Rheum Part 3-Paulson (Exam 2) Flashcards

1
Q

A child might have a form of JIA if they have at least ___ affected joint(s) for ___ week(s).

A

at least 1 joint

for at least 6 weeks

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2
Q

JIA can manifest in many different ways, but the main mechanism of the disease if ________________________.

A

persistent joint swelling that comes from synovial fluid increase (with inflammatory cells) + expansion of pannus=joint damage

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3
Q

This subtype of JIA is most common in ages 1-7yrs, involves 4 joints or less (asymmetrically); most commonly seen in the knee joint. This kiddos might also have prominent morning stiffness.

A

Oligoarticular JIA (aka pauriarticular JIA)

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4
Q

Children with oligoarticular JIA (esp ones with positive ANA) have an increased risk of developing what type of eye disorder?

A

Uveitis (can be asymptomatic and/or occur without the joint symptoms)

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5
Q

This subtype of JIA affects 5 or more joints and typically has an elevated ESR.

A

Polyarticular JIA

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6
Q

If a child with polyarticular JIA has a positive Rf, it is referred to as ____________.

A

Seropositive

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7
Q

___________(seropositive or seronegative) is the more aggressive form of polyarticular JIA and resembles the adult version of RA.

A

Seropositive

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8
Q

T/F? Pt’s with Seronegative polyarticular JIA usually have other extraarticular sx’s like rheumatoid nodules, vasculitis, and lung dz.

A

False. Seronegative does not have extraarticular sx’s, while seropositive does.

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9
Q

The other/ technical name for “Still’s Disease is _________________.

A

Systemic Onset JIA (SOJIA)

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10
Q

Children with SOJIA are likely to have a daily ________ fever. What does this mean?

A

Daily quotidian fever, is a fever that spikes then resolves spontaneously, can go up and down

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11
Q

If you see a child with a “salmon-colored evanescent macular rash” on their trunk and extremities, what rheumatologic disease would you think about?

A

Systemic Onset JIA (SOJIA)

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12
Q

You are working up an ill-appearing 10 yr old child, who you suspect has SOJIA due to his clinical presentation. You decide to run labs. What labs do you choose and what do you expect them to reflect if he does indeed have SOJIA?

A

Labs: CBC, ESR and Iron panel

Results would show: High leukocytosis, very high ESR, elevated ferritin

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13
Q

______ ________ syndrome is a potentially fatal complication that can occur in kiddos with SOJIA. This syndrome occurs due to excess circulating ___________, which can cause seizures/DIC/bleeding.

A

MAS (Macrophage activation syndrome)

Cytokines

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14
Q

The following are characteristics of what type of JIA? Arthritis in at least 1 joint, DIP synovitis, dactylitis, nail pitting.

A

Psoriatic Arthritis

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15
Q

With enthesitis-related JIA, you would expect tenderness of the ____________ and a positive HLA-B27.

A

Sacroiliac

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16
Q

Which type of JIA includes those with juvenile-onset spondylitis, reactive arthritis, and arthritis associated with IBD?

A

enthesitis-related JIA

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17
Q

How do you diagnose JIA?

A

Clinically diagnosed; no specific lab/imaging to definitely confirm the diagnosis’, but they can guide you in the right direction and exclude other possibilities.

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18
Q

What are the mainstays of treatment for JIA?

A
NSAIDS (ibuprofen, naproxen, meloxicam)
Joint injections (triamcinolone acetate)
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19
Q

Children with SOJIA may need a __________ intially for sx control.

A

steroid

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20
Q

Pt’s with which type of JIA should be treated aggressively with a DMARD?

A

Seropositive polyarticular JIA

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21
Q

Which form of JIA has the highest mortalilty?

A

SOJIA

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22
Q

_______ ________ is a chronic, systemic autoimmune disorder with fibrosis of the skin and other organs.

A

Systemic sclerosis (Scleroderma)

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23
Q

There are 2 forms of Scleroderma; _________ which includes CREST syndrome, and _________.
Which is more common?

A

Limited (affects face, distal to knees/elbows)

Diffuse (throughout he body)

MC=Limited (80%)

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24
Q

What does CREST Syndrome consist of?

A

Calcinosis cutis- Ca deposits in the skin
Raynaud phenomenon(usu 1st sign, can start years before any other sx’s)
Esophageal dysmotility
Sclerodactyly-thickening/tightening of skin on toes/finger
Telangiectasia

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25
Q

There is a higher incidence of scleroderma in which two specific populations?

A

African americans

Choctaw Native Americans

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26
Q

What are some common skin manifestations of scleroderma?

A
Initially, edema + pruritis
Later, thick, hide-like skin
Hyperpigmentation or depigmentation
Digital ulcers
Pitting of fingertips
Loss of appendicular hair
Telangiectasias
Calcinosis
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27
Q

Scleroderma can also manifest in other important organs. One thing that has been found on autopsy is vascular ectasia of the stomach antrum, aka ___________ ___________.

A

Watermelon stomach

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28
Q

________ _______ ______ is a complication of Scleroderma and is the sudden onset of malignant HTN that can lead to renal failure and death.

A

Scleroderma Renal Crisis (SRC)

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29
Q

With Scleroderma pt’s will have a ________(pos/neg) ANA 95% of the time.
_________ is another lab that is positive in 1/3 of pt’s with diffuse dz and 20% with CREST (Indicates poor prognosis).

A

POSITIVE

Anti-SCL-70

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30
Q

What is the tx for scleroderma?

A

No proven disease-modifying drug to treat scleroderma.

Treatment is targeted to organs affected.

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31
Q

How would you treat a scleroderma pt with diffuse skin involvement and/or systemic involvement?

A

Immune process targeted: glucocorticoids, methotrexate, mycophenolate mofetil (MMF), cyclophosphamide, IVIG

32
Q

What class of meds is used to treat Raynaud Phenomenon?

A

Calcium Channel blockers
(Long-acting nifedipine 30-180 mg daily or
Sildenafil 50 mg PO BID)

33
Q

What is the leading cause of mortality in pts with Scleroderma?

A

Lung disease

CHF and CKD also common

34
Q

____________ ______ __________ is a Chronic inflammatory disease with autoantibodies and varying presentations that can affect every organ.

A

Systemic Lupus Erythematosus (SLE)

35
Q

SLE is more prevalent in which population?

A

Childbearing females

36
Q

SLE can have many different manifestations. If a pt presents with a localized malar “butterfly” rash that spares the nasolabial folds (often triggered by sun exposure) OR generalized maculopapular lesions in a photosensitive distribution of any part of the body, they would be said to have ______ ________ ______ _________.

A

Acute cutaneous lupus erythematosus (ACLE)

37
Q

________ _________ _________ __________ is the most photosensitive of all SLE rashes and includes scaly, erythematous papules of the torso/limbs, but spares the face.

A

Subacute cutaneous lupus erythematosus (SCLE)

38
Q

_______ ________ _________ _________ presents with discoid lupus: raised erythematous plaques with a scale/raised erythematous ring around a lesion.

A

Chronic cutaneous lupus erythematosus (CCLE)

39
Q

________ lupus is the most common subtype of CCLE, and consists of raised, erythematous plaques with a scale. Raised erythematous ring around the lesion.
Lead to scarring, skin atrophy, as well as
Lupus panniculitis.

A

Discoid

40
Q

In SLE, pt’s can have MSK sx’s like __________ and __________ arthritis.

A

Arthralgia

Nonerosive (arthritis)

41
Q

SLE commonly involves the renal system; there are several types of lupus ______________.

A

glomerulonephritis

**Renal involvement is a poor prognostic factor.

42
Q

SLE pt’s can also have involvement of the GI system, lungs and heart. What are the most common presentation of each of these symptoms?

A

GI- dysphagia
lungs- pleuritis
cardiac- pericarditis

43
Q

Pts with SLE are at risk for an atypical cardiac condition called _________ _________ ___________, which can lead to arrhythmias.

A

Atypical verrucous endocarditis (Libman-Sacks)

44
Q

What are the most common presentations of the eye, lymph and blood in pt’s with SLE?

A

Eye- keratoconjunctivitis (secondary Sjorgens syndrome possible)
Lymph- LAD; commonly cervical, axillary, and inguinal nodes.
Blood- Anemia of chronic dz is most common. Leukopenia and mild thrombocytopenia is also common.

45
Q

Name 4 antibody labs that can be ordered in pt’s you suspect to have SLE. What is the specificity/ purpose of each?

A
  1. ANA almost always positive, but not specific for SLE.
  2. Anti-dsDNA- Specific but not sensitive. Levels often correspond to disease activity.
  3. Anti-Sm (anti-Smith)- 99% specific, but not very sensitive.
  4. Antiphospholipid antibodies- Lupus anticoagulant. Can prolong aPTT. **False positive for syphilis
46
Q

Name some drugs that can cause Drug-induced lupus?

A

Procainamide, hydralazine, isoniazid, chlorpromazine, methyldopa, minocycline, quinidine

47
Q

Ways to differ b/w SLE and drug-induced lupus? (3 ways)

A

If drug-induced:

  1. Doesn’t normally have nephritis and CNS involvement
  2. No anti-dsDNA antibodies or hypocomplementemia
  3. Lab abnormalities and clinical manifestations usually normalize when drug is discontinued
48
Q

How do you diagnose SLE?

A
ACR criteria (4 out of 11)
or 
SLICC criteria (4 out of 17)
or 
if pt doesnt meet either of these criteria, you can still dx if pt has 2-3 criteria plus at least 1 other feature assicated with SLE: 
Optic neuritis,
Aseptic meningitis,
Pneumonitis, pulmonary hemorrhage, pHTN, ILD,
Myocarditis, Libman-Sacks endocarditis,
Abdominal vasculitis,
Raynaud phenomenon,
Elevated ESR/CRP
49
Q

ACR Criteria for SLE

A

4 out of 11

  • malar rash
  • photosensitivity-skin rash
  • discoid rash
  • oral ulcers-usu painless
  • arthritis-nonerosive,2+ peripheral joints
  • serositis-pleuritis/pericarditis
  • renal disorder
  • neuro disorder-sz/ psychosis
  • hematologic disorder
  • ANA
  • immunologic disorders- anti-DNA or anti-Sm OR antiphospholipid antibody
50
Q

SLICC Classification Criteria

A
4 out of 17
Acute cutaneous lupus
Chronic cutaneous lupus
Nonscarring alopecia
Oral or nasal ulcers
Joint disease
Serositis
Renal
Neurologic
Hemolytic anemia
Leukopenia or lymphopenia
Thrombocytopenia
ANA
Anti-dsDNA
Anti-Sm
Antiphospholipid
Low complement
Direct Coomb’s test
51
Q

What are some general tx considerations for SLE?

A
  • exercise
  • smoking cessation
  • sun protection (DAILY sunscreen of 55+ SPF)
  • healthy diet
  • prevent glucocorticoid-induced osteoporosis–bisphosphanates?
  • many will need to be treated w antimalarials (hydroxychloroquine)
52
Q

How would you treat cutaneous lupus sx’s? MSK sx’s? Serositis sx’s?

A

Cutaneous- intially topical glucocorticoids (start low then move up). Can do intralesional injections for triamcinolone for refractory lesions. Some may need systemic meds (hydroxychloroquine)

MSK–NSAID or Tylenol are 1st line. May add hydroxychloroquine.

Serositis– NSAID and/or low-mod glucocorticoids. Hydroxychloroquine or PO colchicine if persistent/recurrent

53
Q

How would you treat a pt with SLE who has thrombocytopenia <20k or <50k with hx of bleeding and severe autoimmune hemolytic anemia?

A
  • high dose glucocorticoids, then taper once improvement seen.
  • IVIG often used
  • immunosuppressants
  • splenectomy (last resort)
54
Q

What are the commonly used immunosuppressants for SLE?

A
Glucocorticoids
hydroxychloroquine
methotrexate
azathioprine
mycophenolate mofetil
cyclophosphamide
55
Q

T/F? Pt’s with SLE have a 5x higher incidence of MI than general population.

A

True, due to Atherosclerosis from chronic inflammation

56
Q

__________ is a rare autoimmune myopathy characterized by proximal muscle weakness

A

polymyositis

57
Q

What symptoms might a pt with polymyositis present with?

A

Proximal muscle weakness and muscle fatigue (not usu painful)

  • usu gradual and progressive
  • symmetric
  • may c/o trouble rising from chair, climbing stairs, washing hair
58
Q

In a pt with polymyositis, what particular lab would you expect to be elevated? Which lab is most helpful for detecting cardiac involvement? What other labs might also be elevated?

A

CK

Troponin

LDH, aldolase, AST, ALT

59
Q

What is the gold standard for diagnosing inflammatory myopathies?

A
muscle biopsy (of muscle thats weak/affected, but not atrophied--quads or deltoids are good choices)
will show inflammatory infiltrates invading fascicles
60
Q

What could an EMG and MRI help you with/show you when working up a pt with possible polymyositis?

A

Good to identify which muscle you want to biopsy

  • Do bx on contralateral side to avoid needle artifact
  • MRI might be even better than EMG at this
  • Helps differentiate myositis from a neuropathic cause for weakness
  • EMG typically shows early recruitment
  • Increased number of motor units firing rapidly to produce a low level of contraction
  • MRI can show areas of muscle inflammation, edema, fibrosis, calcification
61
Q

How do you make a diagnosis or polymyositis?

A

No validated diagnosis/classification criteria.
Can make the diagnosis without a muscle biopsy if the patient has:
Characteristic clinical symptoms
Proximal muscle weakness
Abnormal muscle enzyme labs
No better explanation for a diagnosis

62
Q

What is the treatment for polymyositis?

A
  • Initially steroids-Prednisone 1 mg/kg/day PO daily (max 80 mg) 4-6 weeks, taper slowly based on tx response/labs.
  • osteoporosis prophylaxis

Frequently combine w/another immunosuppressant, either initially or later if no response to steroids. Azathioprine (2 mg/kg/day) or
Methotrexate (up to 25mg/day).

PT recommended.

63
Q

T/F? Most pt’s with polymyositis don’t regain completely normal muscle function.

A

true
also, worst prognosis in those who had delayed tx.

*Fun fact- Those with anti-Jo-1 antibodies usually don’t completely respond to treatment & do worse in general.

64
Q

In adult pt’s with _________ arthritis, it can be difficult to distinguish from RA.

A

Psoriatic

65
Q

Which presentation of psoriatic arthritis is more common, symmetric polyarthritis or asymmetric oligoarthritis?

A

symmetric polyarthritis

66
Q

An adult pt presents with DIP joint pain/swelling and cervical spine pain, as well as morning stiffness greater than 30 mins. What type of arthritis comes to mind?

A

Psoriatic

67
Q

Pts with psoriatic arthritis can have ______ _______, which is a deforming, destructive arthritis.

A

Arthritis mutilans

68
Q

Psoriatic arthritis can also involve the spine, referred to as ___________.

A

Spondylarthritis

69
Q

Psoriatic arthritis can also cause inflammation at the sits of insertion of tendons causing soft tissue swelling. What is this condition referred to as?

A

enthesitis

70
Q

________ or inflammation of the flexor tendons of hands, as well as __________ or diffuse swelling of an entire finger or toe, are also signs of psoriatic arthritis.

A

Tenosynovitis

Dactylitis “sausage digit”

71
Q

What signs might you see on a pt’s nails if they have psoriatic arthritis?

A

Pitting
Ridging
Onycholysis

Nail bed hyperkeratosis
Splinter hemorrhage

72
Q

The “pencil-in-cup” appearance on xray, is indicative of what type of arthritis?

A

Psoriatic

73
Q

How do you make a diagnosis of psoriatic arthritis?

A
No consensus on diagnostic criteria.
Generally, diagnosis made in a patient with both psoriasis & inflammatory arthritis in a typical pattern.
Can diagnose it in the absence of psoriasis if:
Distal joint involvement
Asymmetric distribution
Nail lesions
Dactylitis
FH of psoriasis
Presence of HLA-C*06
74
Q

What is CASPAR criteria?

A

Used to dx patient with an inflammatory MSK disease with PsA if they have at least 3 points from the following:
-Skin psoriasis that is:
Present (2 points)
Previously present by hx (1 point)
FH of psoriasis, if pt not affected (1 point)
-Nail lesions (1 point)
-Dactylitis (present or past) (1 point)
-Negative RF (1 point)
-Juxtaarticular bone formation on radiographs (1 point)

75
Q

When treating psoriatic arthritis, what medication do you want to avoid? Why?

A

PO steroids

Can cause flare of pustular psoriasis

76
Q

How do you treat psoriatic arthritis?

A
-Mild Peripheral or axial arthritis (<4 joints/ no damage seen on xrays)
  NSAIDS 1st choice
  Naproxen, Celebrex
-More severe or unresponsive to NSAIDS:
  DMARDs
  Methotrexate, sulfasalazine, 
  azathioprine, cyclosporine, 
  hydroxychloroquine
-Biologic TNF inhibitors
  Etanercept, infliximab, 
  adalimumab, golimumab, 
  certolizumab pegol