10 - 66 - RHEUMATOID ARTHRITIS, JUVENILE IDIOPATHIC ARTHRITIS, ADULT-ONSET STILL DISEASE AND RHEUMATIC FEVER Flashcards
Symmetric arthritis of what joints are the most commonly affected in rheumatoid arthritis?
proximal interphalangeal and metacarpophalangeal joints
The most common dermatologic finding in RA
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
main histologic findings of rheumatoid nodule
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
what score is needed in the criteria for a definite diagnosis of RA?
at least 6/10
development of rheumatoid nodules in patients without chronic synovitis or radiographic findings, and mild or no systemic manifestations
Rheumatoid nodulosis
**accelerated nodulosis ** or erythema in and enlargement of preexisting rheumatoid nodules
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
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?
Epstein-Barr virus-associated multifocal cutaneous lymphoproliferative disease
remains the most consistently demonstrated environmental risk factor for rheumatoid vasculitis, particularly in male seropositive patients
Smoking
hyperextension of the proximal interphalangeal joint and compensatory flexion of the distal interphalangeal joint
“swan neck” deformity
Rheumatoid arthritis
flexion contracture of the proximal interphalangeal joint and extension of the distal interphalangeal joint
Boutonnière deformity
Rheumatoid arthritis
predicts disease severity and radiologic damage
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
more specific marker than rheumatoid factor, particularly in early disease where specificity ranges from 94% to 100%
Anticitrullinated protein antibodies (ACPAs)
should be part of the first treatment strategy for RA unless contraindicated
Methotrexate
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
at least 6 weeks and with an onset before 16 years of age
The diagnosis of juvenile psoriatic arthritis requires the coexistence of what features?
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
criteria for systemic Juvenile Idiopathic Arthritis (sJIA)
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:
- characteristic rash - erythematous, salmon pink, evanescent macular rash usually appears with the fever
- generalized symmetrical lymphadenopathy,
- enlargement of liver or spleen
- 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
classic sJIA eruption
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
heralding clinical manifestation of macrophage activation syndrome (MAS)
hange in the fever pattern from the intermittent spikes of sJIA to a continuous high level
Criteria for macrophage activation syndrome
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:
- platelet count ≤181 × 109 /L,
- aspartate aminotransferase >48 units/L,
- triglycerides >156 mg/dL
- fibrinogen ≤360 mg/dL
remains the most widely used conventional DMARD in management of JIA
methotrexate
presenting sign of adult-onset Still disease/AOSD in most patients
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
frequency of symptoms in adult-onset Still disease (AOSD) in descending order
- fever 93%,
- arthralgia 90%,
- rash 70%
- sore throat 64%,
- lymphadenopathy/splenomegaly 53%,
- hepatomegaly 39%,
- pericarditis 13%.
The most often reported atypical presentation of adult-onset Still disease (AOSD)
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.
associated with chronic and complicated adult-onset Still disease (AOSD)
arthritis and thrombocytopenia
predictive of monocyclic AOSD
Fever >39.5°C (103.1°F)
Two serious complications associated with AOSD
-
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**
Major Criteria of AOSD based on Yamaguchi et al
Acute rheumatic fever is an inflammatory response to group A streptococcal infection, which typically occurs how many weeks after a throat infection
2 to 3 weeks
common manifestations of rheumatic fever
- 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
Revised Jones Criteria for rheumatic fever
A history of **rapid improvement with salicylates or NSAIDs **is characteristic of this disease
Acute rheumatic fever
“chicken wire erythema”
erythema marginatum of ARF
The second non-ARF syndrome involving a marginatum-type eruption is as a side effect of what medication?
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
valve most often affected in carditis in ARF
mitral valve is most often affected, followed by the aortic valve