A

Mycobacterial arthritis

30
Q

Hiking in NE US, skin lesion followed later by arthritis of large joint(s) that migrate every 1-2 months. Anti-organism antibody detected in serum.

Organism?

A

Lyme arthritis

Borrelia burgdorferi

31
Q

Causes of viral arthritis (6)

A

HIV, HBV, HCV, EBV, Parvo, Rubella

32
Q

30+ male w/ obesity, alcoholic, and/or metabolic syndrome. Transient attacks of acute arthritis w/ redness and warmth in small extremity joint. Dense neutrophilic infiltrate in synovium. Long, slender, needle-shaped crystals found.

Necessary finding on labs?

Common 1st joint?

A

Gout (acute arthritis)

Hyperuricemia (> 6.8 mg/dL)

BIG TOE

33
Q

MOST causes of hyperuricemia are (known/unknown) and (primary/secondary)

Genetic susceptibility?

Causes of 2º gout? (2)

A

Unknown, Primary

HGPRT mutation (can’t salvage purines)

Chemotherapy (leukemia), Decreased excretion (CKD)

34
Q

Molecular cause of inflammation in gout

A

MSU crystals –> cytokines and crystal phagocytosis –> proteases and lysosomal enzymes –> injury/inflammation

35
Q

Joints most commonly affected in gout

Why?

A

Peripheral joints

MSU crystallizes more at lower temperatures

36
Q

Risk factors for gout (5)

A
  • Prolonged hyperuricemia
  • HGPRT deficiency (X-linked)
  • Heavy alcohol consumption
  • Obesity
  • Drugs (thiazides) that reduce excretion
37
Q

What happens after many episodes of acute gout?

A

Chronic tophaceous arthritis…

MSU crystals encrust the articular surfaces, forming visible deposits. Synovium becomes hyperplastic and thickens to form a PANNUS, which leads to articular bone erosions and even bony ANKYLOSIS

38
Q

Large aggregations of urate crystals surrounded by intense inflammatory reaction of foreign body giant cells

When do these form?

A

Tophi – hallmark of gout

About 12 years after the initial acute attack

39
Q

Gout - non-arthritic association (chronic)

A

Nephropathy - nephrolithiasis and pyelonephritis

40
Q

50+, asymptomatic or mild arthritis of upper extremity joint(s), crystals within articular cartilage, menisci, and intervertebral discs. Chalky white, friable deposits seen as oval purple-blue aggregates on staining. Rhomboid, birefringent. NOT NEEDLE-SHAPED.

Other names?

A

Calcium pyrophosphate crystal deposition (CPPD) disease

Pseudo-gout
Chondrocalcinosis

41
Q

Causes of secondary pseudo-gout (6)

A 
Joint damage
Hyper-PTH
Hypothyroidism
Hemochromatosis
DM
Ochronosis (alkaptonuria)
42
Q

Hereditary form of pseudo-gout

A

Mutation in pyrophosphate transport channel

43
Q

Most cysts and tumors of the joints are due to ____ to trauma or degenerative processes, NOT ____

A

Reactivity

NOT primary

44
Q

Small pea-sized firm, fluctuant, translucent nodule in the wrist. Filled w/ synovial fluid. No cellular lining, no connection to the synovial fluid.

Comes via ____

A

Ganglion cyst

Cystic/myxoid degeneration of connective tissue

45
Q

Herniation of synovium through the joint capsule or enlargement of a bursa, producing a fluctuant mass. Hyperplastic synovial lining w/ inflammatory cells and fibrin.

Most common one?

A

Synovial cyst

Baker cyst (popliteal - RA)

46
Q

Soft tissue tumors are most likely (benign/malignant)

A

Benign

47
Q

40-60, well-encapsulated mass on the trunk or proximal extremity. Soft, mobile, painless. Yellow adipose tissue.

A

Lipoma

48
Q

50-60, poorly differentiated mass in deep tissue of proximal extremity or retroperitoneum. Sheets of anaplastic cells w/ bizarre nuclei and immature adipocytes (lipoblasts).

Genetics?

A

Pleomorphic liposarcoma

(12;16) FUS-DDIT3

49
Q

50-60, mass in deep tissue of proximal extremity or retroperitoneum. Abundant ground substance and rich capillary network w/ scattered immature adipocytes and primitive round-stellate cells

Genetics?

A

Myxoid liposarcoma

(12;16) FUS-DDIT3

50
Q

Irregular or nodular thickening of the palmar fascia, w/ puckering and dimpling. 4th and 5th fingers are partially flexed

What is it?

A

Superficial fibromatosis - Palmar (Dupuytren contracture)

Infiltrative fibroblastic proliferation, causing local deformity

51
Q

Palpable mass on dorsolateral penis. Abnormal curvature of the shaft, difficulty urinating.

What is it?

A

Superficial fibromatosis - Penile (Peyronie disease)

Infiltrative fibroblastic proliferation, causing local deformity

52
Q

Women 13-30, large poorly-demarcated mass, rubbery and tough, with infiltration of surrounding tissues. Anterior abdominal wall, limb girdle, or mesentery.

Genetics?

Familial disease w/ predisposition?

A

Deep fibromatosis (Desmoid tumor)

APC or Beta-catenin mutation

FAP (APC mutation)

53
Q

Almost all skeletal muscle tumors are ____

A

MALIGNANT

54
Q

Child. Infiltrating mass of round and spindle cells in a myxoid stroma. Cells w/ cross striations.

Most common locations?

A

Embryonal rhabdomyosarcoma

Sinuses, head/neck, GU tract

55
Q

Child, Infiltrating mass of uniform round cells w/ little cytoplasm. Fibrous septae divide the cells into clusters or aggregates. Central cells are discohesive, peripheral cells are adherent to the septae.

Supposed to look like what?

Do NOT see ___

Genetics?

A

Alveolar rhabdomyosarcoma

Pulmonary alveoli

Cross striations

(1;13) or (2;13) PAX-FOXO1

56
Q

Small, painful nodules in the skin, fascicles of densely eosinophilic spindle cells that intersect at right angles. Blunt-ended, elongated nuclei.

A

Leiomyoma (of erector pili mm)

57
Q

Painless firm mass in the retroperitoneum or deep extremity. Eosinophilic spindle cells w/ blunt-ended hyperchromatic nuclei in interweaving fascicles. Stains w/ actin-myosin antibodies.

A

Leiomyosarcoma

58
Q

20-49, deep-seated mass present for several years. Uniform spindle cells w/ scant cytoplasm. Potential gland-like structures w/ cuboidal to columnar epithelioid cells. Keratin-positive (some).

Genetics?

A

Synovial sarcoma

(x;18) SS18-SSX1/2/4

59
Q

Synovial sarcoma – usually in the synovium?

A

NO (misnomer) – can be joint, chest wall, head/neck

60
Q

Middle age or older. Large mass in deep soft tissue of thigh. Pleomorphic, anaplastic spindle to polygonal cells w/ irregular/bizarre nuclei. Abundant mitotic figures. Lacking differentiation.

Common?

A

Undifferentiated pleomorphic sarcoma (UPS)

YES - largest category of adult sarcomas

MSK 2 (20 decks)

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