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Fitz 9th > 66 - Rheumatoid Arthritis, Juvenile Idiopathic Arthritis, Adult-Onset Still Disease, and Rheumatic Fever > Flashcards

66 - Rheumatoid Arthritis, Juvenile Idiopathic Arthritis, Adult-Onset Still Disease, and Rheumatic Fever Flashcards

1
Q

Most common dermatologic finding in RA

A

Rheumatoid nodule

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2
Q

Usual location of rheumatoid nodules

A

Pressure points such as the olecranon, extensor surfaces of the forearms, and the Achilles tendon

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3
Q

Development of rheumatoid nodules in patients without chronic synovitis or radiographic findings, and mild or no systemic manifestations
Involves men predominantly, and many develop frank RA

A

Rheumatoid nodulosis

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4
Q

In children, similar lesions of rheumatoid nodulosis are termed
Most regress within 2 years; progression to RA is extremely rare

A

Pseudorheumatoid nodulosis

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5
Q

Distribution of pseudorheumatoid nodulosis

A

Tibia
Dorsal foot
Scalp

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6
Q

Low-dose methotrexate, often used for the treatment of RA, may precipitate erythema in and enlargement of preexisitng rheumatoid nodules, known as

A

Accelerated nodulosis

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7
Q

A papular reaction to _____ has been reported as a syndrome of clustered, erythematous, indurated papules arising most commonly on the proximal extremities and buttocks

A

Methotrexate

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8
Q

Another possible cutaneous complication of ______ therapy (occasionally other immunosuppressive drugs) in RA is development of Epstein-Barr virus-associated multifocal cutaneous lymphoproliferative disease

A

Methotrexate

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9
Q

Reactive granulomatous dermatitis has been proposed as an inclusive term for the following 3 syndromes

A

Interstitial granulomatous dermatitis
Palisaded neutrophilic and granulomatous dermatitis
Interstitial granulomatous drug reaction

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10
Q

Presents as symmetric skin-colored to erythematous smooth, umbilicated, or crusted papules, primarily on the elbows and extremities
Histologically, early features include intense neutrophilic inflammation, karyorrhectic debris, and leukocytoclastic vasculitis. Later findings are piece-meal areas of collagen degeneration and palisades of histiocytes and small granulomas, eventually accompanied by areas of fibrosis

A

Palisaded neutrophilic and granulomatous dermatitis

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11
Q

Tumor necrosis factor inhibitors and allopurinol have been implicated as causes

A

Palisaded neutrophilic and granulomatous dermatitis

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12
Q

Usually asymptomatic, this presents symmetrically on the lateral upper trunk and proximal inner arms and thighs, and occasionally on the buttocks, abdomen, breast, and umbilicus
Histopathologic findings include a dense, diffuse infiltrate of histiocytes arranged in a band-like configuration in middle or deep reticular dermis
Epidermis with basal vacuolization is rarely seen

A

Interstitial granulomatous dermatitis

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13
Q

Probably often used interchangeably with IGD
Present as erythematous to violaceous plaques, often annular, concentrated on inner arms, proximal medial thighs, proximal trunk, and intertriginous sites
Histologic findings are similar to IGD, but should include interface dermatitis with basal vacuolar degeneration, areas of dyskeratosis, and prominent tissue eosinophilia

A

Interstitial granulomatous drug reaction

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14
Q

Commonly implicated drugs in IGDR

A

Calcium channel blockers
Beta-blockers
Lipid lowering agents
Angiotensin-converting enzyme inhibitors

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15
Q

Very rare cutaneous manifestation in patients with severe RA
Lesions are usually chronic, erythematous, and urticaria-like plaques and papules that are sharply marginated
Histopathologically, lesions have a dense infiltrate of neutrophils without leukocytoclasia

A

Rheumatoid neutrophilic dermatosis

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16
Q

Produces urticarial lesions in association with SLE, RA, and secondary Sjogren syndrome, which lesions regress within 24 hours
Unlike urticaria, they are typically nonpruritic
Histopathologically, lesions show interstitial and perivascular neutrophlic infiltrate with leukocytoclasia without vasculitis, vacuolar alteration at the dermo-epidermal junction

A

Autoimmune neutrophilic dermatoses syndrome

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17
Q

Patients treated with _____ may develop an eruption that is clinically and histologically indistinguishable from psoriasis. This can occur in those receiving these agents for any condition, but those with RA appear to be at greatest risk

A

TNFalpha inhibitors

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18
Q

Most often affects men and smokers with longstanding disease, who are seropositive to rheumatoid factor and to cittrulinated peptides

A

Rheumatoid vasculitis

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19
Q

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

A

Smoking

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20
Q

In rheumatoid vasculitis patients, cutaneous vasculitis was the most common presentation, followed by

A

Vasculitic neuropathy

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21
Q

Although complement levels are normal or elevated in RA, hypocomplementemia is often seen in patients with

A

Rheumatoid vasculitis

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22
Q

Rheumatoid vasculitis primarily affects small- to medium-sized vessels systemically and shares many features with _____, albeit without the development of microaneurysms

A

Polyarteritis nodosa

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23
Q

Minute digital ulcerations or petechiae and digital pulp papules
Manifestation of mild vasculitis and typically occur without systemic signs of vasculitis

A

Bywaters lesions

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24
Q

Should be suspected if deep liquefying ulcers with a characteristic purple, undermined border occur in patients with RA
Occurs more frequently and more severely in females

A

Pyoderma gangrenosum

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25
Q

Leg ulcers may also appear in patients with _____, a combination of chronic RA, hypersplenisms, and leukopenia

A

Felty syndrome

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26
Q

Synovial inflammation in RA usually results in warmth, but not _____ of the affected areas

A

Erythema

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27
Q

Characteristic involvement of the proximal interphalangeal and metacarpophalangeal joints with sparing of the distal interphalangeal joints
Hand and foot involvement is predominant
Symmetric and diffuse pattern

A

Rheumatoid arthritis

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28
Q

Characteristic deformities of RA include (ulnar/radial) deviation of the hand with (ulnar/radial) deviation of the digits

A

Radial

Ulnar

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29
Q

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

A

“Swan neck” deformity

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30
Q

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

A

Boutonniere deformity

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31
Q

An important posttranslational modification of protein is the conversion of _____ to its polar analog _____. This greatly enhances immune recognition of joint-associated proteins, which are selectively targetted by autoreactive T and B cells in patients with RA

A

Arginine

Citrulline

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32
Q

Antibodies that are highly specific for RA and can be found is approximately 50% of early RA patients
Predicts disease severity and radiologic damage

A

Anticitrullinated protein antibodies (ACPAs)

33
Q

Process where the amino acid lysine is changed to become homocitrulline
Enhanced by smoking

A

Carbamylation

34
Q

More than 100 genetic loci are associated with RA, the most important being within the HLA class II region, encoding the

A

HLA-DRB1 molecule

35
Q

Autoantibody that reacts with the Fc portion of immunoglobulin G
Found in sera of 85% of patients with RA

A

Rheumatoid factor

36
Q

More specific marker than rheumatoid factor, particularly in early disease

A

ACPAs

37
Q

New recommendation for RA means that therapy with _____ should be started as soon as the diagnosis is made, with the aim of sustained remission or low disease activity

A

Disease-modifying antirheumatic drugs

38
Q

Should be part of the first treatment strategy in RA, unless contraindicated

A

Methotrexate

39
Q

Suggested as a first treatment strategy in RA when methotrexate is contraindicated

A

Leflunomide

Sulfasalazine

40
Q

Should be considered when initiating or changing conventional DMARDs

A

Short-term glucocorticoids

41
Q

Group of conditions encompassing all forms of arthritis of unknown etiology lasting for at least 6 weeks and with an onset before 16 years of age

A

Juvenile idiopathic arthirtis

42
Q

Juvenile idiopathic arthiritis encompasses 7 entities

A 
Systemic onset arthritis
Oligoarthritis (persistent or extended)
Rheumatoid factor-negative polyarthritis
Rheumatoid factor-positive polyarthritis
Psoriatic arthritis
Enthesitis-related arthritis
Undifferentiated
43
Q

Of the types of JIA, only the _____ forms have skin findings

A

Systemic-onset JIA

Psoriatic arthiritis

44
Q

There is increasing evidence that juvenile psoriatic arthritis is not a homogeneous disease entity, but includes at least 2 distinct subgroups

A

Same characteristics as early-onset antinuclear antibody-positive JIA
Spectrum of spondyloarthropathies

45
Q

Systemic-onset JIA accounts for _____% of children with JIA

A

5% to 15%

46
Q

The International League of Associations for Rheumatology criteria for sJIA require the presence of

A

Arthritis accompanied or preceded by a documented quotidian fever of at least 2 weeks duration

47
Q

The fever of sJIA has a typical _____ pattern, with 1 or 2 daily spikes, up to 39C or higher, followed by rapid return to baseline

A

Intermittent

48
Q

The erythematous, salmon pink, evanescent macular rash usually appears

A

With the fever

49
Q

Arthritis of sJIA is often (symmetrical/asymmetrical) and (mono-/oligo-/polyarticular)

A

Symmetrical

Polyarticular

50
Q

The eruption of sJIA is identical to that often seen in

A

Adult-onset Still disease

51
Q

Usually affects boys older than age 6 years and presents with lower-limb asymmetrical arthritis associated with enthesitis
Later, these children can develop inflammatory lumbosacral pain and are at risk of developing acute anterior uveitis

A

Enthesitis-related arthritis

52
Q

Secondary or acquired form of hemophagocytic lymphohistiocytosis (HLH)
Potentially life-threatening complication of rheumatic disorders, most commonly with sJIA and adult-onset Still disease

A

Macrophage activation syndrome

53
Q

Often the heralding manifestation of macrophage activation syndrome

A

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

54
Q

Most widely used conventional DMARD in JIA

A

Methotrexate

55
Q

Characterize a syndrome of seronegative polyarthritis, salmon-colored mostly macular and evanescent eruption, fever, and raised erythrocyte sedimentation rate, which along with neutrophilic leukocytosis, mirrors the presentation of sJIA

A

Adult-onset Still disease

56
Q

Two serious complications associated with AOSD

A

Reactive hemophagocytic syndrome

Thrombotic thrombocytopenic purpura

57
Q

An important newly recognized complication of AOSD is its association with

A

Malignancy

58
Q

The malignancies associated with AOSD were 50% _____, 50% _____

A 
Hematopoietic (mostly lymphomas)
Solid tumors (breast, lung, esophagus, and liver angiosarcoma)
59
Q

Rare but potentially life-threatening complication of AOSD that responds positively to corticosteroids and other immunomodulatory drugs

A

Myocarditis

60
Q

Standard criteria set for AOSD

A

Yamaguchi criteria

61
Q

A very elevated serum ferritin with a lowered concentration of glycosylated ferritin, is strongly suggestive of, but not specific for this diagnosis

A

AOSD

62
Q

More specific for the diagnosis of AOSD

A

Fraction of glycosylated ferritin

63
Q

In healthy individuals, 50% to 80% of serum ferritin is glycosylated, but this drops to _____% in patients with inflammatory diseases

A

20 to 50

64
Q

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

A

2 to 3 weeks

65
Q

Most cases of ARF occur in

A

Children ages 5 to 15 years

66
Q

Most probably involves cross-reactivity of streptococcal antibodies against myocardium, synovial tissue, and, in chorea, the basal ganglea`

A

RF

67
Q

Diagnosis of initial acute rheumatic fever

A

2 major criteria or

1 major plus 2 minor criteria

68
Q

Diagnosis of recurrent acute rheumativ fever

A

2 major criteria or
1 major plus 2 minor or
3 minor criteria

69
Q

ARF major criteria

A 
Carditis
Chorea
Erythema marginatum
Subcutaneous nodules
Arthritis
70
Q

Rapid improvement with salicylates or NSAIDs is characterisitic

A

RF

71
Q

Generally, the arthritis in ARF runs a self-limited course, even without therapy, lasting approximately

A

4 weeks

72
Q

Y/N: Both erythema marginatum and subcutaneous nodules are rare in ARF

A

Yes - each occurring in less than 5% of cases

73
Q

Much like the eruption in sJIA and Still disease, is evanescent, but differs by its tendency to develop annular or serpiginous erythema
Typically occurs on the torso, upper arms, and legs, and spares the face

A

Erythema marginatum

74
Q

Typically less than 2 cm in diameter, firm, painless, and mobile nodules
Usually appear during the first weeks of the inflammatory phase and tend to localize over extensor surfaces

A

Subcutaneous nodules

75
Q

Subcutaneous nodules often occur in association with

A

Carditis

76
Q

Typically occurs after a longer latent period (up to 6 months) after a sterptococcal infection, by which time the other inflammatory features of rheumatic fever have resolved

A

Chorea

77
Q

Treatment is first directed at eradication of group A beta-hemolytic streptococci from the oropharynx using

A

Phenoxymethylpenicillin (penicillin VK) 250 mg twice daily in children; 500mg twice daily in adolescents or
Single dose of IM benzathine penicillin followed by institution of long-term prophylaxis

78
Q

Recommended alternative in instances of penicillin allergy

A

Erythromycin

Fitz 9th (108 decks)

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