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Pediatrics > Rheumatology > Flashcards

Rheumatology Flashcards

1
Q

Henoch-Schonlein Purpura

Definition

Epidemiology

A
  • Systemic IgA-mediated vasculitis involving the skin, joints, GI tract & kidneys
  • Disease of young children
    • 75% <10 YO
    • Median age of onset: 5 YO
    • Male:female = 2:1
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2
Q

What is the prodrome of Henoch-Schonlein Purpura?

A
  • Viral syndrome or URI
  • 20% comitant or prior group A ß-hemolytic stretococcal infection
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3
Q

What are the skin manifestations of Henoch-Schonlein Purpura?

A
  • Urticarial or erythematous maculopapular lesions progress to petechiae & palpable purpuric lesions concentrated on the buttocks & LE
  • Children: edema of hands, feet, scrotum, scalp
  • Infants: facial rash + edema
  • GI & joint symptoms may precede diagnostic rash by days/wks (30%)
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4
Q

What are the joint manifestations of Henoch-Schonlein Purpura?

A
  • Occur in 80% of patients
  • Arthralgia or arthritis
  • Most involved: knees & ankles
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5
Q

What are the GI manifestations of Henoch-Schonlein Purpura?

A
  • 67% of patients
  • Colicky abdominal pain
  • GI bleeding (occult blood or bloody stool)
  • Increased risk of intussusception
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6
Q

What are the renal manifestations of Henoch-Schonlein Purpura?

A
  • Wide range of presentation
    • Mild hematuria & trace proteinuria
    • Gross hematuria
    • Nephrotic syndrome
    • Chronic renal insufficiency
    • ESRD (1%)
  • May not become clinically apparent for up to 3 mo after initial presentation in 25% of nephritis patients
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7
Q

How is Henoch Schonlein Purpura diagnosed?

A
  • History & characteristic physical exam
  • Routine lab tests are not specific/diagnostic
  • Increased serum IgA levels (50%)
  • Circulating IgA immune complexes in serum & IgA deposition in skin & glomeruli
  • Platelet counts normal despite petechiae & purpura (skin rash is a nonthrombycytopenic purpura)
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8
Q

Henoch Schonlein Purpura

management

prognosis

A
  • Relief of symptoms (pain control & hydration)
  • Steroids for abdominal pain & arthritis
  • Prognosis
    • Recover w/i 4 wks
    • Recurs at least once in 50%
    • Long-term morbidity depends on severity of nephritis
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9
Q

What is Kawasaki Disease (Mucocutaneous Lymph Node Syndrome)?

A
  • Acute febrile vasculitis of childhood
  • Unknown origin
  • Multiple organ systems
    • Heart
    • Skin
    • Mucous membranes
    • GI tract
    • CNS
    • Joints
    • Peripheral vascular bed
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10
Q

What is the epidemiology of Kawasaki disease?

(sex, ethnicity, age)

A
  • The most common cause of acquired heart disease in the US
  • Male:female = 3:2
  • Children of Asian ethnicity
  • Mean age: 18-24 mo
  • 80% cases in children <5 YO
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11
Q

What is the diagnostic criteria for Kawasaki Disease?

A
  • Fever >102oF (38.9oC) lasting _>_5 days
  • 4 of the following:
    • Bilateral conjunctivitis: bulbar injection w/ limbic sparing w/o exudate
    • Oropharyngeal changes: pharyngitis, strawberry tongue, red/cracked/swollen lips
    • Cervical adenopathy: unilateral nonsuppurative cervical LN _>_1.5 cm diameter
    • Rash: truncal polymorphous rash: erythematous maculopapular, morbilliform, scarlatiniform
    • Changes in distal extremities
      • Early (first 7-10 days): brawny edema & induration of the hands/feet w/ erythematous palms & soles
      • Later (7-10 days after fever): peeling around nail beds or distal extremities
  • The illness must not be explainable by any other disease process
    • Bacterial, viral, rickettsial infections
    • Rheum conditions (JRA)
    • Drug rxns
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12
Q

What are 6 other clinical features of Kawasaki Disease?

A
  1. CV manifestations
  2. Urethritis (sterile pyuria)
  3. Aseptic meningitis
  4. Hydrops of the gallbladder (10%, RUQ pain)
  5. Arthritis (sterile) or arthralgias
  6. Anterior uveitis
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13
Q

What are the cardiovascular manifestations of Kawasaki Disease?

A
  • Coronary artery aneurysms (20%)
    • Untreated patients
    • Subacute phase (days 7-14)
  • Low grade myocarditis (common)
  • CHF
  • Arrhythmias
  • Aneurysms of brachial arteries
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14
Q

What is the time course of Kawasaki Disease?

A

Triphasic

  • Phase I: acute phase (1-2 wks)
  • Phase II: subacute phase (wks-mo)
  • Phase III: convalescent phase (wks-yrs)
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15
Q

What are the laboratory findings of Kawasaki Disease?

A
  • No laboratory tests are pathognomonic
  • Acute phase
    • Increased ESR & CRP
  • Subacute phase
    • Increased platelet count
    • Decreasing ESR & CRP
  • Convalescent phase
    • Lab findings normalize w/i 6-8 wks
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16
Q

What are the options for managing Kawasaki Disease?

A
  • Anti-inflammatory therapy, serial ECGs
  • IVIG
    • High dose (2 g/kg) IVIG + ASA
    • Initiated w/i 10 days of fever onset
    • Decrases prevalence of coronary artery dilatation & aneurysms detected 2-7 wks later
  • ASA
    • Acute phase: high-dose ASA (anti-inflammatory)
    • Subacute phase: low-dose ASA (anti-platelet)
  • Steroids
    • Controversial; increased morbidity
    • Patients unresponsive to IVIG
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17
Q

Acute phase (I)

  • time course
  • clinical
  • lab
  • treatment
A
  • time course
    • 1-2 wks
  • clinical
    • Fever, conjunctivitis, oropharyngeal changes, cervical adenopathy, rash, swollen hands
  • lab
    • Increased ESRD & CRP
  • treatment
    • HIgh-dose IVIG, high-dose aspirin
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18
Q

Subacute phase (II)

  • time course
  • clinical
  • lab
  • treatment
A
  • time course
    • wks-mo
  • clinical
    • Defervescence of inflammation, peeling from nailbeds or distal extremities, coronary artery aneurysms
  • lab
    • Decreased ESR & CRP
    • Increased platelet count
  • treatment
    • Low-dose aspirin
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19
Q

Convalescent phase (III)

  • time course
  • clinical
  • lab
  • treatment
A
  • time course
    • wks-yrs
  • clinical
    • Gradual resolution of aneurysms
  • lab
    • Normalization of all laboratory findings
  • treatment
    • Continue low-dose aspirin only if aneurysms remain
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20
Q

What is the prognosis of Kawasaki disease?

A
  • If CAD absent, no long-term sequelae
  • Even if CAD present, mortality <1% & aneurysms commonly regress
  • Long-term prognosis unclear, increased risk of atherosclerotic heart disease in adulthood possible
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21
Q

What is the definition of Juvenile Rheumatoid Arthritis?

A

Disorder characterized by chronic joint inflammation in children, w/ or w/o extra-articular involvement

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22
Q

What is the epidemiology of JRA?

(age, sex)

A
  • Most common pediatric rheumatic disease w/ arthritis as the distinguishing manifestation
  • Mean age of onset: 1-3 YO (<6 mo unusual)
  • Most commonly in females, exceptions:
    • Males equally likely to have systemic onset JRA
    • Males more likely to have late-onset pauciarticular JRA (M:F = 10:1)
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23
Q

How is JRA classified?

A
  • Determined on the basis of clinical features during first 6 mo of disease
  • 3 categories of JRA
    • Pauciarticular
    • Polyarticular
    • Systemic
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24
Q

Pauciarticular JRA

  • definition
  • epidemiology
  • subtypes
  • articular involvement
A
  • < 4 joints involved, 40% of cases
  • Subtypes
    • Early-onset
    • Late-onset
  • Swelling of 1 or 2 joints, not necessarily symmetric
  • Most common joints: knees & hips
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25
Q

What is early-onset pauciarticular JRA?

A
  • Female predominant
  • Present 1-5 YO
  • +ANA (75%)
  • High risk for chronic uveitis (50%)
    • Inflammation of the iris & ciliary body
    • Monitor by regular slit-lamp evaluations
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26
Q

What is late-onset pauciarticular JRA?

A
  • Male predominant
  • Present >8 YO
  • Typical patient (like ankylosing spondylitis)
    • Male, HLA-B27 positive
    • Hips & sacroiliac joints
    • Uveitis less common
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27
Q

Polyarticular JRA

  • definition
  • subtypes
  • articular involvement
A
  • >4 joints involved, 40% of cases
  • Systemic involvement & extra-articular features mild or absent
  • Subtypes
    • RF-negative disease
    • RF-positive disease
  • Symmetric polyarthritis
    • Small joints (hands, feet)
    • Large joints (knees, ankles, hips)
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28
Q

Describe the subtypes of Polyarticular JRA

A
  • Based on absence/presence of serum RF
    • IgM molecule directed against IgG
  • Females > Males
  • RF negative disease
    • Early & late in childhood
  • RF positive disease
    • Children >8 YO
    • More severe, high risk of severe arthritis & rheumatoid nodules
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29
Q

What is Systemic-onset JRA (Still’s disease)?

What are the top 4 clinical symptoms?

A
  • 20% of cases, severe > joint
  • Clinical
    • High spiking fevers
      • >39oC (102.2oF)
      • Late afternoon/evening
      • Return quickly to baseline/normal
      • Diff: “fever of unknown origin”
    • Transient salmon-colored rash
      • Trunk & proximal extremities
      • During febrile episodes
      • Evanescent (w/ fevers then fades)
      • Non-pruritic
    • Hepatosplenomegaly
    • Lymphadenopathy
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30
Q

What are some additional clinical features of Still’s disease?

A
  • Fatigue, anorexia, weight loss, failure to thrive
  • Serositis (pericarditis, pleuritis)
  • CNS involvement (meningitis, encephalopathy)
  • Myositis, tenosynovitis
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31
Q

What is the diagnostic criteria for JRA?

A
  • Age of onset _<_16 YO
  • Arthritis in _>_1 joint defined as:
    • Swelling or effusion *OR*
    • Limitation of motion, tenderness, increased warmth
  • Duration of disease >6 wks
  • Exclusion of other causes of arthritis
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32
Q

What are the laboratory findings of JRA?

A
  • Non-specific, reflect existence/extent of inflammation
  • Anemia
    • Microcytic & hypochromic
    • Anemia of chronic disease
  • Elevated acute-phase reactants
    • ESR, CRP, platelet count
  • Rheumatoid markers
    • RF negative in a majority of patients
    • ANA
      • 75% of early pauci JRA,
      • 50% of polyarticular JRA
      • NOT present in Still’s/late onset pauci
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33
Q

How is JRA managed?

A
  • Control of inflammation
    • NSAIDs ease pain & inflammation
    • Immunomodulation for severe symptoms
    • Glucocorticoids, MTX, sulfasalazine, hydroxychloroquine
  • Mechanical & physical measures
    • PT/OT, selective splinting to minimize joint contractures
  • Surgery
    • Recalcitrant joint contractures/destruction
  • Psychosocial support
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34
Q

Systemic Lupus Erythematosus (SLE)

  • definition
  • epidemiology
  • etiology
A
  • Multisystem autoimmune disorder
    • Widespread inflammation of the connective tissues
    • Immune complex-mediated vasculitis
  • Female:Male = 8:1
  • Age of onset rare before 10 YO, peaks in adolescence
  • Cause unknown
    • Triggers?: drug rxns, excessive sun exposure, infections, hormone changes
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35
Q

What are the 9 clinical features of SLE?

A
  1. Constitutional symptoms
    1. Fever, weight loss, malaise
  2. CNS
  3. Skin findings
  4. Arthralgias & arthritis
  5. GI involvement
  6. CV involvement
  7. Pulmonary involvement
  8. Renal involvement
  9. Heme manifestations
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36
Q

CNS involvement of SLE

A
  • Headache
  • Encephalopathy
  • Seizures
  • Psychosis
  • Transverse myelitis
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37
Q

Skin findings of SLE

A
  • Malar rash
    • “butterfly” covering nasal bridge & cheeks
  • Photosensitivity
  • Alopecia
  • Raynaud’s phenomenon
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38
Q

Arthralgias & arthritis of SLE

A
  • Migratory & transient
  • Rarely causes joint deformity or erosion
  • Myositis
39
Q

GI involvement of SLE

A
  • Hepatosplenomegaly
  • Splenic infarction
  • Mesenteric thrombosis (secondary to vasculitis)
  • Sterile peritonitis
40
Q

CV involvement of SLE

A
  • Most frequent manifestation: pericarditis
  • CHF
  • Arrhythmias
  • Sterile valvular vegetations (Libman-Sacks endocarditis)
  • Neonates born to mothers w/ SLE
    • Congenital heart block
    • Secondary to transplacental passage of maternal antibodies
41
Q

Pulmonary involvement of SLE

A
  • Pleuritis
  • Pulmonary hemorrhage
  • Interstitial fibrosis
42
Q

Renal involvement of SLE

A
  • Nearly universal, lupus nephritis sub-clinical
  • Glomerulonephritis
  • Nephrotic syndrome
  • HTN
  • Subsequent renal failure
43
Q

Hematologic manifestations of SLE

A
  • Low WBC counts (leukopenia)
  • Anemia of chronic disease
  • Thrombocytopenia
  • Coombs+ hemolytic anemia
44
Q

What is the diagnostic criteria for SLE?

A

SOAP BRAIN MD

  • Serositis (pleuritis or pericardial inflammation)
  • Oral or nasal mucocutaneous ulcerations
  • Arthritis, non-erosive
  • Photosensitivity
  • Blood cytopenias
    • Leukopenia, hemolytic anemia, thrombocytopenia
  • Renal disease (hematuria, proteinuria, HTN)
  • ANA+
  • Immunoserology abnormalities
    • dsDNA Ab, anti-Smith An, false+ RPR or VDRL assays
  • Neuro symptoms
    • Encephalopathy, seizures, psychosis
  • Malar rash (butterfly rash)
  • Discoid lupus
45
Q

What are the laboratory findings of SLE?

A
  • Elevated ESR & CRP
  • Anemia of chronic disease or anemia secondary to hemolysis
  • Leukopenia
  • Thrombocytopenia
  • Urinalysis may show proteinuria
    • Depends on extent of renal disease
  • Rheumatologic markers
  • Antiphospholipid Ab = increased risk of thrombotic events
  • Decreased complement (C3, C4)
    • Active disease
    • Immune-complex mediated complement activation
46
Q

What are the rheumatologic markers of SLE?

A
  • ANA
    • Universally elevated (>95%)
    • Non-specific
  • RF
    • Elevated, non-specific
  • Anti-dsDNA Ab
    • Specific (only in SLE)
    • Used as markers for active disease, especially nephritis
  • Anti-Smith Ab
    • Less prevalent, but very specific
    • Can’t be used as measure of disease activity
47
Q

How is SLE managed?

A
  • Control of inflammation
    • NSAIDs - myalgias & arthralgias
    • Immunosuppressive meds
      • Glucocorticoids: mainstay of therapy; low-dose oral or high-dose IV pulse
      • Cyclophosphamide: IV pulse; severe lupus nephritis; adverse effects include infertility & gonadal failure, secondary malignancies, hemorrhagic cystitis
      • Azathioprine, MTX, cyclosporine
  • Treatment of complications
    • Thrombosis & anti-phospholipid Ab: anticoagulation w/ LMWH or warfarin
    • Renal failure: dialysis, fluid & electrolyte management, renal transplant
  • Psychosocial & family support
48
Q

What is the prognosis of SLE?

A
  • Survival rate 90% at 5-10 yrs after diagnosis
  • Major causes of mortality
    • Infection
    • Renal failure or nephritis
    • CNS complications
49
Q

Dermatomyositis

definition

epidemiology

A
  • Inflammatory condition of muscle
  • Progressive muscle weakness w/ characteristic skin findings
  • 5-14 YO, mean age of onset 6 YO
  • Female:male = 2:1
50
Q

What are the top 3 clinical features of Dermatomyositis?

A
  • Constitutional symptoms
    • Fatigue, anorexia, malaise, low-grade fevers, weight loss
  • Cutaneous findings (sun-exposed areas)
  • Proximal muscle weakness
51
Q

What are the characteristic cutaneous findings of Dermatomyositis?

A
  • Periorbital violaceous heliotrope rash
    • May cross nasal bridge
  • Gottron’s papules
    • Skin over MCP/PIP joints
    • Erythematous & hypertrophic
52
Q

Describe the proximal muscle weakness associated w/ Dermatomyositis

A
  • Hip girdle & legs
  • Insidious in onset
  • Wks-mo after eruption of skin findings
  • +Gowers’ sign
    • Difficulty standing from the sitting position & having to “climb” up the thighs for support
53
Q

What are 6 other common manifestations of Dermatomyositis?

A
  • Neck flexor muscle weakness
  • Calcinosis
    • Ca deposition in muscle, fascia & subq
    • 40% of children
  • Nail bed telangiectasias
  • Constipation
    • GI smooth muscle dysfunction
  • Dysphagia
  • Cardiac involvement
    • Conduction abnormalities
    • Dilated CM
54
Q

How is Dermatomyositis diagnosed?

A
  • Classic clinical presentation
    • Proximal muscle weakness
    • Characteristic rashes
  • Abnormal electromyography findings
  • Abnormal muscle biopsy findings
  • Increased muscle enzymes
    • Creatinine phosphokinase
    • AST, ALT, LDH, aldolase
55
Q

How is Dermatomyositis managed?

A
  • Mainstay of therapy: corticosteroids
  • Other immunosuppressive agents (severe)
    • MTX
    • Cyclophosphamide
    • Cyclosporine
  • Vitamin D & Calcium supplementation
    • Repair osteopenia
    • Decrease fracture frequency
56
Q

What are some complications of Dermatomyositis?

A
  • Aspiration pneumonia
    • Diminished gag reflex
  • Intestinal perforation
    • GI vasculitis
  • Osteopenia
    • Steroid therapy & muscle weakness
    • Frequent fractures
57
Q

What is the prognosis of Dermatomyositis?

A
  • Children > adults
  • No associated w/ malignancy in pediatric dermatomyositis (25% in adults)
  • Mortality rate ~3%
58
Q

What is the definition of Rheumatic Fever?

A
  • Delayed, nonsuppurative, autoimmune complication of URI w/ group A ß-hemolytic streptococcus (Streptococcus pyogenes)
  • Inflammation of the connective tissues
  • Heart, BV, joints, CNS, skin
59
Q

Rheumatic fever epidemiology

A
  • Rare in the US & Western Europe
    • Worldwide problem in 1960s
  • Children 5-15 YO
  • No gender predilection
  • Major risk factor
    • Pharyngitis caused by GABHS
    • Strep strains that cause skin infection (impetigo) DON’T cause RF
60
Q

The cause of Rheumatic Fever is ______.

A

Unknown

*appears to be autoimmune*

61
Q

What are the 4 major clinical features of Rheumatic Fever?

A
  • Cardiac involvement
  • Polyarthritis
  • Sydenham’s chorea
  • Skin involvement
62
Q

Cardiac involvement of Rheumatic Fever

A
  • 50% of patients
  • Hallmark & most important complication
  • Endocarditis
    • Most common cardiac finding
    • Insufficiency of L sided valves (mitral/aortic)
  • Myocarditis
    • Tachycardia out of proportion to extent of fever, cardiac dilatation, HF
  • Pericarditis
    • Pericardial effusions, less common
63
Q

Polyarthritis of Rheumatic Fever

A
  • Migratory, asymmetric, painful
  • 70% of patients
  • Elbows, knees, ankles, wrists
  • Does NOT result in chronic joint disease
64
Q

Sydenham’s chorea of Rheumatic Fever

A
  • Occurs later, begins subtly, months after GABHS pharyngitis
  • Reflects involvement of the basal ganglia & caudate nuclei
  • Hand clumbsiness –> choreoathetoid movements w/ emotional lability
65
Q

Skin involvement of Rheumatic Fever

A
  • Erythema marginatum
    • Nonpruritic rash, pink to red macules
    • Coalesce & spread centripetally w/ central clearing over the trunk & proximal limbs
  • Subcutaneous nodules
    • Rarely seen, associated w/ severe cardiac involvement
    • Small, mobile, painless nodules
    • Bony prominences of the extensor surfaces of the extremities
66
Q

What are the minor features of Rheumatic Fever?

A
  • Fever
  • Arthralgias
  • Leukocytosis
  • Increased ESR
  • Prolonged PR interval on ECG
67
Q

How is Rheumatic Fever diagnosed?

What is the Jones Criteria?

A
  • Evidence of recent strep infection & 2 major criteria or 1 major + 2 minor criteria
  • Major criteria
    • Migratory polyarthritis
    • Carditis
    • Sydenham’s chorea
    • Erythema marginatum
    • Subcutaneous nodules
  • Minor criteria
    • Fever
    • Arthralgia
    • Previous rheumatic fever
    • Leukocytosis
    • Elevated ESR
    • Elevated CRP
    • Prolonged PR interval on ECG
68
Q

What are the laboratory findings of Rheumatic Fever?

A
  • Nonspecific inflammatory markers
    • Elevated ESR & CRP
    • Elevated WBC count
  • Serologic markers
    • Anti-streptolysin-O titers
      • Abnormally elevated (70-80%)
      • Evidence of recent GABHS infection
    • Anti-DNase & anti-hyaluronidase Ab
      • Document GABHS infection
  • ECG shows evidence of carditis
    • Decreased ventricular function
    • Valvular insufficiency
    • Pericardial effusion
69
Q

Management of Rheumatic Fever

What drugs are used?

Supportive therapy?

A
  • Eradication of GABHS infection
    • Benzathine penicillin IM (one dose)
    • Penicillin orally for 10 days
  • Control of inflammation
    • NSAIDs for joint pain/swelling
      • Only after diagnosis of RF certain
    • Corticosteroids for severe cardiac
      • CHF, severe valvular dysfunction
  • Supportive therapy
    • CHF - diuretics, dietary salt restriction, digoxin, bed rest
    • Sydenham’s chorea - haloperidol
  • Long-term management
    • Continuous antimicrobial prophylaxis
70
Q

What is the prognosis of Rheumatic Fever?

A
  • No chronic sequelae of the joint, skin & CNS manifestations
  • Cardiac inflammation
    • Severe valvular dysfunction: intervention immediately or yrs later
    • Valvular insufficiency/stenosis: delayed (>3 yrs after RF), valve replacement or valvuloplasty
71
Q

What is Lyme Disease?

A
  • Reactive inflammatory disorder of the skin, heart, CNS & connective tissues
  • Spirochetal infection w/ Borrelia burgdorferi
  • Transmitted via tick bite
72
Q

What is the epidemiology of Lyme Disease?

A
  • High risk areas reflect the natural habitat of ticks of the Ixodes species (vectors)
  • Woodlands & fields
  • New England states, Pacific coast, Midwest
73
Q

What is the etiology of Lyme Disease?

Vector? Organism?

A
  • Vector
    • Deer tick: Ixodes scapularis (US)
    • Ixodes pacificus (Western US, less common)
  • Organism
    • B. burgdorferi
    • Bloodstream of human host while tick engorges itself w/ blood
    • Infected tick must be attached >36-48 hrs
74
Q

Clinical features of Lyme disease are caused by __________, and in later stages by _______.

A
  • Invasion of B. burgdorferi into local & distant tissues
  • Systemic inflammatory response against the spirochete
75
Q

What are the stages of Lyme disease?

A
  • Early disease (1-4 mo)
    • Early localized disease
      • Local cutaneous invasion
      • Subsequent inflammation
    • Early disseminated disease
      • Cutaneous findings
      • Other organ systems
  • Late disease (5-12 mo)
    • ​7% children
    • Arthritis
76
Q

Early localized disease of Lyme disease

A
  • Erythema migrans
    • 2/3 of patients
    • Annular & “targetlike” rash
    • Variable degrees of central clearing
    • Asymptomatic, pruritic, painful
    • May expand >12 in diameter
  • Constitutional symptoms
    • Fever, headache, myalgias, fatigue, arthralgias, lymphadenopathy
77
Q

Early disseminated disease of Lyme disease

A
  • Skin
    • 25% children
    • Multiple secondary erythema migrans lesions (smaller than initial lesion)
  • Constitutional symptoms
  • Neurologic
    • Aseptic meningitis (1%)
    • Facial nerve palsy (3%)
    • Encephalitis
  • Carditis (rare)
    • Heart block or myocarditis
78
Q

How is Lyme disease diagnosed?

A

Strongly suggested in the presence of the epidemiologic risk factors & classic erythema migrans rash

79
Q

Laboratory diagnosis of Lyme disease

A
  • Measurement of Ab to B. burgdorferi
  • Serologic testing
    • ELISA - high sensitivity
    • Western blot - confirm ELISA
  • Other lab tests offer no advantage
80
Q

Management of Lyme disease

A
  • Aimed at eradicating B. burgdorferi
  • Early localized disease/late disease w/ arthritis
    • Doxycycline (children _>_9 YO)
    • Amoxicillin
  • Carditis & meningitis
    • IV ceftriaxone or penicillin
81
Q

What is the prognosis of Lyme Disease?

A
  • Children treated have excellent prognosis
  • Recurrent symptoms or chronic sequelae rare
82
Q

What are the seronegative spondyloarthropathies?

A
  • Reactive arthritis
  • Psoriatic arthritis
  • Ankylosing spondylitis
  • Arthritis of IBD
83
Q

What is Reactive arthritis?

A
  • Inflammation of the joints triggered by a microorganism
  • Enteric or sexually transmitted pathogen
  • Reiter’s disease
    • Arthritis, uvethritis, conjunctivitis
    • Chlamydia trachomatis
84
Q

What is Psoriatic arthritis?

A
  • Arthritis of the small & large joints
  • Patients w/ psoriatic skin disease
  • Scaly skin plaques, nail pitting, onycholysis (separation of the nail from the nailbed)
85
Q

What is Ankylosing spondylitis?

A
  • Male-predominant, HLA-B27-related
  • Joints of the LE & axial skeleton
  • Enthesitis: inflammation of the tendinous insertions on the bone
  • High risk: males w/ late-onset pauciarticular JRA
86
Q

What is arthritis of IBD?

A
  • Ulcerative colitis, Crohn’s disease
  • Some patients HLA-B27+
    • Involvement of axial skeleton
    • Pattern indistinguishable from ankylosing spondylitis
87
Q

What are 3 examples of rheumatologic vasculitides?

A
  • Takayasu’s arteritis
  • Polyarteritis nodosa
  • Wegener’s granulomatosis
88
Q

What is Takayasu’s arteritis?

A
  • Large vessel vasculitis
  • Asian female adolescent or young adult
  • Constitutional symptoms & aneurysmal dilatation or thrombosis of the aorta, carotid or subclavian arteries
89
Q

What is Polyarteritis nodosa?

A
  • Aneurysms & thrombosis of the small & medium-sized vessels
  • Brachial, femora, mesenteric arteries
90
Q

What is Wegener’s granulomatosis?

A
  • Necrotizing granulomas in multiple organs
    • Respiratory tract
    • Kidneys
  • Constitutional symptoms
  • Severe sinusitis
  • Hemoptysis
  • Glomerulonephritis
91
Q

What is Sjogren’s syndrome?

A
  • Classic triad
    • Sicca syndrome (dry mouth & eyes)
    • High titers of autoAb (ANA, RF)
    • Connective tissue disease
92
Q

What are the two types of scleroderma?

A
  • Systemic scleroderma
  • CREST syndrome
93
Q

What is Systemic Scleroderma?

A
  • Systemic sclerosis
  • Excessive fibrosis & subsequent dysfunction of multiple organ systems
  • Skin & vessels of the heart, kidneys, lungs, GI
  • Hallmark: skin thickening w/ loss of dermal ridges, resembling “tightened” skin
94
Q

What is CREST syndrome?

A
  • Calcinosis
  • Raynaud’s phenomenon
  • Esophageal involvement
  • Sclerosis of the skin
  • Telangiectasias

Pediatrics (24 decks)

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