Rheumatology Flashcards
Symptoms suggestive of rheumatic disease
joint pain
fever
fatigue
rash
Arthralgia WITHOUT physical findings of arthritis suggests
infection malignancy orthopedic conditions benign syndromes pain syndromes like fibromyalgia
Kawasaki Disease (Mucocutaneous Lymph Node Syndrome)
FEVER for at least 5 days + 4 of the ff
CRASH
Conjunctivitis - bilateral, bulbar injection (-) exudate
Rash
Adenopathy - solitary, cervical adenopathy > 1.5 cm
Strawberry tongue and other oropharyngeal changes
Hand changes - brawny edema, induration, peeling around the nail beds
Common in ACUTE phase especially in younger then 1 y/o patients
PERINEAL DESQUAMATION
Begins 1-3 weeks AFTER the onset of illness
PERIUNGUAL DESQUAMATION
Develop in the 2nd-3rd week of illness in untreated cases
Coronary Artery Aneurysms
Clinical phases of Kawasaki Disease
Acute Febrile
Subacute
Convalescent
Lasts 1-2- weeks; fever and other acute signs of illness
Acute Febrile Phase
Begins when fever and other signs have abated
Highest risk of sudden death in those who developed aneurysms
Lasts until 4 weeks
Subacute Phase
Begins when all clinical signs have disappeared and continues until ESR and CRP return to normal
Convalescent Phase
Any infant or child with 5 or more days of fever with 2-3 of the principal clinical features but have compatible laboratory findings
May have manifestations such as – renal failure, unilateral facial nerve palsy, pulmonary infiltrates
Atypical Kawasaki Disease
Considered in all children with unexpected fever for 5 or more days with 3 or less of the clinical criteria
More common in young infants
Incomplete Kawasaki Disease
This should be performed at diagnosis of Kawasaki and repeated after 2-3 weeks of illness
If both are normal – repeat study after 6-8 weeks
2D-Echo
Treatment of Kawasaki Disease
Acute Stage
IVIg 2 g/kg over 10-12 hrs
Aspirin 80-100
Treatment of Kawasaki Disease
Convalescent Stage
Aspirin 3-5 mg/kg OD daily until 6-8 weeks
Long term therapy for patients w/ Coronary Abnormalities
Aspirin 3-5 mg
Clopidogrel 1 mg/kg/day
Warfarin or LMWH
Persistent of recrudescent fever 36 hr after completion of the initial IVIG infusion
INCREASED RISK FOR CAA
IVIG Resistant KD
give another dose of IVIG at 2 g/kg
methylprednisolone - if poor response pa din
Serious, rare condition in children in which the body’s own immune system overreacts to a stimulus
Results in inflammation of multiple organ systems → leads to impaired organ function and organ failure
Diagnosed in <21 years of age in mostly Europe and the USA
Unknown cause
Mounting evidence that is linked to COVID-19
Multisystem Inflammatory Syndrome in Children (MIS-C)
HEART - commonly affected
MIS-C
Patient is 21 years of age or younger and has had a fever of 38°C for at least 24 hours
blood work shows indications of inflammation
requires treatment at a hospital (often in the intensive care unit)
due to severe illness that includes dysfunction of two or more organs, particularly the heart, blood vessels, GI organs, lungs, kidneys, skin, eyes, or nervous system
The MC of pediatric inflammatory myopathies
Systemic vasculopathy with characteristic cutaneous findings and focal areas of myositis – progressive proximal muscle weakness
JUVENILE DERMATOMYOSITIS
Gottron papules
Heliotrope eyelids/Heliotrope rash
Shawl sign