Recurrent idiopathic anaphylaxis

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Recurrent idiopathic anaphylaxis Presented by Wat Mitthamsiri, M.D. May2, 2014

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<ul><li>1.Recurrent (Idiopathic?) Anaphylaxis By Wat Mitthamsiri, MD. Allergy and Clinical Immunology Fellow King Chulalongkorn Memorial Hospital </li></ul> <p>2. Outline Case presentation Review of anaphylaxis Definition Diagnostic criteria Review of idiopathic anaphylaxis Definition Estimated incidence Classification Theories of pathogenesis Differential diagnosis Investigations Approach Therapy and management Prognosis and future therapy 3. CASE 4. A 40 years old Thai woman Oct 2012 Facial edema, no wheezing -&gt; Dx: R/O anaphylaxis from Tiffy Nov 2012 Wrist pain with multiple PIP pain and erythematous rash on extremities -&gt; W/U RF&amp;ANA: Negative -&gt; On NSAIDs -&gt; Improved -&gt; Stop NSAID 21 Feb 2013 Facial edema and wheezing -&gt; Dx: Anaphylaxis, Admit 5. A 40 years old Thai woman 26 Feb 2013 Edema occurred again No medical Rx 20 Mar 2013 Oral ulcer -&gt; Got colchicine from clinic After 9 tablets taken (1 hr after last tablet) -&gt; facial edema, lungs clear 6. A 40 years old Thai woman 20 Mar 2013 Hx of penicillin, Bactrim, Brufen, ASA, colchicines, diclofenac allergy (no detail about symptom) W/U: Serum tryptase 1.9 C3=1.23, C4=0.4 (0.1-0.4), CH50 = 75% R/O Complement deficiency HM: Atarax, CPM, cetirizine Refer to KCMH 7. A 40 years old Thai woman 9 Apr 2013 At GenMed Clinic -&gt; Initial W/U CBC: Hct 39.4, Hb 13.4, WBC 9010 (N 47, L 44, E 2.3), plt 334000 AST 17, ALT 24, ALP 68 UA WNL -&gt; Sent to Allergy Clinic 8. A 40 years old Thai woman 23 Apr 2013: 1st KCMH Allergy Clinic visit Hx of facial/orbital angioedema Probable anaphylactic reaction R/O from Tiffy, ASA, Brufen Symptoms usually occurred 15-30 min post tablet and persisted for 2 days There were 2 episodes that occurred without any medication PH: Mild AR, no AA, no CRS PE: No nasal polyp 9. A 40 years old Thai woman 23 Apr 2013: (Continued) Imp: Recurrent severe angioedema with probable anaphylaxis NSAIDs/analgesic sensitivity (angioedema) W/U: SIgE to mixed food -&gt; Negative Rx: Adrenaline kit, cetirizine 1x2, montelukast 1x2, prednisolone(5) 3x2 After went home and do some cleaning -&gt; symptoms occurred again 10. A 40 years old Thai woman 29 Apr 2013 Symptom occurred 40 min after meal with 7 May 2013 F/U -&gt; Taper Prednisolone(5) to 2x2, continue montelukast, cetirizine 11. A 40 years old Thai woman 17 May 2013 After exhaustive workout (without any medication, or food within 5 hr), she had erythroderma at extremities, facial edema, no itching She went to a hospital &gt; Adrenaline im &gt; 10 min after that, symptoms improved &gt; Completely resolved after 1 day 12. A 40 years old Thai woman 17 May 2013 (continued) PE: Steroid acne found W/U: Baseline serum tryptase Rx: Stop antihistamine (prevention of obscuring late detection of anaphylaxis) Increased prednisolone(5) to 4x3 for 10 days, then 3x3 Continue cetirizine, montelukast Add ranitidine(150) 1x1 13. A 40 years old Thai woman 9 Jul 2013 During June, she had 2 severe generalized urticaria episodes with mild angioedema 1 of these had chest tightness without wheezing. She self-injected adrenaline both times -&gt; symptoms improved within 10 min but completely resolved after 1 day 14. A 40 years old Thai woman 9 Jul 2013 (continued) She said that eating jackfruit caused neck tightness without other symptom BUT she can wear rubber gloves and boots W/U: ANA, CH50, C3, C4 Rx: RM 17 May 2013 15. A 40 years old Thai woman 11 Aug 2013 During housekeeping -&gt; palpitation, facial edema, rash -&gt; Adrenaline self-injection 25 Sept 2013 During housekeeping -&gt; palpitation, facial edema, rash -&gt; Adrenaline self-injection 16. A 40 years old Thai woman 1 Oct 2013 Result W/U came back: Serum tryptase 2.21 ( Chest tightness with erythroderma without wheezing &gt; Adrenaline self-injection and went to a hospital &gt; Received 3 more unknown iv injection and observed until 23.00 &gt; HM: Prednisolone(5) 3x3 until 25 Feb 2014, then 6x1 &gt; Continued other medication 19. A 40 years old Thai woman 3 Mar 3014 F/U: Lab results back: Total IgE 453 (normal /=2 of the following that occur rapidly after exposure to a likely allergen for that patient (minutes to several hours): Involvement of skin-mucosal tissue e.g., generalized hives, itch-flush, swollen lips-tongue- uvula Respiratory compromise e.g., dyspnea, wheeze-bronchospasm, stridor, reduced PEF, hypoxemia Reduced BP or associated symptoms of end-organ dysfunction e.g., hypotonia [collapse], syncope, incontinence Persistent gastrointestinal symptoms e.g., crampy abdominal pain, vomiting Sampson HA et al.. J Allergy Clin Immunol 2006;117: 391-7. 26. Diagnostic criteria: 1 of these 3) Reduced BP after exposure to known allergen for that patient (minutes to several hours): Infants and children: Low systolic BP (age specific) or greater than 30% decrease in systolic BP* Adults: Systolic BP less than 90 mm Hg or greater than 30% decrease from their baseline Sampson HA et al.. J Allergy Clin Immunol 2006;117: 391-7. 27. REVIEWS Idiopathic anaphylaxis 28. Idiopathic anaphylaxis Definition Idiopathic anaphylaxis is anaphylaxis not explained by a proved or presumptive cause or stimulus A diagnosis of exclusion after other causes have been considered PA. Greenberger, Immunol Allergy Clin N Am 27 (2007) 273293 29. Estimated incidence Antibiotics: 22% of all drug-related episodes, 1.9-27.2 million US Latex: 2.7-16 million US Perioperative anaphylaxis: 9%-19% of complications Radiocontrast media: 0.16% of ionic media, 0.03% of nonionic media administration Hymenoptera stings: 0.4%-0.8% of children, 3% of adults Food: 0.0004% of the US per year NSAIDs: Varied between reports Antisera: 2-10% of cases that used the agents Hemodialysis-associated: 21 cases in 260,000 dialysis Idiopathic: 2/3 of adults presenting to allergist/immunologist Extrapolated data: 20,592 to 47,024 cases in U.S. population S G A Brown, et al., Middletons Allergy 8th edition, 2013, 1237-1259. 30. Estimated incidence S G A Brown, et al., Middletons Allergy 8th edition, 2013, 1237-1259. 31. Estimated incidence S G A Brown, et al., Middletons Allergy 8th edition, 2013, 1237-1259. 32. Classification By frequency and presentation PA. Greenberger, Immunol Allergy Clin N Am 27 (2007) 273293 K Lenchner, et al., Curr Opin Allergy Clin Immunol 3 (2003) 305311 33. Classification By treatment difficulty PA. Greenberger, Immunol Allergy Clin N Am 27 (2007) 273293 K Lenchner, et al., Curr Opin Allergy Clin Immunol 3 (2003) 305311 34. Classification By variations PA. Greenberger, Immunol Allergy Clin N Am 27 (2007) 273293 K Lenchner, et al., Curr Opin Allergy Clin Immunol 3 (2003) 305311 35. Theories of pathogenesis PA. Greenberger, et al., Article In-press, J Allergy Clin Immunol Pract 2014 Hidden allergen Food additives? Food itself? Latex? 36. Theories of pathogenesis PA. Greenberger, et al., Article In-press, J Allergy Clin Immunol Pract 2014 Aberrant cytokine profile lowering the threshold for mast cell degranulation Increase in Th2 cytokines (IL-4, IL-5, and IL-13) 37. Theories of pathogenesis PA. Greenberger, et al., Article In-press, J Allergy Clin Immunol Pract 2014 Female hormone effect on mast cells and/or basophils Episodes are more common in females patients why? 38. Theories of pathogenesis PA. Greenberger, et al., Article In-press, J Allergy Clin Immunol Pract 2014 An alteration in the T-cell population Current acute idiopathic anaphylaxis patients had a higher percentage of CD3+HLA-DR+ cells than those in remission Patients with breakthrough episodes during prednisone Rx and who were in remission had significantly higher percentage of activated B cells (CD19+CD23+) than normal volunteers 39. Theories of pathogenesis PA. Greenberger, et al., Article In-press, J Allergy Clin Immunol Pract 2014 Increased sensitivity to histamine at the target organ site Patients with idiopathic anaphylaxis had Increased sensitivity to the injection of histamine Equal sensitivity to histamine as CIU patients Less reactivity to histamine than AR/asthma patients Impaired inactivation of PAF 40. Theories of pathogenesis PA. Greenberger, et al., Article In-press, J Allergy Clin Immunol Pract 2014 Presence of serum histamine releasing factor Presence of IgE autoantibodies No evidence whether these antibodies are active in producing mast cell degranulation 41. Theories of pathogenesis PA. Greenberger, et al., Article In-press, J Allergy Clin Immunol Pract 2014 Spontaneously increased mast cells? A study of mast cell no. from skin Bx Normal, 38 cells/mm2 Idiopathic anaphylaxis or unexplained flushing, 72 cells/mm2 Urticaria pigmentosa or indolent systemic mastocytosis, nonlesional skin, 168 cells/mm2 Urticaria pigmentosa, lesional skin, 597 cells/mm2 indolent systemic mastocytosis, lesional skin, 721 cells/mm2 42. Differential diagnosis PA. Greenberger, Immunol Allergy Clin N Am 27 (2007) 273293 43. Differential diagnosis PA. Greenberger, Immunol Allergy Clin N Am 27 (2007) 273293 44. Differential diagnosis PA. Greenberger, Immunol Allergy Clin N Am 27 (2007) 273293 45. Differential diagnosis PA. Greenberger, Immunol Allergy Clin N Am 27 (2007) 273293 46. Differential diagnosis PA. Greenberger, et al., Article In-press, J Allergy Clin Immunol Pract 2014 47. Differential diagnosis PA. Greenberger, et al., Article In-press, J Allergy Clin Immunol Pract 2014 48. Differential diagnosis PA. Greenberger, Immunol Allergy Clin N Am 27 (2007) 273293 49. Investigations PA. Greenberger, et al., Article In-press, J Allergy Clin Immunol Pract 2014 Skin tests to foods or to drugs Using standard commercially available extracts Using fresh food Serum-specific IgE to foods and drugs Diagnostic-therapeutic trial with prednisone Oral challenge 50. Investigations PA. Greenberger, et al., Article In-press, J Allergy Clin Immunol Pract 2014 Serum anti-alpha-gal IgE Baseline and during anaphylaxis serum tryptase Baseline and during anaphylaxis 24- hr urinary histamine metabolites Prostaglandin D2 (urine or plasma or urinary metabolite 9a, 11b- prostaglandin F2) 51. Investigations PA. Greenberger, Immunol Allergy Clin N Am 27 (2007) 273293 Peripheral blood c-KIT mutation for codon D816V Bone marrow examination Skin biopsy Bone scan Complement (C4) determination 52. Approach K Lenchner, et al., Curr Opin Allergy Clin Immunol 3 (2003) 305311 53. Approach K Lenchner, et al., Curr Opin Allergy Clin Immunol 3 (2003) 305311 54. Approach K Lenchner, et al., Curr Opin Allergy Clin Immunol 3 (2003) 305311 55. Approach K Lenchner, et al., Curr Opin Allergy Clin Immunol 3 (2003) 305311 56. Therapy of anaphylaxis S G A Brown, et al., Middletons Allergy 8th edition, 2013, 1237-1259. IMMEDIATE ACTION Perform assessment. Check airway and secure if needed. Rapidly assess level of consciousness. Vital signs TREATMENT Epinephrine Supine position, legs elevated Oxygen Tourniquet proximal to injection site 57. Therapy of anaphylaxis S G A Brown, et al., Middletons Allergy 8th edition, 2013, 1237-1259. DEPENDENT ON EVALUATION Start peripheral intravenous fluids H1 and H2 antihistamines Vasopressors Corticosteroids Aminophylline Glucagon Atropine Electrocardiographic monitoring Transfer to hospital 58. Therapy of anaphylaxis S G A Brown, et al., Middletons Allergy 8th edition, 2013, 1237-1259. 59. Therapy of anaphylaxis S G A Brown, et al., Middletons Allergy 8th edition, 2013, 1237-1259. 60. Therapy of anaphylaxis S G A Brown, et al., Middletons Allergy 8th edition, 2013, 1237-1259. 61. Long-term managements PA. Greenberger, Immunol Allergy Clin N Am 27 (2007) 273293 For IA-I patient (</p>