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Pediatrics > Rheumatology > Flashcards

Rheumatology Flashcards

1
Q

How long should you give penicillin prophylaxis in rheumatic fever?

A
  • Rheumatic fever, no evidence of carditis, no evidence of residual heart disease: 5 years or until age 21, whichever is longer
  • Rheumatic fever, + evidence of carditis, no evidence of residual heart disease: 10 years or until age 21, whichever is longer
  • Rheumatic fever, + evidence of carditis, + evidence of residual heart disease: 10 years or until age 40, whichever is longer
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2
Q

Diagnostic Criteria for Systemic Lupus Erythematosus

A

“MD SOAP BRAIN”
Malar rash
Discoid rash

Serositis
Oral and mucosal ulcers (painless)
ANA positive
Photosensitivity

Blood findings: thrombocytopenia, leukopenia, hemolytic anemia
Renal disease i.e. proteinuria, hematuria, biopsy findings
Arthritis (non-erosive) in 2 or more joints
Immunological findings: +dsDNA, +anti-smith, False positive RPR, +ve lupus anticoagulant test result, elevated anticardiolipin immunoglobin IgG or IgM antibody
Neurological changes i.e. behaviour, seizures

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3
Q

What is the diagnostic criteria for Rheumatic Fever?

A

Positive ASO or GAS throat culture + [2 major or 1 major and 2 minor criteria]

Major Criteria: "JONES"
Joints - migrating arthritis
O - myOcarditis
Nodes - nodular findings such as EN
Erythema marginatum 
Syndenham's Chorea
Minor Criteria: "CAFE PAL"
CRP elevation
Arthralgia
Fever
ESR elevated

PR prolongation
Anamnesis of rheumatism
Leukocytosis

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4
Q

What should be given as prophylaxis in rheumatic fever?

A

Pen G 600 000 IU IM (children ≤60 lbs) q 4 weeks

Penicillin V 250 PO BID

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5
Q

What are the long term consequences of rheumatic fever

A
  • mitral valve or aortic valve regurgitation (acute)

* mitral or aortic stenosis

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6
Q

Diagnostic criteria for Kawasaki Disease

A

“Warm CREAM”

• Fever x 5 days

  • Conjunctivitis (bilateral, non-purulent)
  • Rash (mobiliform, maculopapular)
  • Erythema and desquamation of hands and feet
  • Adenopathy (at least greater than 1 cm, usually asymmetric)
  • Mucous membrane swelling/redness (i.e. strawberry tongue)
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7
Q

Treatment for Kawasaki Disease (acute and long term)

A
  • IV Ig 2g x 1 dose given over 8-12 hours

* Low dose ASA 3-5 mg/kg/day

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8
Q

Incomplete Kawasaki Disease criteria

A
  • Sterile pyuria
  • Hypoalbuminemia
  • Normocytic Anemia
  • Elevated CRP/ESR
  • Hyperferritinemia
  • Leukocytosis with left shift
  • Hyponatremia (higher risk CAA)
  • (50% have) Elevated transaminases
  • Abnormal lipids (>TG, >LDL,
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9
Q

What follow up should be done for a child with Kawasaki Disease?

A
  • ECHO - at diagnosis, at 6 weeks
  • ASA - daily until fevers stop x 24 hours
  • No immunizations until 11 months after dose of IV IG
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10
Q

Diagnostic features of Macrophage Activating Syndrome

A
  • Fever (continuous/persistent)
  • Splenomegaly
  • Cytopenias (anemia, thrombocytopenia, neutropenia) • Elevated triglycerides
  • Decreased fibrinogen
  • Elevated ferritin
  • Hemophagocytosis on bone marrow, lymph node, liver or spleen biopsy
  • Persistently raised CRP, but decreasing ESR
  • Pronlonged INR/PTT (like DIC)
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11
Q

What are the clinical features of Familial Mediterranean Fever?

A

Clinical diagnosis “FARS”
• Fever episodes last for 1-3 days and occur without true periodicity
• Arthritis - Monoarthritis, myalgia
• Rash - Erysipelas-like on shins and dorsum of feet (consider if recurrent erysipelas-like erythema)
• Serositis (peritonitis, pleuritis, synovitis) – severe pain, often unilateral
- Asx between episodes

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12
Q

Diagnostic Criteria for Behçet Disease

A

• Recurrent oral ulceration + 2 of the following:

  • Recurrent genital ulcers
  • Eye lesions (anterior/posterior uveitis, retinal vasculitis)
  • Skin lesions (erythema nodosum, pseudofolliculitis or papulopustular lesions)
  • Pathergy (skin reaction to a needle prick observed by physician at 24-48 hours)
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13
Q

Diagnostic Criteria for Oligoarthritis

A
  • Arthritis in 4 or less joints in the first 6 months of disease (usually knees, ankles, wrists and elbows)
  • Persistent type - 4 or less joints involved throughout disease course
  • Extended type - more than 4 joints involved after the initial 6 month period

Note: not needed for diagnosis, but these patients are classically young girls, ANA+ and at risk for uveitis

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14
Q

Diagnostic Criteria for Systemic Juvenile Idiopathic Arthritis

A

• Arthritis in at least 1 joint for 6 weeks
• Associated with or preceded by fever of at least 2 weeks’ duration that is documented to be daily, or “quotidian” for at least 3 days
• Accompanied by 1+ of:
○ Evanescent (non-fixed) erythematous rash - salmon-coloured, evanescent rash
○ Generalized lymph node enlargement
○ Hepatomegaly and/or splenomegaly
○ Serositis

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15
Q

Diagnostic Criteria for RF+ Polyarthritis

A
  • Arthritis in at least 5 joints for 6 weeks

* At least 2 positive RF tests at least 3 months apart during the first 6 months of illness

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16
Q

Diagnostic Criteria for RF- Polyarthritis

A
  • Arthritis in at least 5 joints for 6 weeks

* Rf negative testing

17
Q

Diagnostic Criteria for Enthesitis Associated Arthritis

A

• Arthritis and enthesitis OR
• Arthritis or enthesitis and 2 of the following:
○ Presence of HLA-B27
○ History of ankylosing spondylitis, enthesitis related arthritis, sacroiliitis with inflammatory bowel disease or acute anterior uveitis in a first-degree relative
○ Onset of arthritis in a male over 6 years of age
○ Presence or history of SI joint tenderness and/or inflammatory back pain
○ Acute (symptomatic) anterior uveitis

18
Q

Diagnostic Criteria of Psoriatic Arthritis

A

• Arthritis in at least 1 joint with psoriasis
• Arthritis with at least 2 of the following:
○ Dactylitis
○ Nail-pitting or onycholysis
○ Psoriasis in a first-degree relative

19
Q

How do you best prevent adverse reactions from IV Ig?

A
  • Give for correct indications (minimize use)
  • Give by SC/IM (less rxn)
  • Slow infusion
  • Pre-medicate if HA develops
  • Ensure hydration before
20
Q

What are immediate adverse reactions to IV Ig?

A

• Rate-related reactions
○ Chills, fever, flushing, aches
○ Anaphylaxis-like reaction (but >BP not

21
Q

What are late adverse reactions to IV Ig?

A
  • Thromboembolic events (in those with >age, >dose)
  • H/A (often >1d long)
  • +/- aseptic meningitis
  • AKI: Rare (<1%); ?osmotic mechanism
  • HypoNa: Rare; dilutional mechanism
  • Hemolysis: Isoagglutinins (e.g. antiRh(D), Anti-A, Anti-B) from IVIG
  • Neutropenia: Often transient
  • Dermatologic (?vesicular rash)
  • NEC, ileitis, uveitis, hypothermia, non infxn hepatitis, serum sickness
  • Impair response to live virus
  • (Transiently +ve serologic tests for Ab – e.g. ANA, ANCA, RF – IgG half life 21-28d, so by 60d they should clear)
22
Q

Diagnostic Criteria for Granulomatosis with Polyangitis

A

At least 3/6 of the following:
• Renal involvement (proteinuria >0.3 g in 24 hrs, hematuria, or red blood cell casts, impaired renal function)
• ANCA positive
• Upper airway involvement (chronic purulent or bloody nasal discharge, recurrent epistaxis, nasal septum perforation, saddle nose deformity, chronic or recurrent sinus inflammation)
• Laryngo-tracheo-bronchial involvement (subglottic, tracheal or bronchial stenoses)
• Pulmonary involvement (nodules, cavities, or fixed pulmonary infiltrates)
• Histopathology showing granulomatous inflammation within wall of artery or in perivascular or extravascular area

23
Q

What is the classic triad of granulomatosis with polyangitis?

A
  • Small vessel vasculitis with granulomatous inflammation
  • Second decade of life, with a female preponderance

Triad of

1) Upper resp tract inflammation
2) Lower resp tract inflammation
3) Renal disease
24
Q

How do you diagnose Juvenile Dermatomyositis?

A

Must have characteristic skin changes (i.e. Gottron papules, heliotrope rash) and at least 3/4 of:
• Symmetrical proximal muscle weakness
• Elevated muscle enzymes (CK, AST, LDH, aldolase)
• Abnormal EMG demonstrating denervation and myopathy
• Abnormal muscle biopsy demonstrating necrosis and inflammation

25
Q

Provide a differential diagnosis for proximal muscle weakness

A
  • Polymyositis
  • Infection-related myositis (influenza A and B, coxsackievirus B)
  • Muscular dystrophies
  • Myasthenia gravis
  • GBS
  • Endocrinopathies (hyperthyroidism, hypothyroidism, Cushing syndrome, Addison disease, parathyroid disorders)
  • Mitochondrial myopathies
  • Metabolic disorders (glycogen and lipid storage diseases
  • Infections associated with prominent muscular symptoms: Bartonella, staph, toxoplasmosis
  • Vaccinations, drugs, growth hormone, and GVHD
26
Q

What are the “3 D’s of Juvenile Dermatomyositis”?

A

3D’s: dysphagia, dysphonia, and dyspnea

Note: Children with respiratory muscle weakness do not manifest with WOB
Hypercarbia rather than hypoxia

27
Q

What are some systemic inflammatory diseases associated with Uveitis?

A
  • All forms of JIA
  • Behcet disease
  • Infantile sarcoidosis
  • Kawasaki disease
  • Tubulo-interstitial nephritis and uveitis
28
Q

What medication classes are implicated in Drug Induced Lupus?

A

Biologics
Antiepileptics
Betablockers
Antibiotics

Pediatrics (27 decks)

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