Treatment of choice of Kawasaki disease:
Ans. a. Intravenous immunoglobulin (Ref: Nelson 19/e p863; Harrison 19/e p2192, 18/e p2800; Fitzpatrick7/e p1626-1632)Treatment of choice of Kawasaki disease is intravenous immunoglobulin."Intravenous immunoglobulin (IV Ig) is given in Kawasaki disease, GBS and Myasthenia gravis."Kawasaki disease is generally common in children, not in adults.DiseaseAffected Age GroupTakayasu arteritisYounger than 50 yearsSusac syndromeAdults, 20-40 yearsHenoch-Scbonlein purpuraChildren and young adultsKawasaki diseaseChildren'Susac's syndrome (Retinocochleocerebral Vasculopathy) is a microangiopathy characterized by encephalopathy, branch retinal artery occlusions and hearing loss. The cause is unknown but the current thinking is that antibodies are cproduced against endothelial cells in tiny arteries, which leads to damage and the symptoms related to the illness. The syndrome usually affects women around the age of 18 years old, with female to male ratio of cases of 2:1.' Immune-Mediated VascuiitidesTypeExamplesDescriptionLarge vessel VasculitisGiant-cell(temporal)arteritisQ* Granulomatous inflammation: frequently involves the temporal arteryQ.* Usually occurs in patients older than age 50* Associated with polymyalgia rheumatica.Aorta and large branches to extremities, head and neckTakayasuarteritisQ* Granulomatous inflammation* Usually occurring in patients younger than age 50Medium vessel VasculitisPolyarteritisnodosaQ* Necrotizing inflammation* Typically involving renal arteries but sparing pulmonary vesselsQMain visceral arteries and their branchesKawasakidiseaseQ* Arteritis with mucocutaneous lymph node syndromeQ: usually occurs in childrenQ.* Coronary arteries can be involved with aneurysm formation and/or thrombosisQ.Small vessel VasculitisWegenergranulomatosis* Granulomatous inflammation involving the respiratory tract and necrotizing vasculitis affecting small vessels, including glomerular vessels.* Associated with PR3-ANCAsQ.Arterioles, venules, capillaries, and occasionally small arteriesChurg-Strausssyndrome* Eosinophil-rich granulomatous inflammation involving the respiratory tract and necrotizing vasculitis affecting small vesselsQ.* Associated with asthma and blood eosinophilia. Associated with MPO-ANCAsQ Microscopicpolyangitis* Necrotizing small-vessel vasculitis with few or no immune deposits; necrotizing arteritis of small and medium-sized arteries can occurQ.* Necrotizing glomerulonephritis and pulmonary capiliaritis are common.* Associated with MPO-ANCAsQ.Kawasaki (Mucocutaneous-Lymph Node) SyndromeAcute multisystem vasculitis of unknown etiologyAssociated with marked activation of T cells and monocyte or macrophages.Primarily an illness of young children ranging from 7 weeks- 12 years, with - 80% being younger than 5 years of ageQ.Male: female = 1.5: 1.Three Clinical phases of Kawasaki SyndromeAcute febrile phase:Ends with resolution of fever in 7-14 daysQIt has conjunctival injection, mouth and lip changes, erythema of hand and feet, rash and cervical lymphadenopathyQ.Subacute phase:From end of fever to 25 daysIt has desquamation, arthralgia, arthritis and thrombocytosisConvalescent phase:From disappearance of clinical sign to normalcy of ESR (usually 6- 8 weeks after onset of illness).Associated features/Complications:Cardiovascular manifestations are leading cause of morbidity and mortalityQ and include myocarditis, arterial aneurysm, pericarditis, MR, AR. ventricular arrhythmias.Arthralgia and arthritisUrethritis with sterile pyuriaAseptic meningitisHydrops of gall bladderDiarrhea, vomiting or abdominal painSensorineural hearing lossUveitisHepatic dysfunctionDiagnostic Criteria of Kawasaki SyndromeSymptomOccurrence (%)Fever lasting > 5 days (with spikes) plus at least 4 of the following:1001. Bilateral painless bulbar non exudative conjunctival injectionQ922. Oro-pharyngeal mucous membrane changes (> 1)- Cherry red (injected) or fissured LipsQ (+-drying, cracking and fissuring)- Injected (red) pharynx- Strawberry tongueQ (i.e. hypertrophic tongue papillae with hyperemia)1008472323. Extremity changes (>1)- Erythema of palms or soles- Edema (non pitting) of hands or feetQ(tenderness may limit walking and hand use)- Desquamation (perigenital and perineal followed by periungual, starting at the tips of finger)Q.724856Diagnostic Criteria of Kawasaki Syndrome SymptomOccurrence (%)4. Rash: polymorphous erythematous exanthema1005. Acute non-suppurative cervical lymphadenopathy (>1 node, > 1.5 cm in diameter)72Treatment:Treatment of choice in KS in first 10 days of fever is intravenous gamma-globulin/ immunoglobulin (IVGG or IVIG) as a large single dose of 2gm/kg given over 10-12 hours plus high dose aspirin (80-100 mg/kg/ day in four doses)Q.It reduces risk of coronary artery disease from 20% to <5%.Aspirin is reduced to antithrombotic (3-5 mg/kg/day as single dose) in 14 days when fever has resolved and can be discontinued after 6-8 weeks if Echo shows no evidence of coronary artery disease. And if abnormality is detected, low dose aspirin is continued indefinitelyQ
Intravenous immunoglobulin
Prednisolone
Dapsone
Methotrexate
Vasculitis