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Cuccurullo Book > Ch 3 - Rheumatology: Rheumatoid Diseases > Flashcards

Ch 3 - Rheumatology: Rheumatoid Diseases Flashcards

1
Q

What is SLE?

A

Multisystemic, autoimmune disease that affects every organ in the body

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2
Q

What is required for classification of SLE?

A

Positive for any 4 of 11 ACR classification criteria

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3
Q

What is the classification criteria for SLE?

A 
DOPAMINE RASH: Discoid rash
Oral ulcers
Photosensitivity
Arthritis (nonerosive)
Malar (butterfly) rash
Immunologic disorder
Neurologic disorder
Renal disorder
Abnormal ANA titer
Serositis
Hematologic disorder
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4
Q

Describe malar rash.

A

Rash of the malar eminences that spares nasolabial folds

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5
Q

Describe discoid rash.

A

Raised erythematous patches with keratotic scaling

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6
Q

What is affected in serositis of SLE?

A

Pleuritis or pericarditis

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7
Q

What is the MC cardiac event of SLE?

A

Pericarditis

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8
Q

What is affected in renal disorders of SLE?

A

Proteinuria or cellular casts

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9
Q

What neurologic disorders are seen in SLE?

A

Seizure or psychosis

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10
Q

What hematologic disorders are seen in SLE?

A

Hemolytic anemia
Leukopenia
Thrombocytopenia
Lymphopenia

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11
Q

Describe the arthritis of SLE.

A 
– Small joints of the hands, wrist, and knees – Symmetric
– Migratory, chronic, nonerosive 
– Soft-tissue swelling 
– Subcutaneous nodules 
– Jaccoud’s arthritis
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12
Q

What is Jaccoud’s arthritis?

A
  • Nonerosive deforming arthritis

* Ulnar deviations of the fingers and subluxations that are reversible early

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13
Q

What is seen on lab work in SLE?

A
  • Dec C3 and C4
  • Ds-DNA
  • Anti-SM
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14
Q

What is scleroderma?

A

Progressive chronic multisystem disease causing fibrosis-like changes in the skin and epithelial tissues of affected organs

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15
Q

How is scleroderma classified?

A

By degree of skin thickening

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16
Q

What are subtypes scleroderma?

A

Diffuse cutaneous
Limited cutaneous
Localized

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17
Q

Describe features of Diffuse cutaneous scleroderma.

A

Affects Heart, lung, GI, kidney
Rapid onset after Raynaud’s phenomenon
Variable course—poor prognosis

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18
Q

What is seen on lab work in Diffuse cutaneous scleroderma?

A

ANA(+)

Anti-centromere antibody (–)

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19
Q

Describe features of Limited cutaneous scleroderma.

A

Progression after Raynaud’s phenomenon

Good prognosis

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20
Q

What is seen on lab work in Limited cutaneous scleroderma?

A

Anti-centromere antibody (+)

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21
Q

Describe features of Localized scleroderma.

A

Morphea

Linear scleroderma

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22
Q

What is Raynaud’s phenomenon?

A

Vasospasm of the muscular digital arteries that can lead to ischemia and ulceration of the fingertips

23
Q

What can trigger Raynaud’s phenomenon?

A

Cold

Emotional stresses

24
Q

What is treatment of Raynaud’s phenomenon?

A

– Avoid cold, smoking
– Rewarming
– Ca channel blockers (nifedipine)
– EMG and biofeedback

25
Q

What is Polymyositis/ Dermatomyositis?

A

Inflammatory myopathies involving striated muscle and clinically presents with profound symmetrical weakness of the proximal muscles

26
Q

When can dysphagia occur in Polymyositis/ Dermatomyositis?

A

Pharyngeal involvement

27
Q

Describe the clinical features of Type I primary idiopathic polymyositis.

A 
– Insidious onset
– Weakness starts at the pelvic girdle> shoulder girdle >neck 
– Dysphagia/dysphonia 
– Moderate-severe arthritis 
– Atrophic skin over knuckles
– Remission and exacerbation common
28
Q

Describe the clinical features of Type II primary idiopathic dermatomyositis.

A

– Acute onset
– Proximal muscle weakness, tenderness
– Heliotrope rash with periorbital edema
– Malaise, fever, and weight loss

29
Q

Describe the clinical features of Type III dermatomyositis or polymyositis.

A

– 5% to 8% associated with malignancy
– Male, > 40 years old
– Poor prognosis

30
Q

Describe the clinical features of Type IV Childhood dermatomyositis or polymyositis.

A

– Rapid progressive weakness
– Respiratory weakness
– Severe joint contractures—more disabling in a child

31
Q

Describe the clinical features of Type V dermatomyositis or polymyositis.

A

Associated with collagen vascular disease

32
Q

What is seen on muscle biopsy in dermatomyositis or polymyositis?

A

– Perifascicular atrophy
– Evidence of necrosis of type I and II fibers
– Variation in fiber size
– Large nuclei

33
Q

What is elevated on lab work in dermatomyositis or polymyositis?

A

Creatinine phosphokinase
Aldolase levels
Transaminases
LDH

34
Q

What is seen on EMG in dermatomyositis or polymyositis?

A

– Small amplitude, short duration polyphasic motor units
– Early recruitment pattern
– + sharp waves, fibs
– CRDs

35
Q

What are dermatologic features seen in dermatomyositis?

A

– Lilac heliotrope rash with periorbital edema

– Gottron’s papules—scaly dermatitis over the dorsum of the hand—MCP, PIP

36
Q

What are poor prognositc factors of dermatomyositis or polymyositis?

A
  • Old age
  • Malignancy
  • Cardiac involvement
  • Delayed initiation of corticosteroid therapy
  • Respiratory muscle weakness—aspiration pneumonia
  • Joint contracture
37
Q

What is 1st line treatment of dermatomyositis or polymyositis?

A

Corticosteroids: generally 1 mg/kg/day prednisone for 4 to 6 weeks, then taper

38
Q

What is 2nd line treatment of dermatomyositis or polymyositis?

A

azathioprine or MTX

39
Q

What is treatment of Refractory dermatomyositis or polymyositis?

A

IV immunoglobulin

40
Q

Describe rehab for dermatomyositis or polymyositis?

A

ROM, isometric exercises—defer strengthening exercises until inflammation controlled

41
Q

Describe clinical features of Juvenile dermatomyositis.

A
  • Female>male
  • Heliotrope rash**
  • Clumsiness unrecognized
  • Transient arthritis
  • 80% to 90% respond well to corticosteroids
42
Q

What is Juvenile dermatomyositis associated with?

A
  • Generalized vasculitis

* No association with malignancy in children

43
Q

What are Mixed connective tissue disorders (MCTDs)?

A

D/O w/ characteristics of several other diseases
– SLE
– Scleroderma
– Polymyositis

44
Q

What are overlapping symptoms of Mixed connective tissue disorders (MCTDs)?

A 
– Raynaud’s phenomenon
– Synovitis of the hand 
– Arthritis 
– Myopathy
– Esophageal dysmotility 
– Acrosclerosis 
– Pulmonary HTN
– ABN Abs
45
Q

Which disorders are ANA (+)?

A 
MCTD
RA
SLE
Scleroderma (PSS)
Polymositis
Sjögren’s syndrome
46
Q

Which disorders are RF (+)?

A

MCTD
RA
Sjögren’s syndrome

47
Q

Which disorders are HLA-B27 (+)?

A
  • AS
  • Reactive arthritis
  • Psoriatic arthritis
  • Enteropathic arthropathy
  • Pauciarticular JRA
48
Q

What is Sjögren’s syndrome?

A

AI mediated disorder of the exocrine glands

49
Q

What is the clinical presentation of Sjögren’s syndrome?

A
  • Dry eyes
  • Dry mouth
  • Skin lesions
  • Parotid involvement
50
Q

What is primary Sjögren’s syndrome?

A

Occurs in people with no other rheumatologic disorders

51
Q

What is secondary Sjögren’s syndrome?

A

Occurs in patients with other rheumatologic disorders, most commonly RA and SLE

52
Q

What are the classification features of primary Sjögren’s syndrome?

A

Dry eyes and mouth with ANA(+), RF (+)

53
Q

What are the classification features of secondary Sjögren’s syndrome?

A

Sjögren’s syndrome plus evidence of SLE, RA, PSS, or polymyositis

54
Q

What are extraglandular manifestions of Sjögren’s syndrome?

A
  • Arthralgias

* Raynaud’s phenomenon

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