Rheumatology Flashcards
2 events considered necessary for the juvenile RA to occur
Immunogenetic susceptibility and external environmental trigger
HLA of patients with juvenile RA Polyarticular disease
HLA DR4
2 HLA of patients with juvenile RA pauciarticular disease
HLA DR8 and DR5
Difference between pauciarticular and polyarticular types of juvenile RA in terms of number of joints involved
Poly - 5 or more
Pauci -
Morning stiffness, ease of fatigue especially after school in the early afternoon, joint pain later in the day, and joint swelling
Juvenile RA
Type of juvenile RA that is associated with chronic uveitis
Pauciarticular
(+) RF in juvenile RA portends a poor or good prognosis
Poor
(+) ANA in juvenile RA portends a poor or good prognosis
Good
Most common pediatric rheumatic disease witg arthritis as the distinguishing manifestation
Juvenile idiopathic arthritis or juvenile RA
Systemic juvenile RA has what pattern of fever?
Quotidian pattern (1-3 spikes in 24 hours with rapid return to baseline or subnormal levels)
Duration of juvenile RA
6 weeks or more
Treatment of juvenile RA
NSAIDS (first line)
Methotrexate
Steroids
Physical and occupational therapy
Surgery
Rheumatoid disorder characterized by absence of RF, ANA, and other markers
Juvenile spondyloarthropathy
Triad of arthritis, conjunctivitis, and urethritis caused by Chlamydia trachomatis
Reiter’s disease
HLA for ankylosing spondylitis
B27
Characterized by presence of enthesitis
Ankylosing spondylitis
Inflammation at the sites of attachments of ligaments, tendons, fascia, and capsule to bone
Enthesitis
X-ray finding in ankylosing spondylitis
Sacroilitis
Serologic markers specific for SLE
Anti-dsDNA AND anti-Sm
Serologic markers for SLE that may be used as marker for active disease especially lupus nephritis
Anti-dsDNA
Mainstay of therapy for SLE
Glucocorticoids
Drug useful for children with severe lupus nephritis and CNS lupus
Cyclophosphamide
Other: azathioprine, methotrexate, cyclosporine
5-yr survival rate of SLE
90%
Heliotrope discoloration of the eyelids with periorbital edema and erythematous papules over the extensor surfaces of the joints, and Gottron papules in the ankle
Dermatomyositis
Most common of the pediatric inflammatory myopathies
Dermatomyositis
Etiology of dermatomyositis
Coxsackie B and GABHS
Exposure to sun is a cofactor
HLA in dermatomyositis
Ag DQA1*0501
Excessive accumulation of collagen in the skin and other organs
Scleroderma
Skin thickening with loss of dermal ridges, resembling tightened skin
Scleroderma
Antibodies in limited scleroderma
Anti centromere
Antibodies for systemic scleroderma
Anti Scl 70
Earliest manifestation of scleroderma
Reynauds phenomenon
Scleroderma associated with CREST
Limited scleroderma
HSP is mediated by
IgA
Involved vessels in HSP
Small blood vessels
Skin biopsy result of leukocytoclastic angiitis; renal biopsy result of IgA mesangial deposition
HSP
Treatment of HSP
Symptomatic
Self limiting
Steroids for severe abdominal pain
Monitoring for HSP patients
Urinalysis every 3 months for 1 years (high recurrence rate)
Polyarteritis nodosa is associated with what infection in adults? In children?
Hep B; GABHS
Medium vessel or small vessel vasculitis characterized by aneurysms and thrombosis (brachial, femoral, or mesenteric arteries)
Polyarteritis nodosa
Immune complex disease
Livedo reticularis, subcutaneous nodules with skin, GI, and kidney involvement
Polyarteritis nodosa
With HPN, peripheral neuropathy
Treatment of polyarteritis nodosa
High dose corticosteroid
Cyclophosphamide
Characterized by necrotizing granulomas in multiple organs, most commonly the respiratory tract and kidneys
Wegener’s granulomatosis
Severe sinusitis, hemoptysis and glomerulonephritis are seen in
Wegener’s granulomatosis
Antibodies in wegener’s granulomatosis
c-ANCA
A vasculitis syndrome characterized by a triad of aphthous stomatitis, genital ulceration, and uveitis
Behcet’s disease
CNS and GI involvement
Treatment of Behcet’s disease
Corticosteroids and thalidomide
Skin reaction to a needleprick seen in Behcet’s disease
Pathergy
What is the most serious cardiac complication of neonatal lupus?
Heart block
Puffiness around fingers, dorsum of the hands and face, followed by skin tightening
Systemic sclerosis
Sclerodactyly, digital pitting scars, and bibasal pulmonary fibrosis are minor criteria for
Systemic sclerosis
Major criteria: proximal scleroderma
Medications that may prevent fingertip ulcerations
Calcium channel blockers and ACE inhibitors
