Rheumatology Flashcards
How long should you give penicillin prophylaxis in rheumatic fever?
- 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
Diagnostic Criteria for Systemic Lupus Erythematosus
“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
What is the diagnostic criteria for Rheumatic Fever?
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
What should be given as prophylaxis in rheumatic fever?
Pen G 600 000 IU IM (children ≤60 lbs) q 4 weeks
Penicillin V 250 PO BID
What are the long term consequences of rheumatic fever
- mitral valve or aortic valve regurgitation (acute)
* mitral or aortic stenosis
Diagnostic criteria for Kawasaki Disease
“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)
Treatment for Kawasaki Disease (acute and long term)
- IV Ig 2g x 1 dose given over 8-12 hours
* Low dose ASA 3-5 mg/kg/day
Incomplete Kawasaki Disease criteria
- 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,
What follow up should be done for a child with Kawasaki Disease?
- ECHO - at diagnosis, at 6 weeks
- ASA - daily until fevers stop x 24 hours
- No immunizations until 11 months after dose of IV IG
Diagnostic features of Macrophage Activating Syndrome
- 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)
What are the clinical features of Familial Mediterranean Fever?
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
Diagnostic Criteria for Behçet Disease
• 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)
Diagnostic Criteria for Oligoarthritis
- 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
Diagnostic Criteria for Systemic Juvenile Idiopathic Arthritis
• 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
Diagnostic Criteria for RF+ Polyarthritis
- 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
Diagnostic Criteria for RF- Polyarthritis
- Arthritis in at least 5 joints for 6 weeks
* Rf negative testing
Diagnostic Criteria for Enthesitis Associated Arthritis
• 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
Diagnostic Criteria of Psoriatic Arthritis
• 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
How do you best prevent adverse reactions from IV Ig?
- Give for correct indications (minimize use)
- Give by SC/IM (less rxn)
- Slow infusion
- Pre-medicate if HA develops
- Ensure hydration before
What are immediate adverse reactions to IV Ig?
• Rate-related reactions
○ Chills, fever, flushing, aches
○ Anaphylaxis-like reaction (but >BP not
What are late adverse reactions to IV Ig?
- 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)
Diagnostic Criteria for Granulomatosis with Polyangitis
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
What is the classic triad of granulomatosis with polyangitis?
- 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
How do you diagnose Juvenile Dermatomyositis?
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
Provide a differential diagnosis for proximal muscle weakness
- 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
What are the “3 D’s of Juvenile Dermatomyositis”?
3D’s: dysphagia, dysphonia, and dyspnea
Note: Children with respiratory muscle weakness do not manifest with WOB
Hypercarbia rather than hypoxia
What are some systemic inflammatory diseases associated with Uveitis?
- All forms of JIA
- Behcet disease
- Infantile sarcoidosis
- Kawasaki disease
- Tubulo-interstitial nephritis and uveitis
What medication classes are implicated in Drug Induced Lupus?
Biologics
Antiepileptics
Betablockers
Antibiotics
