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

Question 1

Most common dermatologic finding in RA

Answer 1

Rheumatoid nodule

Question 2

Usual location of rheumatoid nodules

Answer 2

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

Question 3

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

Answer 3

Rheumatoid nodulosis

Question 4

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

Answer 4

Pseudorheumatoid nodulosis

Question 5

Distribution of pseudorheumatoid nodulosis

Answer 5

Tibia
Dorsal foot
Scalp

Question 6

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

Answer 6

Accelerated nodulosis

Question 7

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

Answer 7

Methotrexate

Question 8

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

Answer 8

Methotrexate

Question 9

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

Answer 9

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

Question 10

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

Answer 10

Palisaded neutrophilic and granulomatous dermatitis

Question 11

Tumor necrosis factor inhibitors and allopurinol have been implicated as causes

Answer 11

Palisaded neutrophilic and granulomatous dermatitis

Question 12

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

Answer 12

Interstitial granulomatous dermatitis

Question 13

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

Answer 13

Interstitial granulomatous drug reaction

Question 14

Commonly implicated drugs in IGDR

Answer 14

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

Question 15

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

Answer 15

Rheumatoid neutrophilic dermatosis

Question 16

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

Answer 16

Autoimmune neutrophilic dermatoses syndrome

Question 17

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

Answer 17

TNFalpha inhibitors

Question 18

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

Answer 18

Rheumatoid vasculitis

Question 19

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

Answer 19

Smoking

Question 20

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

Answer 20

Vasculitic neuropathy

Question 21

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

Answer 21

Rheumatoid vasculitis

Question 22

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

Answer 22

Polyarteritis nodosa

Question 23

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

Answer 23

Bywaters lesions

Question 24

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

Answer 24

Pyoderma gangrenosum

Question 25

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

Answer 25

Felty syndrome

Question 26

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

Answer 26

Erythema

Question 27

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

Answer 27

Rheumatoid arthritis

Question 28

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

Answer 28

Radial | Ulnar

Question 29

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

Answer 29

"Swan neck" deformity

Question 30

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

Answer 30

Boutonniere deformity

Question 31

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

Answer 31

Arginine | Citrulline

Question 32

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

Answer 32

Anticitrullinated protein antibodies (ACPAs)

Question 33

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

Answer 33

Carbamylation

Question 34

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

Answer 34

HLA-DRB1 molecule

Question 35

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

Answer 35

Rheumatoid factor

Question 36

More specific marker than rheumatoid factor, particularly in early disease

Answer 36

ACPAs

Question 37

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

Answer 37

Disease-modifying antirheumatic drugs

Question 38

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

Answer 38

Methotrexate

Question 39

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

Answer 39

Leflunomide | Sulfasalazine

Question 40

Should be considered when initiating or changing conventional DMARDs

Answer 40

Short-term glucocorticoids

Question 41

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

Answer 41

Juvenile idiopathic arthirtis

Question 42

Juvenile idiopathic arthiritis encompasses 7 entities

Answer 42

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

Question 43

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

Answer 43

Systemic-onset JIA | Psoriatic arthiritis

Question 44

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

Answer 44

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

Question 45

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

Answer 45

5% to 15%

Question 46

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

Answer 46

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

Question 47

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

Answer 47

Intermittent

Question 48

The erythematous, salmon pink, evanescent macular rash usually appears

Answer 48

With the fever

Question 49

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

Answer 49

Symmetrical | Polyarticular

Question 50

The eruption of sJIA is identical to that often seen in

Answer 50

Adult-onset Still disease

Question 51

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

Answer 51

Enthesitis-related arthritis

Question 52

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

Answer 52

Macrophage activation syndrome

Question 53

Often the heralding manifestation of macrophage activation syndrome

Answer 53

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

Question 54

Most widely used conventional DMARD in JIA

Answer 54

Methotrexate

Question 55

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

Answer 55

Adult-onset Still disease

Question 56

Two serious complications associated with AOSD

Answer 56

Reactive hemophagocytic syndrome | Thrombotic thrombocytopenic purpura

Question 57

An important newly recognized complication of AOSD is its association with

Answer 57

Malignancy

Question 58

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

Answer 58

``` Hematopoietic (mostly lymphomas) Solid tumors (breast, lung, esophagus, and liver angiosarcoma) ```

Question 59

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

Answer 59

Myocarditis

Question 60

Standard criteria set for AOSD

Answer 60

Yamaguchi criteria

Question 61

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

Answer 61

AOSD

Question 62

More specific for the diagnosis of AOSD

Answer 62

Fraction of glycosylated ferritin

Question 63

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

Answer 63

20 to 50

Question 64

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

Answer 64

2 to 3 weeks

Question 65

Most cases of ARF occur in

Answer 65

Children ages 5 to 15 years

Question 66

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

Answer 66

RF

Question 67

Diagnosis of initial acute rheumatic fever

Answer 67

2 major criteria or | 1 major plus 2 minor criteria

Question 68

Diagnosis of recurrent acute rheumativ fever

Answer 68

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

Question 69

ARF major criteria

Answer 69

``` Carditis Chorea Erythema marginatum Subcutaneous nodules Arthritis ```

Question 70

Rapid improvement with salicylates or NSAIDs is characterisitic

Answer 70

RF

Question 71

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

Answer 71

4 weeks

Question 72

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

Answer 72

Yes - each occurring in less than 5% of cases

Question 73

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

Answer 73

Erythema marginatum

Question 74

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

Answer 74

Subcutaneous nodules

Question 75

Subcutaneous nodules often occur in association with

Answer 75

Carditis

Question 76

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

Answer 76

Chorea

Question 77

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

Answer 77

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

Question 78

Recommended alternative in instances of penicillin allergy

Answer 78

Erythromycin