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Pod Med I > 11) Connective Tissue Diseases > Flashcards

11) Connective Tissue Diseases Flashcards

1
Q

Connective Tissue Diseases

A
  • Systemic Lupus Erythematosis
  • Scleroderma (Progressive Systemic Sclerosis)
  • Dermatomyositis/Polymyositis
  • Mixed Connective Tissue Disease
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2
Q

Lupus (SLE) demographic

A
  • 90% female
  • Most between ages 14 and 45
  • Most common and severe in black females
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3
Q

Signs/symptoms of SLE

A
  • Fever, fatigue, weight loss
  • Joint pain
  • Malar rash
  • Pleuritis, pericarditis, endocarditis
  • Nonbacterial verrucous
  • Raynaud’s phenomenon
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4
Q

SLE kidney effects

A
  • Wire loop lesions in the kidney with immune complex deposition
  • Death from renal failure and infections
  • False positives syphilis tests (RPR/VDRL)
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5
Q

Lab tests for SLE detect the presence of

A
  • Antinuclear antibodies (ANA): sensitive, but not specific for SLE
  • Antibodies to double stranded DNA (anti-ds DNA) very specific
  • Anti-Smith antibodies (anti-Sm): very specific
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6
Q

Procainamide, INH, hydralazine effects in SLE

A
  • Can produce an SLE-like syndrome

- Commonly reversible

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7
Q

Wire loop erythematosis (SLE)

A
  • Wire loop lesions in the kidney with immune complex deposition
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8
Q

Lupus meaning

A
  • Latin for wolf

- A reference to the malar rash (on cheeks) causing wolflike facies (30-50%)

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9
Q

SLE differentiating features

A
  • Glomerulonephirtis
  • Photosensitivity
  • Characteristic skin rashes
  • CNS disease
  • Coombs positive hemolytic anemia
  • Leukopenia
  • Thrombocytopenia
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10
Q

Butterfly-malar (SLE butterfly rash)

A
  • A confluent erythematous eruption
  • Distribution over the bridge of the nose and cheeks
  • Rash is also present around the mouth and on the forehead
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11
Q

SLE interarticular dermatitis

A
  • Erythematous rash over the dorsum of her hands and fingers
  • Band of erythema across proximal phalanges (sparing the joints)
  • Periungual erythema
  • Proximal interphalangeal joint swelling
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12
Q

SLE hand and cutaneous lesions

A
  • Erythematous lesions and telangiectasias on fingertips and palmar eminences
  • These vascular lesions blanch with pressure
  • Similar lesions are seen in other related rheumatic diseases such as mixed connective tissue disease, rheumatoid arthritis, and dermatomyositis
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13
Q

Jaccoud’s Arthropathy associated with Systemic Lupus Erythematosus

A
  • Ulnar deviation, metacarpophalangeal subluxation, and swan-neck deformities
  • Diffuse soft-tissue swelling
  • Reducible deformity
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14
Q

Cause of Jaccoud’s Arthropathy

A
  • Tendon laxity (not bony destruction)

- May also be seen in post-rheumatic fever arthritis

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15
Q

SLE Libman-Sacks endocarditis (gross specimen)

A
  • Non-bacterial endocarditis
  • Mitral valve typically affected
  • Vegetations, small
    strands of fibrin, neutrophils,
    lymphocytes and histiocytes
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16
Q

Libman-Sacks endocarditis (pictomicrograph)

A
  • Verruca consists of compact fibrin (fibrinoid), debris, and a few inflammatory cells (chiefly histiocytes)
  • Connected by a base of granulation tissue to the endocardium and underlying myocardium
  • Verruca contains a mass of aggregated hematoxylin bodies (hematoxylin-eosin, medium power)
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17
Q

Scleroderma

A
  • Cyanotic phase of Raynaud’s phenomenon in the hands

- Telangiectasias and palmar erythema may also be present

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18
Q

Clinical symptoms of SLE

A
  • Fever
  • Fatigue
  • Arthralgias, myalgias
  • Morning stiffness
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19
Q

SLE high yield facts

A
  • ANA positive in > 98% of cases (ANAs frequently occur in normal people as well)
  • Anti-DNA presence associated with glomerulonephritis
  • Compliments C3 and/or C4 often low
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20
Q

SLE treatment (clinical therapeutics)

A
  • Rest, NSAIDS, corticosteroids, hydrocloroquine, and avoiding sun exposure
  • More severe disease calls for azothioprine methotrexate, and cyclophosphamide
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21
Q

When to use a biologic in SLE

A
  • Active or corticosteroid-dependent SLE
  • Treated with hydroxychloroquine
  • Minimum of 2 successive immunosuppressive therapies such as methotrexate, cyclophosphamide, azathioprine, or mycophenolate mofetil
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22
Q

Scleroderma

A
  • Excessive fibrosis and collagen deposition throughout the body
  • 75% are female
  • Commonly sclerosis of the skin but also cardiovascular and GI systems and kidney
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23
Q

Two major categories of scleroderma

A
  • Diffuse scleroderma

- CREST syndrome

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24
Q

Diffuse scleroderma

A
  • Widespread skin involvement
  • Rapid progression
  • Early visceral involvement
  • Associated with anti-Scl-70 antibody
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25
Q

CREST syndrome

A
  • Calcinosis
  • Raynaud’s phenomenon
  • Esophageal dysmotility
  • Sclerodactyly (localized tightness/ thickening of skin)
  • Telangectasia
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26
Q

Scleroderma high yield facts

A
  • Limited skin involvement often confined to the fingers and face
  • More benign clinical course
  • Associated with anticentromere antibody
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27
Q

Dermatomyositis and scleroderma: periungual involvement

A
  • Changes of nailfold capillary patterns in certain patients with systemic sclerosis, dermatomyositis, mixed connective tissue disease, and Raynaud’s syndrome
  • Changes are characterized by loss of (drop-out) nailfold capillary loops that surround the remaining, enlarged dilated capillaries
28
Q

Raynaud’s Phenomenon hand blanching

A
  • The marked pallor of the fourth and fifth digits
  • Vasospastic changes are common in systemic sclerosis but may also occur in rheumatoid arthritis, systemic lupus erythematosus, and idiopathic Raynaud’s disease
29
Q

Scleroderma skin induration

A
  • Induration of the skin over the fingers, hands, and wrists
  • Loss of skin folds
  • Fingers appear shiny and slightly puffy
30
Q

Acrosclerosis (sclerodactyly) in scleroderma

A
  • Flexion contractures of the fingers secondary to a tightened indurated skin
  • Characteristic of systemic sclerosis
  • Skin changes proximal to the metacarpophalangeal joints are more specific for systemic sclerosis than changes only in the fingers
  • Areas of increased and decreased pigmentation
31
Q

Digital pitting scars in scleroderma

A
  • Pulps of the fingers

- Secondary to chronic microvascular disease

32
Q

“Mauskopf” (mousehead)

A
  • Facial changes in scleroderma (systemic sclerosis)
  • Drawn pursed lips
  • Shiny skin over the cheeks and forehead
  • Atrophy of muscles in the temple, face, and neck
33
Q

Raynaud’s Phenomenon

A
  • Reversible skin color change (white to blue to red)
  • Due to vasospasm
  • Induced by cold or emotion
34
Q

Raynaud’s phenomenon in hand arteriogram

A
  • Irregular narrowing and occlusion of the digital arteries
  • Proximal vessels, arcades, and metacarpal vessels are widely patent
  • May be reversible depending on the extent of the underlying condition
35
Q

Abnormal motility in esophagus associated with scleroderma

A
  • Decreased peristaltic activity and dilatation of the esophagus
  • Imaging conducted with patients in the prone position, and usually a right anterior oblique view is obtained
36
Q

Duodenum dilatation in scleroderma

A
  • 2nd/3rd segments of the duodenum are distended
  • Relative lack of peristaltic activity
  • May result in the barium remaining in the same location for several minutes
  • Changes are similar to those in the esophagus
37
Q

A barium-enema study (roentgenogram) of a patient with systemic sclerosis shows

A
  • Multiple wide-mouthed diverticula of the colon
  • Diverticula characteristically have a broad base or neck
  • Usually remain asymptomatic
38
Q

Things to avoid in managing Raynaud’s Phenomenon

A
  • Skin exposure to cold
  • Tobacco use
  • Precipitating events for vasospasm (stressful events, use of vibrating tools)
39
Q

Treatment of Raynaud’s Phenomenon

A
  • Vasodilator drugs
  • Temperature biofeedback
  • Treat associated disorders
  • Treat underlying disorders
40
Q

Raynaud’s Phenomenon standard medical therapies

A
  • Calcium channel blockers
  • ACE inhibitors
  • Losartan
  • Fluoxetine
41
Q

Sildenafil

A
  • Used in Raynaud’s treatment
  • 25 mg 3x daily
  • Increase to 50 mg 3x daily if necessary
42
Q

Intravenous prostanoid (usually iloprost)

A
  • Used in Raynaud’s

- Up to a frequency of ever 6-8 weeks if necessary

43
Q

Dermatomyositis/Polymyositis

A
  • Idiopathic autoimmune disease that affects the proximal limb muscles with inflammation
  • Onset usually insidious
44
Q

Dermatomyositis/Polymyositis symptoms

A
  • Proximal muscle weakness

- Difficulty ascending stairs, arising from a car seat, or rising from hands and knees

45
Q

Physical examination of Dermatomyositis/Polymyositis patient shows

A
  • Diffuse symmetrical upper arm weakness
  • Difficulty and abdominal curl or rising from a chair without the use of hands
  • Usually painless, but can be painful in some instances
  • Gait may be slow and wide-based
46
Q

Clinical presentations of Dermatomyositis/Polymyositis

A
  • Proximal and symmetrical muscle weakness
47
Q

Dermatomyositis presents with

A
  • Periorbital lilac rash

- Gottron papules on knuckles

48
Q

DM/PM diagnostic studies

A
  • Elevated serum creatinine kinase (CK), or lactate dehydrogenase (LDH)
  • Characteristic electromyographic abnormalities
  • Muscle biopsy is imperative in making an accurate diagnosis
  • ANA may be present
49
Q

DM/PM clinical therapeutics

A
  • Initial treatment = moderate dose corticosteroids
  • Methotrexate or azathioprine is used early to avoid steroid side effects
  • Exercises after the inflammation is controlled restore some strength and range of motion
50
Q

Proposed diagnostic criteria for polymyositis & dermatomyositis

A
  • Symmetric proximal muscle weakness
  • Elevated muscle enzymes (CPK, Aldolase, Transaminases, LDH)
  • Myopathic EMG abnormalities
  • Typical changes on muscle bodies
  • Typical rash of dermatomyositis
51
Q

Polymyositis diagnosed as definite with

A
  • 4/5 criteria

- Probable when 3/5

52
Q

Dermatomyositis diagnosed as definite with

A
  • Rash +3/4 criteria

- Probable with rash +2/4

53
Q

Polymyositis classification

A

I. Adult polymyositis
II. Adult dermatomyositis
III. Inflammatory myositis associated with cancer
IV. Childhood dermatomyositis or polymyositis
V. Myositis associated with connective tissue disease

54
Q

Drug-induced myopathy (polymyositis differential)

A
  • Alcohol
  • Corticosteroids
  • AZT
  • DDI
  • Clofibrate
  • Lovastatin
  • Penicillamine
  • Chloroquine
  • Emetine
55
Q

Endocrine myopathies (polymyositis differential)

A
  • Cushing’s Disease
  • Adrenal insufficiency
  • Acromegaly
  • Hyperthyroidism
  • Hypothyroidism
  • Hyperparathyroidism
  • Hypoparathyroidism
56
Q

Differential diagnosis for polymyositis

A
  • Drug induced myopathy
  • Endocrine myopathies
  • Polymyalgia Rheumatica (and other CT diseases)
  • Infectious myositis
  • Neurologic disorders
  • Electrolyte disturbances
  • Metabolic myopathies
  • Other inflammatory myopathies
57
Q

Polymyalgia Rheumatica and other CT diseases (polymyositis differential)

A
  • RA
  • SLE
  • Scleroderma
  • Vasculitis
58
Q

Infectious myositis (polymyositis differential)

A
  • Viral
  • Pyomyositis
  • Toxoplasmosis
  • Trichinosis
59
Q

Neurologic disorders (polymyositis differential)

A
  • Muscular dystrophies
  • Myotonia
  • Myasthenia Gravis
  • Amyotrophic Lateral Sclerosis
  • Eaton-Lambert Syndrome
60
Q

Electrolyte distrubances (polymyositis differential)

A
  • Hypokalemia
  • Hypomagnesemia
  • Hypophosphatemia
  • Hypocalcemia
61
Q

Metabolic myopathies (polymyositis differential)

A
  • Inherited or acquired
62
Q

Other inflammatory myopathies (polymyositis differential)

A
  • Inclusion Body Myositis
  • Eosinophilia Myalgia Syndrome
  • Eosinophilic Fasciitis
  • Sarcoid Myopathy
  • Carcinomatous Neuromyopathy
63
Q

Dermatomyositis: acute myositis (photomicrograph)

A
  • Lymphocytes and histiocytes infiltrate muscle fibers
  • Some SkM fibers appear normal, necrotic, and fragmented/invaded by macrophages
  • Muscle changes in polymyositis are identical to those found in dermatomyositis (hematoxylin-eosin, medium power)
64
Q

Mixed connective tissue disease

A
  • An overlap of scleroderma, polymyositis, and lupus
65
Q

Mixed connective tissue disease is associated with

A
  • Very high titers of anti-RNP antibodies (antibodies to ribonucleoprotein)
  • Can also be found in other MCTD’s
66
Q

Mixed Connective Tissue Disorder (MCTD) is actually

A
  • Classification V of polymyositis

Pod Med I (3 decks)

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