SM_241a: Pediatric Rheumatology Flashcards
Describe juvenile idiopathic arthritis
Juvenile idiopathic arthritis
- Arthritis for 6 weeks
- Diagnosis of exclusion
- Onset type defined by type of disease in first 6 months
- Onset 16th birthday or younger
- Prevalence is 20-100 / 100k
Describe epidemiology of sJIA
sJIA epidemiology
- 5-15% of children with JIA
- Any age including adults
- Male = female
- Greatest morbidity and mortality of all JIA subtypes
sJIA can onset at ___ age and is ____ common in males compared to females
sJIA can onset at any age and is equally common in males compared to females
(greatest morbidity and mortality of all JIA subtypes)
Describe the clinical presentation of sJIA
sJIA clinical presentation
- Arthritis (often polyarticular): large and small joints involved, usually symmetric
- Spiking quotidian (1-2x/daily) fever for at least three weeks
- One or more of evanescent rash, hepatosplenomegaly, lyphadenopathy symmtric and generalized, and serositis (pericardial or pleural effusions)
_____ rash is seen in sJIA
Evanescent rash is seen in sJIA
- Salmon colored patches or macules
- Trunk, proximal extremities, axilla, and groin
- Appears with fever
Describe clinical signs of sJIA
sJIA clinical signs
- Symptoms may not all present simultaneously
- Fever can be isolated initially
- 10% have no arthritis at presentation
- Wide range of presentation from mild to critically ill
Describe sJIA pathophysiology
sJIA pathophysiology
- IL-1, IL-6, and IL-18 driven inflammation via abnormal cytokine expression - autoinflammatory
- No consistent HLA associations
- T-lymphocyte abnormalities later in course of disease
sJIA inflammation is driven by abnormal expression of ____, ____, and ____ cytokines (autoinflammatory)
sJIA inflammation is driven by abnormal expression of IL-1, IL-6, and IL-18 cytokines (autoinflammatory)
(treated by glucocorticoids and anti IL-1 and anti IL-6 biologics)
Describe sJIA labwork
sJIA labwork
- CBC signs of inflammation: anemia, leukocytosis, thrombocytosis
- Elevated ESR, CRP, ferritin
- Elevated LFTs
- Coagulation changes: prolonged PT/PTT, high d-dimers, and high fibrinogen
____ is a severe hyperinflammatory state that is a complication of sJIA
Macrophage activation syndrome is a severe hyperinflammatory state that is a complication of sJIA
Describe macrophage activation syndrome
Macrophage activation syndrome
- Complication of sJIA: severe hyperinflammatory state that can be fatal, 10% of sJIA patients but subclinical in 30-40%
- Secondary hemophagocytic lymphohistiocytosis
- Labs: DIC -> low ESR, low fibrinogen, pancytopenia, elevated triglycerides, LFTs especially LDH, sky-high ferritin, hemophagocytosis in BM
sJIA therapy includes ____ and ____
sJIA therapy includes glucocorticoids and anti IL-1 and anti IL-6 biologics
(early biologic therapy alters course of disease)
Describe Kawasaki disease diagnostic criteria
Kawasaki disease diagnostic criteria
- Fever for ≥ 5 days
- Plus at least four of: changes in peripheral extremities (edema / erythema) or perineum, polymorphous rash, bilateral non-exudative conjunctivitis, changes of lips and oral cavity, and cervical lymphadenopathy
- Arthralgia is uncommon, arthritis is rare
____ rash is present in Kawasaki disease
Polymorphous rash is present in Kawasaki disease
(erythematous macular, papular, annular, morbiliform, no vesicles)
Describe Kawasaki disease epidemiology
Kawasaki disease
- Highest incidence in Japan
- In US, most common among Asians
- 85% under age 5, peak at 2-3 years old
- Slight male predominance
Kawasaki disease is most common in ____ and ____ and onsets most often before age ____
(treated with aspirin and IVIG in first 10 days)
Kawasaki disease is most common in Asians and men and onsets most often before age 5
Describe Kawasaki disease pathogenesis
Kawasaki disease pathogenesis
- Likely infectious trigger
- May be superantigen stimulating large numbers of T cells
- May be genetic predisposition
- Systemic necrotizing vasculitis with predilection for coronary arteries (small and medium size vessels)
Kawasaki disease likely has a(n) ____ trigger and involves ____
Kawasaki disease likely has an infectious trigger and involves systemic necrotizing vasculitis with predilection for coronary arteries (small and medium size vessels)
Disease course of Kawasaki disease involves ____, ____, and ____ phase
Disease course of Kawasaki disease involve acute febrile phase, subacute phase, and convalescent phase
- Acute febrile phae (10-14 days): preceding URI or GI symptoms, fever and cutaneous symptoms (criteria), carditis / pericarditis / abdominal pain / irritability
- Subacute phase (2-4 weeks): desquamation, arthritis, and aneurysms
- Convalescent phase (months): asymptomatic
Describe Kawasaki disease laboratory data
Kawasaki disease laboratory data
- Increased ESR and CRP
- Elevated WBC count
- Anemia
- Platelets rise by 2nd week
- Transaminitis, sterile pyuria, low albumin common
- ECG and echo: carditis and coronary aneurysms
- Shock and macrophage activation syndrome can occur
Kawasaki disease therapy involves ____ and ____
Kawasaki disease therapy involves aspirin and IVIGz
- Reduces risk of coronary involvement if within first 10 days
Describe epidemiology of IgA vasculitis
IgA vasculitis epidemiology
- Most common childhood vasculitis
- Most frequent between 3-15 yo: rare but very severe in adults
- Female to mael: 1:1.5
- More cases in winter months
IgA vasculitis is the ____ vasculitis, is predominant in ____, and manifests primarily in ____ months
IgA vasculitis is the most common vasculitis, is predominant in males, and manifests primarily in winter months
____ rash is in IgA vasculitis
Palpable purpura rash is in IgA vasculitis
- Dependent distribution
- Can be preceded by macules and urticaria
Describe pathogenesis of IgA vasculitis
IgA vasculitis
- Infectious trigger: Group A strep, preceded by URI
- IgA-mediated dysregulated immune response to antigen
- Alternative complement pathway is triggered
- Familial clustering: HLA prediposition
IgA commonly occurs after a ___, involves ___ and ___, and has a ___ prediposition
IgA commonly occurs after a URI, involves IgA-mediated dysregulated immune response to antigen and triggered alternative complement pathway, and exhibits HLA predisposition
Describe clinical signs of IgA vasculitis
IgA vasculitis clinical signs
- Palpable purpura: dependent distribution, can be preceded by macules / urticaria
- GI manifestations due to vasculitis: currant jelly stools, colicky abdnominal pain (risk of ileo-ileal intussusception)
- Kidney involvement (30%) with 3-6 weeks of rash: microscopic hematuria, proteinuria, renal failure
- Arthralgia, arthritis in 50-80% predominantly lower extremities
- Subcutaneous edema: often periarticular
- Scrotal pain, swelling
IgA lab data shows ____ and is often ____
IgA lab data shows NO thrombocytopenia and is often unremarkable
(elevated IgA, check urinalysis and BP)
Juvenile dermatomyositis is ____, has ____, involves progressive, and is ____
Juvenile dermatomyositis is inflammatory myopathy with vasculopathy, has cutaneous findings, involves progressive muscle weakness, and is NOT associated with cancer in children
Describe epidemiology of juvenile dermatomyositis
Juvenile dermatomyositis
- Incidence: 1-3 million children/year
- Most common in Caucasians
- Female predominance
- Average age at onset 6.7 years but 25% are below age 4
Juvenile dermatomyositis is most common in ____, has ____ predominance, and average onset at ____
Juvenile dermatomyositis is most common in Caucasians, has female predominance, and average onset at 6.7 years
Describe etiology of juvenile dermatomyositis
Juvenile dermatomyositis etiology
- Case clusters
- UV exposure
- Antibodies
- Genetic susceptibility
____ exposure causes juvenile dermatomyositis
UV exposure causes juvenile dermatomyositis
Describe pathogenesis of juvenile dermatomyositis
Pathogenesis of juvenile dermatomyositis
- Putative antigen and/or immune complexes activate endothelial cells
- Endothelium damaged by complement activation -> release of von Willebrand factor antigen
- Occlusion of capillaries & arterioles results in infarction of local tissues
Describe clinical signs of juvenile dermatomyositis
Juvenile dermatomyositis clinical signs
- Usually insidious onset, nonspecific symptoms, acute
- Rash + weakness in 50% of cases
- Rash occurs first in 25% of cases
- Rash is prominent on sun-exposed surfaces: torso (shawl or V distribution), extensor surfaces of extremitis, and scalp with hair loss due to chronic inflammation
Rash is prominent on ____ in juvenile dermatomyositis
Rash is prominent on sun exposed surfaces in juvenile dermatomyositis
____ seen in ____ are hypertrophic reddish pink areas of skin which are papular and scaly, occur over the MCP and interphalangeal joints as well as elbows and knees, and heal as atrophic colorless bands
Gottron’s papules seen in juvenile dermatomyositis are hypertrophic reddish pink areas of skin which are papular and scaly, occur over the MCP and interphalangeal joints as well as elbows and knees, and heal as atrophic colorless bands
____ seen in ____ is periorbital violaceous erythema that may cross the nasal bridge
Heliotrope rash seen in juvenile dermatomyositis is periorbital violaceous erythema that may cross the nasal bridge
Describe clinical signs of juvenile dermatomyositis
Juvenile dermatomyositis clinical signs
- Diffuse vasculopathy correlates with severe disease: nail bed telangiectasia, infarction of oral epithelium and skin-folds, digital / skin / gastrointestinal ulceration
- Insidious proximal muscle weakness
- Muscle and fat atrophy
- Calcium cutis (calcium deposits)
Laboratory studies of juvenile dermatomyositis show elevated ____
Laboratory studies of juvenile dermatomyositis show elevated muscle enzymes
(myositis specific antibodies predict findings and prognosis)
____ are used to treat juvenile dermatomyositis
Oral and IV steroids are used to treat juvenile dermatomyositis
(early identification and aggressive treatment)
Summarize Kawasaki disease, IgA vasculitis, systemic JIA, and juvenile dermatomyositis
Kawasaki disease, IgA vasculitis, systemic JIA, and juvenile dermatomyositis
