Path Musculoskeletal 1 & 2 Flashcards

1
Q

All Diseases in these 2 modules

A

1) Behcet’s
2) Sjogren’s Syndrome
3) Reactive Arthritis
4) Temporal Arteritis
5) Osteogenesis Imperfecta
6) Fibrous Dysplasia
7) Paget’s Disease of Bone
8) Osteopetrosis

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2
Q

Chronic disease consisting of inflammatory lesions of multiple systems

A

Behcet’s

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3
Q

Clinical features of Behcet’s

A

a) Oral and Genital Ulcers
b) Ocular Inflammation (hypopyon - pus in anterior chamber)
c) Skin Lesion
d) CNS involvement (paralysis, dementia)

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4
Q

What disease has an association with HLA-B51 (middle aged women)

A

Behcets

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5
Q

Diagnosis of Behcets

A

Complicated combination of clinical findings

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6
Q

Treatment of Behcets

A

Steroids and Immunosuppressive drugs (b/c both deal with inflammation)

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7
Q

Prognosis of Behcets

A

Depends on involvement
- CNS involvement = poor prognosis

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8
Q

M or F get behcets more?

A

Middle aged women

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9
Q

Apthous ulcers

A

canker sores

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10
Q

Females get apthous ulcer more than men (T/F)

A

True

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11
Q

where do apthous ulcers occur

A

Unattached mucosa (not the gingiva)

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12
Q

how do apthous ulcers differ from intraoral herpes?

A

Apthous ulcers - unattached mucosa
Intraoral Herpes - attached mucosa (usually)

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13
Q

Sjogren Syndrome

A

Autoimmune disease affecting salivary, lacrimal and other glands
- Cell-mediated and Humoral immunity involved

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14
Q

What syndrome does Sjogren syndrome lead to?

A

Sicca Syndrome

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15
Q

What is sicca syndrome

A

Hyposalivation (xerostomia)
Hypolacrimation (xerophthalmia)

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16
Q

what can xerophthalmia from hypolacrimation lead to

A

Keratoconjunctivitis sicca (eye damage)

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17
Q

M or F more likely to get sjogren syndrome?

A

Female

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18
Q

Primary Sjogren Syndrome

A

With no other autoimmune diseases

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19
Q

Secondary Sjogren Syndrome

A

In combo with other autoimmune diseases

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20
Q

Oral manifestations of Sjogren Syndrome

A
  • Xerostomia (lymphocytes destroyed salivary glands)
  • Sandy mouth feeling
  • cracked lips
  • Papillary atrophy of tongue (beefy red tongue)
  • higher rates of caries, periodontal disease, Candidiasis
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21
Q

Extraoral manifestations of Sjogrens Syndrome

A

A) Enlarged Parotid Glands (50% of pts)
B) Xerophthalmia (keratoconjuctivitis)
- Burning, itching, photophobia, damage to cornea
C) Raynaud Phenomenon
D) Others
- Myalgia (Pain),
- Arthalgia (Joint Stiffness)
- Chronic Fatigue

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22
Q

What is Raynaud Phenomenon in Sjogren Syndrome?

A

Transient vasocontriciton of digits leading to:
a) Reduced blood flow
b) Pallor
c) Pain

Induced by:
a) Cold
b) Emotional Stress

Seen in other autoimmune diseases too

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23
Q

Laboratory Findings

A

A) Rheumatoid Factor
- IgM to IgG

B) Sjogren Specific Abs
- Anti-SS A (Anti-Ro)
- Anti-SS N (Anti-La)

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24
Q

Diagnosis of Sjogren Syndrome

A

ID’ing combo of signs and symptoms of disease

May include:
- Biopsy of Minor salivary glands
- Sialography (x-ray of salivary gland or ducts) FRUIT LAIDEN BRANCHLESS TREE with technitium-99 pertechnetate

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25
Q

Treatment of Sjogren Syndrome

A

a) saliva/tear substitutes (usually not enough)
b)Meds:
- Steroid/Immunosuppressive Drugs
c) Oral Hygiene (Fluoride)

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26
Q

Prognosis of Sjogren Syndrome

A

heightened risk of other autoimmune diseases and LYMPHOMA

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27
Q

Why does sjogren syndrome lead to higher risk of lymphoma

A

Very high turnover of lymphocytes (autoimmune) so lots of synthesis and mitosis which equals higher change of mutations

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28
Q

Reactive Arthritis

A

Constellation comprised of
- Conjunctivitis
- Urethritis
- Arthritis

*May not have all 3 findings

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29
Q

Trick to remember Reactive arthritis

A

Cant see, pee, or climb a tree

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30
Q

Reactive arthritis happens after patient has what

A

Venereal or GI infection

31
Q

Time frame of Reactive Arthritis

A

1-4 weeks

32
Q

M or F predilection in Reactive Arthritis

A

Male 15:1

33
Q

Conjunctivitis in RA

A

Mild

34
Q

Urethritis in RA

A

Usually develops later than other two

35
Q

Arthritis in RA

A
  • Lower extremity
  • Radiographically visible periosteal proliferation of bone
36
Q

Oral lesions in RA

A
  • Apthae-like
  • Geographic tongue-like
37
Q

What is HLA-B27 associated with?

A

Reactive Arthritis (Young men) and Ankylosing Spondylitis (inflammation of spine and joints)

38
Q

Temporal Arteritis

A

Autoimmune Vasculitis

39
Q

Temporal Arteritis has Predilection for

A

Large to small sized arteries of the head and neck
- other sites including aorta also possible

40
Q

Morphology of Temporal Arteritis

A
  • Granulomoas with multinucleate giant cells in 75% of lesions
  • Reduction of lumen diameter = ischemia risk
41
Q

Clinical Features of Temporal Arteritis

A
  • Nodular thickening at sites of involvement
  • Fever, Fatigue, weight loss, Facial pain, headache
  • TEMPORAL ARTERY MAY BE PAINFUL TO PALPATION
  • Ocular involvement may lead to BLINDNESS
  • NECROSIS OF TONGUE (rare)
42
Q

Treatment for Temporal Arteritis

A

Responds well to Cortical Steroids

43
Q

Osteogenesis Imperfecta (general)

A

A*) Defect in gene for Type 1 Collagen (Heterozygosity in 1 of 2 genes) = SOFT BONES
- COL1A1 on Chromosome 17
- COL1A2 on Chromosome 7

  • AD or AR (AD most common)
44
Q

Extraoral Clinical Findings of Osteo Imperfecta

A

a) Bones Fracture Easily
- Exuberant Callous formation in healing
b) Blue Sclera
c) Joint Hypermobility

45
Q

Introoral Clinical Findings of Osteo Imperfecta

A

a) Opalescent appearing teeth at eruption
- poorly formed dentin
- enamel easily chips off
b) Primary teeth more commonly affected than permanent

46
Q

Fibrous Dysplasia (FD)

A

Developmental tumor like replacement of normal bone by proliferation of cellular fibrous CT with admixture of irregular hard tissue
- growth is diffuse

47
Q

FD Radiographic Findings

A

Ground Glass
- Disorganized, poorly calcified bone

48
Q

What causes FD

A

GNAS1 Mutation

49
Q

Onset of FD in development correlation with Tissues affected?

A

Early in dev. = more tissues (polyostitic)
Later in Dev. = less tissues (monostitic)

50
Q

Variants of FD

A

a) monostitic
b) Craniofacial (perhaps artificial)
c) Polyostitic (2 or more)
- Jaffe-Lichtenstein Syndrome
- McCune-Albright Syndrome

51
Q

Jaffe-Lichtenstein Syndrome

A

a) Polystotic FD
b) Cafe au lait spots (jagged borders, borders of maine)

52
Q

McCune-Albright Syndrome (M for Multiple)

A

a)Polyostotic FD
b) Cafe au lait spots
c) Multiple endocrinopathies
- Precocious (early) puberty
- Pituitary adenoma
- Hyperthyroidism

53
Q

FD Treatment

A

a) surgery (shave down for cosmetics
b) Bisphosphonates (Polyostotic)

54
Q

Why is radiation avoided with FD?

A

Leads to osteosarcoma

55
Q

Paget’s Disease of Bone

A

Metabolic disease - Abnormally high rate of bone turnover

56
Q

Diff names for Pagets disease of bone

A

a) Osteitis deformans
b) Leontiasis osseas

57
Q

M or F predilection

A

Adult male

58
Q

Most common sites for Paget’s

A

a) Lumbar
b) Pelvis
c) Femur
d) Skull
- Maxilla > Mandible
- Constriction of Foramina

59
Q

What happens to bone in Paget’s

A

Bone enlargement

60
Q

What does bone enlargement lead to clinically?

A

a) pinching of nerves
- Headaches, Dizziness, and Deafness
b) Enlargement of Skull
- hat doesn’t fit anymore
c) Teeth Discplacement
- Dentures don’t fit anymore

61
Q

Paget’s Radiographically

A

COTTON WOOL mixed radiolucency-radiopacity

62
Q

Laboratory features of Paget’s

A

Elevated ALKALINE PHOSPHATASE

63
Q

Osteopetrosis

A

Group of rare genetic disorders characterized by defective osteoclast and resultant stone-like bone
- deposition of bone on bone
- infantile is most sever and lethal

64
Q

Clinical Finding of Osteopetrosis

A

a) Bone is weak and fracture prone
b) Constricted Foramina (Cranial nerve impingement)
- Deafness, Blindness
c) Extramedullary hematopoiesis
- Reduced marrow space –> Granulocytopenia –> Infection prone
- Hepatosplenomegaly

65
Q

Behcet’s Simple

A
  • Chronic
  • Inflammatory Lesions
  • HLA-B51
66
Q

Sjogren Syndrome Simple

A
  • Autoimmune
  • Targets Glands (dry mouth, dry eyes)
  • Beefy Red Dry Tongue
  • Raynaud Phenomenon
  • Fruit Laden Branchless Tree (Sialography)
  • High Risk for Lymphoma
  • Rheumatoid Factor, Sjogren Specific Abs
67
Q

Reactive Arthritis Simple

A
  • Cant see, pee, or climb tree
  • HLA-B27 (ANKYLOSING SPONDYLITIS inflamm. spine & joints)
68
Q

Temporal Arteritis Simple

A
  • Autoimmune vasculitis
  • Arteries of Head and Neck
  • Granulomas
  • Lumen diameter reduction –> ischemic risk
  • Temporal artery pain on palpation
  • Blindness
69
Q

Osteogenesis Imperfecta Simple

A
  • Defect in gene for Type1 collagen
  • COL1A1 = 17, COL1A2 = 7
  • Soft Bones
  • Opalescent appearing teeth at eruption
70
Q

Fibrous Dyplasia Simple

A
  • Developmental (Early or Late) Tumor replaces normal bone
  • Diffuse Growth
  • Ground Glass
  • GNAS1 mutation
  • Cafe au Lait Spots
71
Q

Paget’s Disease of Bone Simple

A
  • High rate of bone turnover
  • Larger Bones
  • Nerve pinch, Hat and Dentures dont fit
  • Cotton Wool Radiograph
  • Elevated Alkaline Phosphatase
72
Q

Osteopetrosis Simple

A
  • Genetic disorders - defective osteoclast –> stone like bone
  • Bone fracture prone
  • Constricted foramina
  • Extramedullary hematopoiesis (Hepatosplenomegaly)
73
Q
A