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88 Cards in this Set
- Front
- Back
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Kawasaki's disease also known as -
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Mucocutaneous lymphnode diease
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Some common facts about Kawasaki's disease -
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Multi-system vasculitis syndrome
Afflicting infants and children Similar to infantile Periarteritis Nodosum Most common cause of acquired heart disease in US and Asia |
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Pathogenesis of Kawasaki's disease -
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Immune dysregulation - Cytokines released from hyper activated stimulated B and T cells with autoantibody production and enhanced adhesion molecules expression resulting in enodthelial cell damage..
Super antigens - nonspecific immune response stimulated CD8 cells predominate in arterial walls on patients along with oligoclonal IgA plasma cells May be assoc with propionibacterium acne |
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Genetic polymorphism of Kawasaki's disease -
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HLA Bw 22
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Who does Kawasaki disease most commonly present in?
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Children between age of 6mo - 5yrs
Asian > other ethnicities Boys> Girls |
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Diagnosing criteria for Kawasaki's disease -
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Spiking fever of 102 atleast 3-5 days
Red palm and soles (along with edema of the same) Diffused cervical lymphadenopathy Strawberry tongue Conjunctivitis ( no exudate) |
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What is most common complication of Kawasaki's disease?
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Coronary artery aneurysms - destruction of elastic intima of coronary vessels leading to the aneurysms
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Some laboratory findings in Kawasaki's disease -
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Profound thrombocytosis ( <1mil)
high sed rate along with high IgE levels |
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Definition of Acute Rheumatic Fever -
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Nonsuppurative sequela of S. pyogenes infection..NO PUS INVOLVED
Acute, febrile, inflammatory condition - involving heart, CNS, skin and subcutaneous tissue |
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Etiology of Strep pyogenes -
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Group A, b-hemolytic, step pyogenes.
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What kind of strep infection usually leads to ARF?
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Only throat or pharyngeal strep - not skin or other types
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Main difference between staph and strep as far as spread of infection -
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Staph - localizes and forms abscesses
Strep - spreads throughout subcutaneous tissue |
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Serogroups
Serotypes |
Serogroups - for cell wall carb (A-T)
Serotypes - for M protein (1-100) |
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Some exotoxins produced by Group A Strep -
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SLO
Erythrogenic toxins (A,B,C) DNA ases Hyaluronidase Streptokinase NADase |
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Which titer usually goes up after Strep infection?
What if it doesn't go up ? |
ASO - which measure host (human) Ab to this particular exotoxin.
If it doesn't go up and throat culture is positive, colonized infection, but no invasion... |
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Mechanism behind most of pathogenic cross reactivity of Strep infection -
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Molecular mimicry
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Some examples of strep cross reactivity -
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Hyaluronic acid capsule and articular synovial tissue
M protein and myocardial tissue Strep protoplast membrane and human brain tissue Cell wall carbohydrate and valvular glycoprotein ARF pts have increased reactivity to strep cell membranes |
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Genetic predesposition to ARF -
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HLA-DR 8/17 over expression
higher incidence in families and twins |
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What age group do ARF and Kawasaki usually target at?
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ARF - between 5-15 yrs
Kawasaki - <5 yrs Both target children more than adults/adolescents |
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Why is ARF so prevalent?
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because 50% of cases are due to mild/sub clinical infection which is not treated so pts don't go to physician
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Two M strains assoc with RF
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M5 and M18
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5 major criteria for RF
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Subcutaneous nodules
Polyarthritis, migratory Erythema marginatum (margins - bright red) Chorea, syndeham's Carditis (MS, MS/AS, AS, TS) no regurg - only fibrosis |
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Treatment for Acute Streptococcal pharyngitis -
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penicillin or erythromycin
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What is Jones criteria for diagnosis of initial ARF
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1 evidence of preceding Group A strep infection + 2 major manifestations.
1 major + 2 minor manifestations.. 1 supporting + 1 major + 2 minor |
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What is included in major manifestations for Jones ARF criteria?
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Carditis
Polyarthritis Chorea Erythema marginatum Subcutaneous nodules |
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What is included in minor manifestations for Jones ARF criteria?
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Arthralgia
Fever - non specific Lab findings - elevated acute phase reactants, ESR, C-reactive protein increased PR interval |
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Supporting evidence of Antecedent Group A streptococcal infection?
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Positive throat culture or rapid strep test
Elevated or rising streptococcal antibody titer (physician diagnosed scarlet fever) |
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What are two large vessel vasculitis?
What do you look for? |
Temporal (Giant Cell) Arteritis
Takayasu Arteritis Look for absent pulse or stroke |
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Vessels affected by Giant Cell Temp Arteritis -
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superficial temporal and opthalmologic arterities aka cranial arteries (facial artery also involved sometimes)
(fragmentation of internal elastic lamina of cranial arteries in affected segments) |
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Histologic appearance of GCA -
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segmental inflammation(“Skip lesions” i.e. patchy involvement of vessel) with granulomas (histiocytes, lymphocytes, and giant cells)
Destruction of internal elastic lamina |
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Clinical presentation of GCA -
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headache with jaw claudication, facial pain, tenderness over arteries, visual disturbances (can progress to blindness)
Scalp tenderness (hurts while putting on glasses or washing hair) Pt usually middle aged to elderly women with elevated ESR flu like pain and muscle weakness because of Polymyalgia rheumatica |
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Dz assoc with GCA -
HLA Assoc |
Polymyalgia rheumatica (proximal muscle and joint pain along with flu like symptoms)
Assoc with HLA DR4, ICAM-1 R241 polymorphism Most COMMON form of vasculitis |
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Why does GCA need prompt attention?
How is diagnosis made? |
can lead to permanent blindness if not treated right away
Mostly Transient monocular blindness (TMB; also called amaurosis fugax) Do a biopsy and multiple sections of artery inflammed (if only do one section, may miss) Have a higher ESR |
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Tx of GCA:
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prednisone
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Takayasu Arteritis affects which vessels
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aortic arch vessels and major branches
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Histologic appearance of vessels in Takayasu arteritis -
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Granulomatous vasculitis with intimal fibrosis of aortic branches and other medium and large sized arteries
Produced characteristic narrowing of arterial orificies |
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Clinical presentation of Takayasu arteritis -
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loss of pulse in upper extremities, pulses in lower intact, fever, night sweats, muscle and join aches, aortic valve insufficiency
May lead to visual loss and other neuro. abnL young to middle aged Asian women more affected (15-45) Systemic: fever, malaise, weight loss, arthralgia and myalgia Vascular: limb ischemia (claudication) Subclavian steal syndrome Organ failure |
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Examples of some middle vessel vasculitis -
What to look for in them? |
PAN
Wegner's granulomatosis Thromboangitis obliterans (Buerger's disease) Raynaud's phenomenon Kawasaki's dz |
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Vessels affected by PAN -
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Spares pulmonary arteries
Small and medium sized arteries in skin, joints, peripheral nerves, kidney, heart and GI tract |
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Pathology in PAN -
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Acute necrotizing transmural inflammation with lots of PMNs, eosinophils, and mononuclear cells (FIBRINOID NECROSIS)
Ischemia or aneurysms of above mentioned arteries - may be episodic Any organ can be affected and infarct typically occur |
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What is PAN associated with?
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P-ANCA (Perinuclear anti neutrophil cytoplasmic antibodies)
HBsAg (Hep B surface antigen) Hairy cell leukemia (10%) |
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Wegner's granulomatosis affects which vessels -
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Upper resp tract (nose, sinuses)
lungs kidneys |
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Pathology in Wegner's granulomatosis -
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necrotizing vasculitis with granulomas
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Clinical presentation of Wegner's granulomatosis -
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triad of -
Cough, Hemoptysis, Hematuria see sinusitis, saddle nose, glomerulonephritis May also see nasopharyngeal ulcerations, renal dz(focal necrotizing glomerunephritis) |
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Strongest assoc of Wegner's granulomatosis with -
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c-ANCA
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Thromboangiitis Obliterans aka Buerger's disease affects which vessels -
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Neutrophilic vasculitis that affects vessels leading to digits (especially tibial and radial arteries)
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Buerger's disease pathology -
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Involves small and medium sized arteries and veins in extremities
Microabscesses and segmental thrombosis lead to vascular insufficiency, ulceration, and gangrene |
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Clinical presentation of Buerger's disease -
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ischemic/gangrenous looking fingers and pain (clinically severe pain and Raynaud phenomenon)
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Who is Buerger's disease mostly found in ?
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Smokers!!!
If diagnosed, must stop all forms of tobacco or may lose fingers. |
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Raynaud's disease and phenomenon - difference between two
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Dz - primary
Phenomenon - assoc w/other dz |
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Vessels affected by Raynaud's
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vasospasm in fingers and toes
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Pathology in Raynaud's
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ulceration/gangrene in chronic cases
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Clinical presentation of Raynaud's -
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Vasoconstrictive response to cold/stress in fingers
White (no flow) Blue (some return) Red (influx of blood) |
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Dz assoc in Raynaud's phenomenon -
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SLE, scleroderma, CREST, Buerger's disease
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What is contraindicated in treatment of Kawasaki's dz -
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treatment with corticosteroids - as they increase risk due to rupture of coronary vessel
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Kawasaki dz affects -
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Segmental necrotizing vasculitis involves large, medium-sized and small arteries.
Coronary arteries commonly affected (70%) |
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Examples of small vessel vasculitis
Look for - |
Microscopic polyangitis
Churg-Strauss syndrome Henoch-Schonlein purpara |
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Vessels affected in microscopic polyangitis
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small vessels (capillaries/venules) mostly in skin, but also in lungs, brain, GIT
Resembles Wegner's granulomatosis but NO granulomas. |
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Another name for microscopic polyangitis -
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hypersensitivity vasculitis or leukocytoclastic vasculitis
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Pathology in microscopic polyangitis -
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Segmental fibrinoid necrosis of media
Some vessels show infiltration with fragmented PMNs = leukocytoclastic angitis |
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Clinical presentation of microscopic polyangitis -
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palpable purpara ( a bruit you can palpate)
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Dz assoc with microscopic polyangitis -
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hypersensitivity reaction to DRUGS, infection, immune diseases
Assoc with p-ANCA 80% ANCA +ve 60% P-ANCA +ve 40% C-ANCA +ve Rarely see immune complexes |
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Churg-strauss syndrome
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small vessel vasculitis of skin, lungs, heart, spleen
Assoc with allergic rhinitis and asthma, and granulomas. Variant of PAN - another dz where p-ANCA is seen (EOSINOPHILIA) |
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Henoch-Schonlein purpara -
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small vessel vasculitis of skin, GIT, joints, and kidneys
Assoc with IgA immune complex deposition - purparas of lower buttocks, polyarthritis and glomeuronephritis of kidneys |
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What is PMR?
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Polymyalgia rheumatica
Common in elderly patients Higher prevalence in Northern latitudes Bilateral shoulder and hip pain Severe AM stiffness, gel phenomenon (Morning stiffness due to edema in the joints) Often acute or sub-acute onset Usually high ESR |
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What is gel phenomenon?
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have a hard time getting up once you sit down..seen in PMR (get this all of a sudden)
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What is PMR-GCA Relationship?
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PMR seen in up to 50% of patients with GCA
GCA found in 15-25% of patients with PMR |
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What is Behcet's syndrome?
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Chronic relapsing inflammatory disease
Most common in middle eastern and Asian populations Recurrent oral ulcers -multiple -3 or more episodes/yr Associated systemic vasculitis |
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What are systemic features of Behcet's syndrome?
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Genital ulcers
Ocular disease (uveitis) Skin lesions -Palpable purpura -Nodules -Pathergy Neurologic Vascular -Venous thrombosis -Arterial aneurysm Arthritis |
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What are 3 clinical phases in Churg Strauss Syndrome?
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Prodromal
-Chronic asthma -Atopic dermatitis -Allergic rhinitis Eosinophilic -Pulmonary opacities -Eosinophilia and eosinophil infiltration of organs, esp. GI tract and lungs Vasculitic -Cutaneous and internal organ vasculitis and granuloma formation |
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What is appearance on CXR for Churg Strauss syndrome?
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Multiple opacities on chest x-ray of patient with Churg-Strauss Syndrome
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How is Dx made for Churg Strauss syndrome?
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Eosinophilia (may be transient or suppressed by corticosteroids)
Positive ANCA => p-ANCA Transient pulmonary opacities Biopsy of affected tissues (lungs, sural nerve) |
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What are some complications in Churg Strauss syndrome?
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Cardiac failure and/or myocardial infarction
Cerebral hemorrhage Renal failure Gastrointestinal bleeding Status asthmaticus |
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Prognosis and Tx of Churg Strauss syndrome?
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Uniformly fatal without corticosteroids
Cytoxan & other immune suppressants for resistant cases |
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What are some clinical signs seen in Henoch Schonlein Purpura?
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Palpable purpura (100%)
Arthritis (82%) Abdominal pain (63%) Renal disease (40%) GI bleeding (33%) |
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Who is HSP mostly seen in?
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Children
-50% under 5 years old Often follows URI Renal disease more severe in older children and adults |
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What ICs are seen in HSP?
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IgA
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How is prognosis in HSP?
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Prognosis
Usually self-limited Complete recovery >90% patients |
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What is cryoglobulinemia?
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Circulating immunoglobulins that precipitate at low temperatures
Decreased blood flow -Raynaud’s phenomenon -Acral cyanosis, ulceration and necrosis |
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What is cryoglobulinemia associated with?
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Hepatitis C associated in 90% (Type II)
Mixed cryoglobulins consisting of monoclonal immunoglobulin complexed with polyclonal IgG |
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When does one suspect Vasculitis?
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Palpable purpura
Ischemic changes in absence of risk factors for atherosclerosis Glomerulonephritis or pulmonary-renal syndrome Multisystem disease (especially in a younger patient) Stroke in a young person Mononeuritis multiplex Unexplained constitutional symptoms (weight loss, malaise, fatigue) or fever |
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What is diagnostic approach to vasculitis?
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Labs
Comprehensive evaluation for internal organ involvement Biopsy Angiography |
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Which vasculitis have ICs involved?
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HSP
Cryoglobulinemia |
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Which are most common vasculitis?
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Hypersensitivity vasculitis (all ages)
GCA (elderly) |
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Which vasculitis involves children?
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Kawasaki's dz
HSP |
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What is isolated CNS Vasculitis?
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GANS (granulomatous angitis of nervous system)
Global CNS dysfunction Headache Cranial neuropathy STRAINS ON PEARLS |
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How is dx of isolated CNS vasculitis made?
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Elevated ESR
Lumbar Puncture (high protein, pressure, wbcs, nL glucose) MRI Angiography |
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Clinical features in PAN -
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Rash: purpura, nodules
Mononeuritis multiplex Abdominal pain Systemic: malaise, weight loss, fever, arthralgia Uncommon: CNS, orchitis, coronary artery vasculitis, RF Wrist dropping due to involvement of radial artery Aneurysms of arteries in legs Tender subcutaneous nodules |
