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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
CREST syndrome
- Calcinosis - Raynaud’s phenomenon - Esophageal dysmotility - Sclerodactyly (localized tightness/ thickening of skin) - Telangectasia
26
Scleroderma high yield facts
- Limited skin involvement often confined to the fingers and face - More benign clinical course - Associated with anticentromere antibody
27
Dermatomyositis and scleroderma: periungual involvement
- 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
Raynaud's Phenomenon hand blanching
- 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
Scleroderma skin induration
- Induration of the skin over the fingers, hands, and wrists - Loss of skin folds - Fingers appear shiny and slightly puffy
30
Acrosclerosis (sclerodactyly) in scleroderma
- 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
Digital pitting scars in scleroderma
- Pulps of the fingers | - Secondary to chronic microvascular disease
32
"Mauskopf" (mousehead)
- 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
Raynaud's Phenomenon
- Reversible skin color change (white to blue to red) - Due to vasospasm - Induced by cold or emotion
34
Raynaud's phenomenon in hand arteriogram
- 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
Abnormal motility in esophagus associated with scleroderma
- 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
Duodenum dilatation in scleroderma
- 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
A barium-enema study (roentgenogram) of a patient with systemic sclerosis shows
- Multiple wide-mouthed diverticula of the colon - Diverticula characteristically have a broad base or neck - Usually remain asymptomatic
38
Things to avoid in managing Raynaud's Phenomenon
- Skin exposure to cold - Tobacco use - Precipitating events for vasospasm (stressful events, use of vibrating tools)
39
Treatment of Raynaud's Phenomenon
- Vasodilator drugs - Temperature biofeedback - Treat associated disorders - Treat underlying disorders
40
Raynaud's Phenomenon standard medical therapies
- Calcium channel blockers - ACE inhibitors - Losartan - Fluoxetine
41
Sildenafil
- Used in Raynaud's treatment - 25 mg 3x daily - Increase to 50 mg 3x daily if necessary
42
Intravenous prostanoid (usually iloprost)
- Used in Raynaud's | - Up to a frequency of ever 6-8 weeks if necessary
43
Dermatomyositis/Polymyositis
- Idiopathic autoimmune disease that affects the proximal limb muscles with inflammation - Onset usually insidious
44
Dermatomyositis/Polymyositis symptoms
- Proximal muscle weakness | - Difficulty ascending stairs, arising from a car seat, or rising from hands and knees
45
Physical examination of Dermatomyositis/Polymyositis patient shows
- 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
Clinical presentations of Dermatomyositis/Polymyositis
- Proximal and symmetrical muscle weakness
47
Dermatomyositis presents with
- Periorbital lilac rash | - Gottron papules on knuckles
48
DM/PM diagnostic studies
- 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
DM/PM clinical therapeutics
- 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
Proposed diagnostic criteria for polymyositis & dermatomyositis
- Symmetric proximal muscle weakness - Elevated muscle enzymes (CPK, Aldolase, Transaminases, LDH) - Myopathic EMG abnormalities - Typical changes on muscle bodies - Typical rash of dermatomyositis
51
Polymyositis diagnosed as definite with
- 4/5 criteria | - Probable when 3/5
52
Dermatomyositis diagnosed as definite with
- Rash +3/4 criteria | - Probable with rash +2/4
53
Polymyositis classification
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
Drug-induced myopathy (polymyositis differential)
- Alcohol - Corticosteroids - AZT - DDI - Clofibrate - Lovastatin - Penicillamine - Chloroquine - Emetine
55
Endocrine myopathies (polymyositis differential)
- Cushing's Disease - Adrenal insufficiency - Acromegaly - Hyperthyroidism - Hypothyroidism - Hyperparathyroidism - Hypoparathyroidism
56
Differential diagnosis for polymyositis
- Drug induced myopathy - Endocrine myopathies - Polymyalgia Rheumatica (and other CT diseases) - Infectious myositis - Neurologic disorders - Electrolyte disturbances - Metabolic myopathies - Other inflammatory myopathies
57
Polymyalgia Rheumatica and other CT diseases (polymyositis differential)
- RA - SLE - Scleroderma - Vasculitis
58
Infectious myositis (polymyositis differential)
- Viral - Pyomyositis - Toxoplasmosis - Trichinosis
59
Neurologic disorders (polymyositis differential)
- Muscular dystrophies - Myotonia - Myasthenia Gravis - Amyotrophic Lateral Sclerosis - Eaton-Lambert Syndrome
60
Electrolyte distrubances (polymyositis differential)
- Hypokalemia - Hypomagnesemia - Hypophosphatemia - Hypocalcemia
61
Metabolic myopathies (polymyositis differential)
- Inherited or acquired
62
Other inflammatory myopathies (polymyositis differential)
- Inclusion Body Myositis - Eosinophilia Myalgia Syndrome - Eosinophilic Fasciitis - Sarcoid Myopathy - Carcinomatous Neuromyopathy
63
Dermatomyositis: acute myositis (photomicrograph)
- 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
Mixed connective tissue disease
- An overlap of scleroderma, polymyositis, and lupus
65
Mixed connective tissue disease is associated with
- Very high titers of anti-RNP antibodies (antibodies to ribonucleoprotein) - Can also be found in other MCTD’s
66
Mixed Connective Tissue Disorder (MCTD) is actually
- Classification V of polymyositis

Pod Med I (3 decks)

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