Pathology of Muscles, Joints, and Soft Tissue Flashcards

1
Q

1) Progressive ataxia of all four limbs
2) Hypertrophic cardiomyopathy
3) Skeletal abnormalities
4) Diabetes mellitus

A

Friedreich ataxia

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2
Q

Most common degenerative joint disease; insidious onset; non-inflammatory

A

Osteoarthritis

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3
Q

loose bodies of cartilage and subchondral bone

A

Joint mice

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4
Q

Degenerated articular surface that looks like polished ivory

A

Bone eburnation

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5
Q

Prominent osteophytes at the distal intraphalangeal joints; common in women

A

Heberden nodes

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6
Q
  • Infiltration of synovial stroma by dense perivascular infiltrates
  • increased vascularity of the synvoium
  • superficial hemosiderin deposits
  • Aggregation of organizing fibrin (rice bodies)
  • Osteoclastic activity in underlying bone
  • Pannus formation
A

Rheumatoid Arthritis

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7
Q

Mass of synovium and synovial stroma consisting of inflammatory cells, granulation tissue, and synovial fibroblasts that grow over the articular cartilage

A

Pannus formation

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8
Q

What do pannus bridges result in

A

Fibrous ankylosis

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9
Q

Firm, non-tender subcutaneous lesion that has a central zone of fibrinoid necrosis surrounded by activated macrophages; associated with RA; commonly found on elbow

A

Rheumatoid nodule

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10
Q

How is rheumatoid vasculitis different from Polyarteritis Nodusum?

A

1) Rheumatoid arthritis does not affect the kidney; PAN does not affect the lungs

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11
Q

What is the believed cause of RA?

A

1) Exposure of genetically susceptible host to arthritogenic antigen triggering an autoimmune response

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12
Q

What are the main activating molecules that are the culprits for RA?

A

IFN-gamma and IL-17

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13
Q

What is the function of IL-17 and IFN-gamma

A

1) Activate synoviocytes and macrophages

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14
Q

What is the effect of activated cartilage matrix metalloproteinases?

A

1) Destruction of articular cartilage

2) Stimulators of osteoclastogenesis and up regulate osteoclast activity (increases RANKL)

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15
Q

How do CD4 T cells do to cause the progression of RA?

A

1) Secrete IL-17 and IFN-gamma
2) Leads to an increase in inflammatory molecules (TNF)
3) Inflammatory molecules activate metaloprotease and increases the transmigration
4) Metaloprotease causes a breakdown in articular cartilage and increases the expression of RANKL
5) RANKL leads to increased osteoclast activity and formation of pannus mass

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16
Q

Joints are swollen, warm, painful, and stiff after inactivity; Greatest damage occurs after 4-5 years

A

Rheumatoid arthritis

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17
Q

What are the radiographic hallmarks of RA?

A

1) Joint effusions
2) Juxtaarticular osteopenia with erosions and narrowing of the joint space
3) Loss of articular cartilage

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18
Q

What confirms an inflammatory arthritis in examination of the synovial fluid?

A

1) High protein content
2) Neutrophils
3) Low mucin content

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19
Q

What are the criteria for diagnosing RA?

A

1) Morning stiffness
2) Arthritis in three or more joints
3) Arthritis of the hand joints
4) Symmetric arthritis
5) Rheumatoid nodules
6) Serum rheumatoid factor
7) Typical radiographic findings

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20
Q

Chronic synovitis that causes destruction of articular cartilage; found in SI joint and vertebral; Common in men; HLA-B27 positive and ARTS1 mutation

A

Ankylosing Spondyloarthritis

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21
Q

What is the initial symptom found in Ankylosing Spondyloarthritis? How does it progress?

A

1) Low back pain

2) Presents in 2nd or 3rd decade and has a chronic progressive course

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22
Q

Form of reactive arthritis defined by arthritis, nongonococcal urethritis, and conjunctivitis

A

Reiter syndrome

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23
Q

Chronic inflammatory arthropathy that affects the peripheral and axial joints and entheses; associated with psoriasis; Affects distal interphalangeal joints causing sausage fingers

A

Psoriatic arthritis

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24
Q

Acute suppurative arthritis that presents with sudden development of an acutely painful and swollen joint, fever, leukocytosis, elevated sedimentation rate

A

Bacterial arthritis

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25
Q

Chronic progressive mono-articular disease that has the formation of confluent granulomas with central caseous necrosis

A

Tuberculous arthritis

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26
Q

X linked disease, complete lack of xanthine guanine phosphoribosyl transferase; causes severe hyperuricemia, neurologic deficits with mental retardation

A

Lesch-Nyhan syndrome

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27
Q

What factors are involved with induction of hyperuricemia into gout?

A

1) Age and duration of hyperuricemia
2) Genetic predisposition
3) Heavy alcohol consumpton
4) Obesity
5) Drugs
6) Lead toxicity

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28
Q

Describe the process of an acute arthritis attack in gout

A

1) Hyperuricemia overtime results in the formation of monosodium urate and microtrophi in the synovium
2) Trauma or some other event results in the monosodium urate to be released into the synovial fluid
3) Macrophages phagocytize MSU and activate NALP3 inflamasome caspase 1
4) NALP3 cleaves and activates cytokines IL-1beta and IL-18 which induces the expression of CXCL8
5) CXCL8 causes the localization of neutrophils
6) Neutrophils release free radicals, leukotrienes, and lysosomal enzymes

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29
Q

What are the morphological changes found in gout?

A

1) Acute arthritis
2) Chronic tophaceous arthritis
3) Tophi
4) Gouty nephropathy

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30
Q

What are the morphologic findings in acute arthritis gout?

A

1) Dense, neutrophilic infiltrate that permeates the synovium and synovial fluid
2) Monosodium urate crystals present in fluid
3) When crystals resolublize the acute attack remits

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31
Q

Morphologic state that evolves from the repetitive precipitation of urate crystals during acute attacks; urates encrust the articular surfaces and form visible deposits in the synovium; urates may form a pannus

A

Chronic tophaceous arhtritis

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32
Q

Pathognomonic of gout

A

Tophi

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33
Q

Large aggregations of urate crystals surrounded by an intense inflammatory reaction of macrophages and other inflammatory cells

A

Tophi

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34
Q

What are the four stages of gout

A

1) Asymptomatic hyperuricemia
2) Acute gouty arthritis
3) Intercritical gout
4) Chronic tophaceous gout

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35
Q

Disease that results in a similar inflammatory reaction as gout, but has crystals that form white chalky friable deposits and do not deposit in mass like tophi

A

Calcium Pyrophosphate Crystal Deposition Disease (Pseudo-Gout)

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36
Q

Small cyst that is almost always located near a joint capsule or a tendon sheath; cyst walls lack true cell lining; fluid within cysts are similar to synovial fluid, however, no connection is made with joints

A

Ganglion

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37
Q

How do ganglions develop?

A

The arise as a result of cystic or myxoid degeneration of connective tissue

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38
Q

How do synovial cysts arise?

A

Caused by herniation of the synovium through a joint capsule or massive enlargment of bursa

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39
Q

Laboratory findings of RA

A

1) Autoantibodies for the Fc portion of autologous IgG

2) Neutrophils and high protein in synovial fluid

40
Q

Morning stiffness that improves with the activity

A

Rheumatoid arthritis

41
Q

Morning stiffness that gets worse with activity

A

Osteoarthritis

42
Q

How does anemia of chronic disease occur?

A

1) Inflammatory disease causes an increased production of IL-6
2) IL-6 stimulates the production of Hepcidin
3) Hepcidin breaks down ferroportin causing decreased Iron, high Ferritin,

43
Q

IgM autoantibody for the Fc portion of IgG

A

Rheumatoid factor

44
Q

What is a complication of RA?

A

1) Anemia of Chronic disease

2) Secondary amyloidosis

45
Q

What are the synovial fluid findings in osteoarthritis?

A

1) Transparent, yellow fluid appears with WBC <2000

46
Q

Acute arthritis gout attack of the first metatarsophalangeal joint

A

Podagra

47
Q

What are the two causes of bone erosion?

A

1) RA

2) Gout

48
Q

What are the synovial fluid findings in RA

A

1) Yellow-green fluid
2) WBC 50% PMNs
4) Findings are associated with an inflammatory response

49
Q

What diseases cause sterile arthritis

A

Reactive arthrisis

  • Reiter syndrome
  • Enteritis-associated Arthritis
50
Q
  • Subchondral cysts
  • Osteophytes causing mushroom shaped margins of the articular surface
  • Bone eburnation
  • Joint mice
A

Osteoarthritis

51
Q

What causes the Erythrocyte sedimentation rate to increase?

A

1) Inflammation
2) Age
3) Anemia
4) Pregnancy (increased fibrinogen)
5) Loss of albumin (nephrotic syndrome)
6) Increased globulins (multiple myeloma)

52
Q

What causes the Erythrocyte sedimentation rate to decrease?

A

1) Polycythemia vera
2) Sickle cell anemia
3) Decreased plasma proteins

53
Q

What are normal findings of synovial fluid?

A

1) Yellow-clear fluid
2) WBC <2000
3) Absence of crystals
4) No blood cells

54
Q

Synovial joint fluid with a WBC >100,000

A

Septic arthritis

55
Q

Appear as negative birefringent needle shaped crystals under red compensated polarized light microscopy

A

Monosodium urate crystals

56
Q

What is a better test for RA: rheumatoid factor or Anti-CCP antibodies?

A

Anti-CCP antibodies

57
Q

Aggregation of organizing fibrin floating in the synovial fluid

A

Rice bodies

58
Q

What is the result of increased TNF in RA

A

Activates the metaloproteases

59
Q

Mesenchymal proliferations that occur in the extraskeletal, non-epithelial tissues of the body

A

Soft Tissue Tumor

60
Q

Well encapsulated soft tissue mass of mature adipocytes; mobile, painless; arising during middle adulthood

A

Lipoma

61
Q

Non-neoplastic lesion that develops in response to trauma/idiopathic; composed of plump reactive fibroblasts (hypercellular, increased mitoses, primitive appearance) ; develop suddenly and grow rapidly

A

Reactive Pseudosarcomatous Proliferation

62
Q

Infiltrative pseudosarcomatous fasciitis; consists of plump fibroblasts with myofibroblasts arranged randomly in intersecting fascicles; abundant mitotic figures; nodular and arises from the dermis, subcutis, or muscle

A

Nodular Fasciitis

63
Q

Reactive pseudosarcomatous with metaplastic bone; develops in adolescence; follows trauma; arises in musculature of the proximal extremities; gross appearance depends on stage

A

Myositis ossificans

64
Q

What are the morphological findings of a myositis ossificans as it changes over time?

A

1) Initially consists of fibroblasts and myofibroblasts as observed in Nodular fascitis
2) Later an intermediate zone that contains osteoblasts is formed
3) The entire lesion eventually ossifies and the intertrabecular spaces become filled

65
Q

Bothersome lesions characterized by nodular or poorly defined broad fasicles of fibroblasts and myofibroblasts surrounded by abundant dense collagen

A

Superficial fibromatosis

66
Q

Rubbery, tough tumor composed of banal well differentiated fibroblasts that do not metastasize; develop extra-abdominally, abdominally, and intra abdominally; large, infiltrative mass that frequently recurs if there is not complete excision

A

Deep Seated Fibromatosis (Dermoid tumor)

67
Q

Cells that contain eccentric eosinophilic granular cytoplasm, contain sacromeres, and stain positive for desmin, MYOD1, and myogenin

A

Rhabdomyoblast

68
Q

What are the three types of rhabdomyosarcomas?

A

1) Embryonal
2) Alveolar
3) Pleomorphic

69
Q

Most common type of rhabdomyosarcoma variant that occurs in children <10, found in nasal cavity, orbit, middle ear, ect

A

Embryonal variant rhabdomyosarcoma

70
Q

What is the chromosomal translocation common in synovial sarcoma

A

1) SYT:SSX fusion (18:X)

71
Q

What are the differences between Duchene and Becker muscular dystrophies?

A

1) Duchene is caused by a deletion of dystropin while Becker is caused by a mutation to dystropin
2) Duchene is more aggressive than Becker
3) Duchene generally has pseudohypertrophy

72
Q

Replacement of musculature with adipose tissue; various muscle fiber sizes, increased internalized nuclei; elevated serum creatinine kinase

A

X-linked muscular dystrophy

73
Q

Most common cause of infectious arthritis

A

N Gonnorrhoeae

74
Q

Sustained involuntary contraction of a group of muscles

A

Myotonia

75
Q

What is the cause of myotonic dystrophy?

A

1) Autosomal dominant expansion of CTG nucleotide on chromosome 19
2) Results in increased of DMPK
3) Anticipation is observed

76
Q

Muscle tissue with presence of ring fibers and dystrophy of the intrafusal fibers of the muscle

A

Myotonic dystrophy

77
Q

Relapsing episode of hypotonic paralysis due to vigorous exercise, cold, or a high carbohydrate meal; may also have myotonia

A

Ion channel myopatheis

78
Q

Abnormalities of the carnitine transport or deficiency of the mitochondrial dehyrgenases

A

Lipid myopathies

79
Q

What are the disorders associated with myopathies that have point mutations in the mitochondrial DNA? Shows maternal inheritance.

A

1) Myoclonic epilepsy with ragged red fibers
2) Leber hereditary optic neuropathy
3) Mitochondrial encephalomyopathy with lactic acidosis and stroke like episodes

80
Q

What are the skin rashes associated with dermatomyositis?

A

1) Purple skin rash on eyelids (heliotrope rash)
2) Malar rash
3) Grutton lesions- scaly lesions on knee and elbow

81
Q

Malar rash with positive anti-Jo-1 antibody; associated with visceral carcinoma

A

Dermatomyositis

82
Q

What are the differences between dermatomyositis and polymyositis?

A

1) Dermatomyositis has cutaneous involvment
2) Dermatomyositis involves perimysial inflammation; Polymyositis involved endomysial inflammation
3) Dermatomyositis involves CD4; Polymyositis involves CD8

83
Q

Inflammation of musculature that begins with involvement of the distal muscles, has asymmetrical presentation, occurs in ages >50; rimmed vacuoles

A

Inclusion body myositis

84
Q

Perifascicular atrophy

A

Dermatomyositis

85
Q

What is exophthalamic ophthalmoplegia? What is it associated with? What are other findings?

A

1) Swelling of the eyelids, edema of the conjunctiva, and diplopia
2) Thyrotoxic myopathy
3) Proximal muscle weakness

86
Q

What drugs cause myopathies?

A

1) Steroids- cause damage to type 2 fibers predominantly
2) Chloroquinine- has vacuoles present within myocytes
3) Statins

87
Q

What are the clinical associations of Myasthenia gravis?

A

1) Thymus hyperplasia/thymoma
2) Ptosis and diplopia
3) Generalized weakness
4) Improvement with anti-acetylcholine esterase
5) Biopsy is unrevealing

88
Q

Commonly a paraneoplastic process (small cell lung carcinoma) that results in proximal muscle weakness and autonomic dysfunction due to autoantibodies that are selective for the Ca channel of the presynapitc neuron of the neuromuscular junction

A

Lambert-Eaton Syndrome

89
Q

What are the different types of superficial fibromatosis?

A

1) Palmar variant - thickening of the palmar fascitis
2) Plantar variant
3) Penile (Peyronie disease)

90
Q

What are the different types of deep fibromatosis?

A

1) Extra-abdominal
2) Abdominal
3) Intra-abdominal

91
Q

Large infiltrative mass that recurs after excision; consists of mature fibroblasts

A

Deep Seated Fibromatosos (desmoid mass)

92
Q

Aggressive tumor that has a herring bone pattern; all degrees of differentiation

A

Fibrosarcoma

93
Q

What is the shape of monosodium urate cyrstals? What is the shape calcium pyrophosphate crystals?

A

1) Needle shape

2) Rhomboid, rod shaped

94
Q

Tadpole or strap cells

A

Rhabdomyosarcoma

95
Q

Sarcoma of retroperitoneum and deep soft tissues of extremities in older adults; common in post radiation

A

Malignant fibrous histiocytoma

96
Q

What is at risk for being damaged in a posterior tibial dislocation?

A

1) Popliteal artery