10 - 66 - RHEUMATOID ARTHRITIS, JUVENILE IDIOPATHIC ARTHRITIS, ADULT-ONSET STILL DISEASE AND RHEUMATIC FEVER Flashcards

1
Q

Symmetric arthritis of what joints are the most commonly affected in rheumatoid arthritis?

A

proximal interphalangeal and metacarpophalangeal joints

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2
Q

The most common dermatologic finding in RA

A

rheumatoid nodule

  • More than 90% of patients with rheumatoid nodules have seropositive RA
  • usual location is over pressure points such as the olecranon, the extensor surface of the forearms, and the Achilles tendon, but they have been described in almost every location, including viscera
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3
Q

main histologic findings of rheumatoid nodule

A

palisaded granulomas in the deep dermis or subcutaneous tissues with fibrinoid degeneration of collagen, a multitude of neutrophils and neutrophilic dust, with surrounding fibrosis and proliferation of vessels

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4
Q
A
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5
Q

what score is needed in the criteria for a definite diagnosis of RA?

A

at least 6/10

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6
Q

development of rheumatoid nodules in patients without chronic synovitis or radiographic findings, and mild or no systemic manifestations

A

Rheumatoid nodulosis

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7
Q

**accelerated nodulosis ** or erythema in and enlargement of preexisting rheumatoid nodules

A

Low-dose methotrexate, often used for the treatment of RA, may precipitate erythema in and enlargement of preexisting rheumatoid nodules, known as accelerated nodulosis. 5

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8
Q

another possible cutaneous complication of methotrexate therapy (occasionally other immunosuppressive drugs) in RA is the development of what virus-associated multifocal cutaneous lymphoproliferative disease, which may regress on discontinuation of therapy?

A

Epstein-Barr virus-associated multifocal cutaneous lymphoproliferative disease

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9
Q

remains the most consistently demonstrated environmental risk factor for rheumatoid vasculitis, particularly in male seropositive patients

A

Smoking

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10
Q

hyperextension of the proximal interphalangeal joint and compensatory flexion of the distal interphalangeal joint

A

“swan neck” deformity

Rheumatoid arthritis

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11
Q

flexion contracture of the proximal interphalangeal joint and extension of the distal interphalangeal joint

A

Boutonnière deformity

Rheumatoid arthritis

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12
Q

predicts disease severity and radiologic damage

A

presence of anticyclic citrullinated peptide antibodies

  • Therefore, ACPA could also be used as a biomarker for patients with a more-severe disease phenotype to target those patients for more aggressive treatment
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13
Q

more specific marker than rheumatoid factor, particularly in early disease where specificity ranges from 94% to 100%

A

Anticitrullinated protein antibodies (ACPAs)

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14
Q

should be part of the first treatment strategy for RA unless contraindicated

A

Methotrexate

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15
Q

Juvenile idiopathic arthritis (JIA) is a group of conditions encompassing all forms of arthritis of unknown etiology lasting for at least how many weeks and with an onset before how many years of age

A

at least 6 weeks and with an onset before 16 years of age

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16
Q

The diagnosis of juvenile psoriatic arthritis requires the coexistence of what features?

A

1. arthritis
2. typical psoriatic rash

or, when the rash is missing, the presence of arthritis and any 2 of the following:
1. family history of psoriasis in a first-degree relative,
2. dactylitis (sausage-like swelling of individual digits that extends beyond the joint margins), and
3. nail pitting or onycholysis

17
Q

criteria for systemic Juvenile Idiopathic Arthritis (sJIA)

A

The Internation League of Associations for Rheumatology criteria for sJIA require the presence of arthritis accompanied or preceded by a documented quotidian fever of at least 2 weeks duration, plus at least 1 of the following:

  1. characteristic rash - erythematous, salmon pink, evanescent macular rash usually appears with the fever
  2. generalized symmetrical lymphadenopathy,
  3. enlargement of liver or spleen
  4. serositis (pericarditis, pleural or pericardial effusion, [rarely] peritonitis).
  • The fever has a typical intermittent pattern, with 1 or 2 daily spikes, up to **39°C (102.2°F)or higher, followed by rapid return to baseline. **
  • Arthritis is often **symmetrical and polyarticular, **but may be absent at onset and develop much later.

  • Diagnosis cannot be considered definite until arthritis is present
18
Q

classic sJIA eruption

A

very transient salmon pink mostly macular eruption coincident with febrile episodes

  • It may at times be indistinguishable from urticaria but is typically not pruritic.
  • The eruption is identical to that often seen in adult-onset Still disease
19
Q

heralding clinical manifestation of macrophage activation syndrome (MAS)

A

hange in the fever pattern from the intermittent spikes of sJIA to a continuous high level

20
Q

Criteria for macrophage activation syndrome

A

MAS is considered present if a febrile patient with known or suspected sJIA who has a ferritin value >684 ng/mL also exhibits any 2 of the following:

  1. platelet count ≤181 × 109 /L,
  2. aspartate aminotransferase >48 units/L,
  3. triglycerides >156 mg/dL
  4. fibrinogen ≤360 mg/dL
21
Q

remains the most widely used conventional DMARD in management of JIA

A

methotrexate

22
Q

presenting sign of adult-onset Still disease/AOSD in most patients

A

High fever of unknown origin exceeding 39°C

Classically, patients present with** 1 to 2 daily fever spikes above 39°C (102.2°F)** occurring in the** afternoon or evening** and receding within hours

23
Q

frequency of symptoms in adult-onset Still disease (AOSD) in descending order

A
  1. fever 93%,
  2. arthralgia 90%,
  3. rash 70%
  4. sore throat 64%,
  5. lymphadenopathy/splenomegaly 53%,
  6. hepatomegaly 39%,
  7. pericarditis 13%.
23
Q

The most often reported atypical presentation of adult-onset Still disease (AOSD)

A

persistent, pruritic papules and plaques

  • These lesions may develop fine scale, and are most commonly located on trunk, extremities, head, and/or neck.
  • In some cases, plaques are linear suggesting triggering by local trauma (Koebner phenomenon).
  • The color of the atypical eruption was either erythematous or brown, and less commonly violaceous.
24
Q

associated with chronic and complicated adult-onset Still disease (AOSD)

A

arthritis and thrombocytopenia

25
Q

predictive of monocyclic AOSD

A

Fever >39.5°C (103.1°F)

26
Q

Two serious complications associated with AOSD

A
  1. reactive hemophagocytic syndrome (2.85% to 6%) - similar if not identical to MAS, and both are essentially part of the hemophagocytic lymphohistiocytosis spectrum
    2.** thrombotic thrombocytopenic purpura**
27
Q

Major Criteria of AOSD based on Yamaguchi et al

29
Q

Acute rheumatic fever is an inflammatory response to group A streptococcal infection, which typically occurs how many weeks after a throat infection

A

2 to 3 weeks

30
Q

common manifestations of rheumatic fever

A
  • migratory polyarthritis (especially knees, ankles, elbows, and wrists)
  • carditis

Other less-common clinical features include abnormal involuntary movements (chorea), and the skin findings of erythema marginatum and subcutaneous nodules

31
Q

Revised Jones Criteria for rheumatic fever

31
Q

A history of **rapid improvement with salicylates or NSAIDs **is characteristic of this disease

A

Acute rheumatic fever

32
Q

“chicken wire erythema”

A

erythema marginatum of ARF

33
Q

The second non-ARF syndrome involving a marginatum-type eruption is as a side effect of what medication?

A

oral multikinase inhibitor sorafenib

  • erythema marginatum hemorrhagicum
  • This was distinguished by a** purpuric margin and presumably was less labile **than the erythema marginatum of ARF
34
Q

valve most often affected in carditis in ARF

A

mitral valve is most often affected, followed by the aortic valve