Connective Tissue Pathology Flashcards

1
Q

where is connective tissue found

A
tendons
blood
cartilage
bone
adipose tissue 
lymphatic tissue
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2
Q

what type of disorder is Marfan Syndrom

A

autosomal dominant disorder

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3
Q

Organ abnormalities of Marfan Disorder

A

organ abnormalities

  • cardiovascular
  • aortic stenosis
  • mitral valve prolapse
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4
Q

what is the most important defect of Marfan syndrome?

A

ascending or descending aorta

there is a high incidence of dissecting aneurysm

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5
Q

Characteristics of Marfan syndrome

A

short torso long limbs

arachnodactyly
long skull

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6
Q

skeletal abnormalities of Marfan syndrome

A
  • pectus excavatum or carinatum
  • weak ligaments, tendon, joint capsules
  • scoliosis
  • hyperflexibility
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7
Q

eye abnormalities of Marfan syndrome

A
  • dislocation of the lens
  • severe myopia due to the elongation of eye
  • retinal displacement
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8
Q

Ehlers-Danlos Syndrome

A

a group of inherited connective tissue disorders

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9
Q

6 types of Ehlers-Danlos Syndrome

A
  • Hyper-elasticity & fragile of the skin
  • Joint hypermobility
  • Bleeding diathesis
  • Severe kyphoscoliosis
  • Blindness from retinal hemorrhage
  • Bladder rupture
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10
Q

3 types of Neurofibromatosis

A
  • Type 1 – von Recklinghausen Disease (NF1)
  • Type II – Central Neurofibromatosis (NF2)
  • Type III – Schwannomatosis
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11
Q

most common autosomal dominant disorder

A

Type 1 – von Recklinghausen Disease (NF1)

1 in 3,500 people of all races

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12
Q

what are Type 1 – von Recklinghausen Disease (NF1) disfiguring neurofibromas and cade-au-lait spots

A
  • benign tumors of peripheral nerve or schwann cells

- areas of dark skin pigmentation (95% of people display six or more spots)

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13
Q

what percentage of people develop cutaneous and subcutaneous neurofibromas in late childhood and adolescence

A

90%

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14
Q

Type II – Central Neurofibromatosis (NF2)

A
  • bilateral 8th cranial nerve tumor
  • meningiomas
  • gliomas
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15
Q

Type III – Schwannomatosis

A

occurs in ~1 in 40,000 births

symptoms usually first appear in adult hoood

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16
Q

What is Type III – Schwannomatosis characterized by?

A
  • Multiple nerve sheath tumors (schwannomas) and associated pain
  • Symptoms limited to one area of the body
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17
Q

what type of disorder is Mixed Connective Tissue Diseases (MCTD)

A

autoimmune disorder

18
Q

Mixed Connective Tissue Diseases (MCTD) is a combination of what diseases?

A

SLE
(butterfly rash, Raynaud’s, arthraglia)

Systemic Sclerosis
(swollen hands, esophageal hypomotility, pulmonary fibrosis)

Dermatomyositis & Polymyositis
(Proximal muscle weakness with or without soreness)

19
Q

Treatment of Mixed Connective Tissue Diseases (MCTD

A

corticosteroids

20
Q

Mixed Connective Tissue Diseases (MCTD) multi-organ involvement

A
  • Lungs, hearts, kidneys
  • Lymphadenopathy
  • GI disorders
  • Sjogren’s Syndrome
21
Q

Mixed Connective Tissue Diseases (MCTD) Neuro involvement

A
  • Peripheral neuropathies
  • Aseptic meningitis
  • Cerebral infarct/hemorrhage (OCCUR IN 10% OF PPL)
  • Trigeminal sensory neuropathy
22
Q

Polymyalgia Rheumatica (PMR) symptoms

A

diffuse pain & stiffness in shoulder and pelvic girdle

23
Q

Polymyalgia Rheumatica (PMR) more likely to occur in women or men

A

women - 2x incidence

24
Q

Chief complaint Polymyalgia Rheumatica (PMR)

A

Difficulty with ADL & IADL for no identifiable reason

  • rolling in bed
  • trouble standing up
  • trouble on stairs
  • trouble w/ dressing because of shoulder pain
25
Q

Clinical Presentation of Polymyalgia Rheumatica (PMR)

A
  • Starts unilaterally - then bilateral and symmetric
  • joint synovitis (wrist & MCP)
  • diffuse distal extremity edema
  • carpal tunnel syndrome
  • tenosynovitis
26
Q

Diagnose & Treatment of Polymyalgia Rheumatica (PMR)

A

Rapid response to prednisone within one week

27
Q

Prednisone Caveats in PMR

A
Weight gain 
Mood swings
Cataracts
Glaucoma 
DM
Easy bruising 
Cushing syndrome 
Osteoporosis
28
Q

PRM Cautions

A

Up to 20% develop giant cell arteritis

29
Q

Myofascial Pain Dysfunction

A

local or regional trigger points within a muscle

hyperirritable taut muscle

palpation can lead to local or referred pain

30
Q

active trigger points

A

pain at rest with activity of specific muscle

31
Q

latent trigger point

A
  • no pain but there is restricted and/or weakness of muscle

- increased muscle tension and shortening

32
Q

satellite trigger point

A

one that develops in a different part of the muscle in different muscles within the referred pain pattern

33
Q

what causes trigger points

A
  • overload or stretching
  • direct impact
  • posture
  • psychological stress
  • repetitive physical stress
  • structural abnormalities
  • overwork fatigue
  • radiculopathy
  • indirect activation
  • visceral disease
34
Q

pathogenesis of Myofascial Pain Dysfunction

A

dysfunction of motor end plates

  • greater spontaneous EMG activity
  • sensitized nociceptors associated with dysfunctional MEPP (radiation to dorsal horn)
35
Q

Theory of “taut band”

A

Trauma = release of excessive amounts of Ca2+ from the sarcoplasmic reticulum

The contraction is sustained = decreased blood flow

=Decreased ATP production
=Anaerobic metabolism

36
Q

clinical presentation of Myofascial Pain Dysfunction

A

a palpable, taut, painful band within a muscle with characteristic and reproducible referred pain in response to sustained pressure

37
Q

Clinical Management of Myofascial Pain Dysfunction

A
injection (dry needling)
Cryotherapy 
Ultrasound
Sustained pressure
Fluoromethane
Post-isometric stretch
38
Q

Fibromyalgia

A

A chronic, system neuroendocrine condition with specific tender musculoskeletal points

39
Q

Fibromyalgia Systemic dysregulation

A
  • Neurologic
  • Immunologic
  • Endocrine
  • Enteric
40
Q

Trigger Points (MPS)

A

-local tenderness, taut band, local twitch response, jump sign

single or multiple

occurs in skeletal muscle

may cause specific referred pain pattern

41
Q

Tender Points (FMS)

A
  • local tenderness
  • occur in specific locations that symmetrically located
  • do not cause referred pain
42
Q

Fibromyalgia Tender Point

A

18 points

-bilateral & symmetrical

diagnosis of FMS usually made if 11 or more tender points are located