Rheuma Flashcards
The primary mimics of rheumatic diseases are
infection and malignancy
Common symptoms include
joint pain, fever, fatigue, and rash.
Common in childhood and are a frequent reason for referral to pediatric rheumatologists.
Arthralgias
A stronger predictor of the presence of rheumatic disease and a reason for referral to a pediatric rheumatologist
Arthritis
Poor sleep, debilitating generalized joint pain that worsens with activity, school absences, and normal physical and laboratory findings in an adolescent suggests
Pain syndrome
Children ages 3 to 10 yr who have a history of episodic pain that occurs at night after increased daytime physical activity that is relieved by rubbing, but who have no limp or complaints in the morning, likely have
Growing pains.
Intermittent pain in a child, especially a girl age 3 to 10 yr, that is increased with activity and is associated with hyperextensible joints on exam is likely
Benign hypermobility syndrome.
Fatigue and/or stiffness after physical inactivity
Gelling phenomenon
Common presenting complaint in juvenile dermatomyositis (JDM)
Fatigue
Large vessels: affected vessels are aorta and its proximal branches
Takayasu arteritis
Medium vessels: affected vessels are renal arteries, mesenteric vasculature, coronary arteries
Polyarteritis nodosa & Kawasaki syndrome
Small vessels: affected vessels are capillaries, arterioles, post capillary venules
HSP, Churg-Strauss syndrome
Clinical criteria for Classical Kawasaki disease
fever for 5 days + at least 4 of the principal features: changes in extremities, polymorphous exanthema, bulbar conjunctival injection without exudates, changes in lips and oral cavity & unilateral cervical lymphadenopathy
Other name for Kawasaki Disease
Mucocutaneous Lymph Node Syndrome
Criteria of Kawasaki Disease
fever for 5 days + Conjunctivitis, Rash, Adenopathy, Strawberry tongue or other oropharyngeal changes, Hand changes
What are the acute changes in extremities of Kawasaki disease
erythema of palms, soles or edema of hands or feet
What are the acute changes in extremities of Kawasaki disease
periungal peeling of fingers, toes in weeks 2 and 3
What are the changes in lips and oral cavity in Kawasaki disease
erythema and cracking of lips, strawberry tongue, diffuse injection of oral and pharyngeal mucosa
What is the cut off size of the cervical lymphadenopathy in Kawasaki disease
=/> 1.5 cm
Clinical phase of Kawasaki that is characterized by fever and other signs of illness
Acute febrile phase
Acute febrile phase usually lasts up to
1-2 weeks
Clinical phase of Kawasaki that is associated with desquamation, thrombocytosis, the development of coronary aneurysms
Subacute phase
Clinical phase of Kawasaki where highest risk of sudden death in patients with aneurysm
Subacute phase
Subacute phase of Kawasaki usually last for about
2 weeks
Clinical phase of Kawasaki where it begins when all clinical signs of illness have disappeared and continues until the erythrocyte sedimentation rate (ESR) return to normal
Convalescent phase
Convalescent phase of Kawasaki typically last for about
6-8weeks after the onset of illness
Initial treatment for Kawasaki disease
Single dose of IVIG (2g/kg) and high dose aspirin (80 to 100mkday divided in 4 doses)
What is the dose of IVIG in Kawasaki disease
2g/kg
What is the dose of high dose of aspirin in Kawasaki disease
80 to 100mkday divided in 4 doses
Why is high dose aspirin administered in Kawasaki
for the anti-inflammatory effect
When should be treatment for Kawasaki initiated
within 10 days of fever onset
Patients who fail to respond to the 1st dose of IVIG are given what
2nd dose of IVIG 2g/kg
Low dose aspirin should be continued as treatment for Kawasaki until
6-8 weeks after disease onset if there are no coronary artery abnormalities or indefinitely if abnormalities are present.
What is the dose of low dose aspirin for Kawasaki
3 to 5 mkday SD
Why is low dose aspirin still administered in Kawasaki disease
for its antiplatelet effect
Clinical criteria for Takayasu Arteritis
Angiographic abnormalities (conventional, CT, or MRA) of the aorta or its main branches and at least 1 of the ff: dec peripheral artery pulse and/or claudication of extremities, BP difference between arms or legs of >10mmHg, Bruits over the aorta and/or its major branches, HPN & elevated acute phase reactant (ESR or CRP)
To diagnose myositis, you need at least 2 of the ff:
Proximal symmetric muscle weakness, Elevated Creatining Kinase, AST, LDG or aldolase, EMG findings characteristic of myopathy and denervation & Muscle biopsy documenting myositis/ MRI showing proximal muscle inflammation
ANA in Juvenile Dermatomyositis is
Positive
Is rarely needed to confirm cutaneous manifestation of Juvenile dermatomyositis
Skin biopsy
MC pediatric inflammatory myopathies
Juvenile dermatomyositis
Systemic vasculopathy with characteristic cutaneous findings and focal areas of myositis resulting in progressive proximal muscle weakness
Juvenile dermatomyositis
Alligator skin like appearance seen in juvenile dermatomyositis
Gottron papules
Described as skin over the metacarpal and proximal IPJ may be hypertrophic and reddish pink
Gottron papules
Described as periorbital violaceous erythema that may cross over the bridge of the nose
Heliotrope eyelids / heliotrope rash
