Churg Strauss Syndrome

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    03-Jun-2015

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Churg Strauss Syndrome

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<ul><li> 1. Case presentation</li></ul> <p> 2. 59 CC: 5 PI:1PTA URI symptom 5PTA . 3PTA . . 3. Past Hx :2PT . 10PTA - 4. Physical examination BT 38 cPR 100/min BP 120/80 mmHg RR 24/min Conscious, not pale, no malar rash, no oral ulcerLN -ve skin :hyperpigmented scar with hemorrhagic crust both forearms and legs lung :clear, equal BS heart:regular, normal s1s2,no murmur abd:soft, not tender, no mass,no hepatosplenomegaly neuro: pupil 3 mm RTL BE, decrease motor tone all extremities 5. Motor: Rt. Lt.DTRRt.Lt. deltoidIV IV bicepsIII+IV01+ tricepsIII+II 01+ wrist EX/FLEXIV/II0/000hip E/FIV/IIIV/IV knee E/FV/IIIV/III02+ ankle E/FV/IIV/I01+ Sensory: impaired both hand and feet above ankle BBK: plantar flexion Propioception: impaired 6. Problem list </p> <ul><li>Acute Symmetrical weakness </li></ul> <ul><li>Hx of asthma</li></ul> <ul><li>Hx recurrent pruritus </li></ul> <p> 7. Laboratory CBC:wbc 21,950Hct 35plt 277,000 N 42.5, L 12.3, Mo 3.4, Eo41.7 normochromic normocytic Rbc UA:sp.gr. 1.015, prot/sugar ve, Wbc 0-1,Rbc 0-1 FBS147,BUN 5,Cr 0.39 E lyteNa126,K 3.4,Cl 82,CO2 25,Ca 2 +9.1 LFTAlb 4.0,Glob4.0, AST 55,ALT 59,Alk 185 CPK356 ESR77 8. LaboratoryANA HT,chorea,seizures, testicular pain </p> <ul><li>Few pathology report </li></ul> <ul><li>Steroid is standard Rx </li></ul> <p> 62. LTRAvsCSS 63. </p> <ul><li>Since 1999-2009 </li></ul> <ul><li>Conflict data </li></ul> <ul><li>Difficult to research design </li></ul> <ul><li>What is hypothesis for LTRA induce CSS ? </li></ul> <p> 64. 65. 66. 67. 68. 69. 70. 71. LTRA ICS SABA LABA 72. 73. 74. 75. Quiz </p> <ul><li>Which of the following is not criteria for Dx CSS in ACR criteria? </li></ul> <ul><li>1. asthma </li></ul> <ul><li>2. eosinophillia &gt; 10% </li></ul> <ul><li>3. systemic vasculitis </li></ul> <ul><li>4. paranasal sinus abnormality </li></ul> <ul><li>5. neuropathy </li></ul> <p> 76. </p> <ul><li>Which HLA may be associated with CSS ? </li></ul> <ul><li>1. HLA-B27 </li></ul> <ul><li>2. HLA-DRB4 </li></ul> <ul><li>3. HLA-C </li></ul> <ul><li>4. HLA-DR2 </li></ul> <ul><li>5. HLA-DR3 </li></ul> <p> 77. </p> <ul><li>All the following are poor prognostic factor for CSS except_______ </li></ul> <ul><li>1. nephrotic range proteinuria </li></ul> <ul><li>2. renal failure </li></ul> <ul><li>3. GI involvement </li></ul> <ul><li>4. mononeuritis multiplex </li></ul> <ul><li>5. cardiomyopathy </li></ul> <p> 78. </p> <ul><li>Subgroup of CSS is differentiate by ANCA status, which one is not clinical of ANCA +ve patient ? </li></ul> <ul><li>1. cardiomyopathy </li></ul> <ul><li>2. small vss. Vasculitis </li></ul> <ul><li>3. glomerulonephritis </li></ul> <ul><li>4. pulmonary hemorrhage </li></ul> <ul><li>5. purpura</li></ul> <p> 79. </p> <ul><li>Which one of the following is true in childhood CSS ? </li></ul> <ul><li>1. incidence of CSS is similar between</li></ul> <ul><li>children and adult </li></ul> <ul><li>2. ANCA +ve 50% of case </li></ul> <ul><li>3. usually require 2 ndline Rx </li></ul> <ul><li>4. HT is common problem in childhood CSS </li></ul> <ul><li>5. pathogenesis of CSS in chlidren differ</li></ul> <ul><li>from adult </li></ul> <p> 80. </p> <ul><li>female 52 yrs Hx asthma 20 yrs on ICS , monteleukast,present with progressive dyspnea on intermittent short course oral steroid</li></ul> <ul><li>PE wheezing and crackle both lung no clubbing </li></ul> <ul><li>CBC wbc 10200 Eo 23 % skin test alternaria , aspergillus +ve </li></ul> <ul><li>ESR 70IgE 500CXR bilateral parenchymal infiltrationCTchest peripheral airspace subpleural consolidationWhat is diagnosis ? </li></ul> <ul><li>1. CSS </li></ul> <ul><li>2. chronic eosinophillic pneumonia </li></ul> <ul><li>3. usual interstitial pneumonia </li></ul> <ul><li>4. ABPA </li></ul> <ul><li>5. Idiopathic eosinophillic syndrome</li></ul> <p> 81. Take home messege </p> <ul><li>Clinical manifestration of CSS </li></ul> <ul><li>Pathogenesis is unknown </li></ul> <ul><li>CSS in children </li></ul> <ul><li>LTRA and CSS association </li></ul> <ul><li>Further research direction </li></ul> <p> 82. THANK YOUFORYOUR ATTENTION 83. 84. Differential diagnosis 1.29 85. 1.13</p>

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