10/29/10. Order of Presentation. Clinical Cases and Questions. Clinical Pearls in Allergy/ Immunology. No real or perceived conflicts of interest

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1 Clinical Pearls in Allergy/ Immunology Katherine Gundling, MD University of California, San Francisco October, 2010 Images from Wikipedia No real or perceived conflicts of interest I will be discussing off label uses of medications. Order of Presentation Clinical Cases and Questions 1

2 Case #1 A 32 year old woman presents with concern about food allergy. For the past three years she has noted increasing symptoms of itching, possible swelling and irritation in the mouth and throat upon eating certain foods, including apples, nectarines and plums. She is concerned about food allergy and desires testing. PMH: Generally healthy Infant eczema that resolved by age 5 s/p appendectomy age 16 Springtime hay fever symptoms including itchy, watery eyes, nasal congestion and drainage; occasional sinusitis with URIs Meds: Oral contraceptives Calcium PRN ibuprofen for headaches and dysmenorrhea Drug allergies: None known FH: Father with HTN; mother with hypothyroidism, eczema One brother with exercise induced asthma SH: Married, no children. Works as an attorney; no significant avocational exposures; nonsmoker ROS: Occasional generalized headaches 2

3 PE: VS: normal HEENT: Slight conjunctival injection; normal tympanic membranes; moderate edema of the nasal mucosa but no exudates; throat clear and glands/lymph nodes normal. Lungs clear even with forced exhalation. Slightly dry skin Remainder of the exam normal No labs available What is the cause of her problems with food? A. Food allergy B. Pollen-Food syndrome C. Ibuprofen sensitivity D. Irritation from chemical constituents of the food Answer: B Pollen-Food Syndrome Oral Allergy Syndrome 3

4 Adverse Food Reactions Immunologic Systemic (Anaphylaxis) Oral Allergy Syndrome Immediate gastrointestinal allergy Asthma/rhinitis Urticaria Morbilliform rashes and flushing Contact urticaria Eosinophilic esophagitis Eosinophilic gastritis Eosinophilic gastroenteritis Atopic dermatitis Protein-Induced Enterocolitis Protein-Induced Enteropathy Eosinophilic proctitis Dermatitis herpetiformis Contact dermatitis Sampson H. J Allergy Clin Immunol 2004;113:805-9, Chapman J et al. Ann Allergy Asthma & Immunol 2006;96:S Mast cell degranulation www-immuno.path.cam.ac.uk Adverse Food Reactions Immunologic Systemic (Anaphylaxis) Oral Allergy Oral Allergy Syndrome Syndrome Immediate gastrointestinal allergy Asthma/rhinitis Urticaria Morbilliform rashes and flushing Contact urticaria Eosinophilic esophagitis Eosinophilic gastritis Eosinophilic gastroenteritis Atopic dermatitis Protein-Induced Enterocolitis Protein-Induced Enteropathy Eosinophilic proctitis Dermatitis herpetiformis Contact dermatitis Sampson H. J Allergy Clin Immunol 2004;113:805-9, Chapman J et al. Ann Allergy Asthma & Immunol 2006;96:S

5 Pollen-Food Syndrome or Oral Allergy Syndrome Clinical features: rapid onset oral pruritus, rarely progressive Epidemiology: prior sensitization to pollens Key foods: raw fruits and vegetables Allergens: Profilins and pathogenesis related proteins Heat labile (cooked food usually OK) Cause: cross reactive proteins pollen/food AAAAI Food Allergy Teaching Slide Key Point The diagnosis of Pollen-Food Syndrome can be made easily by asking the right question: Can you eat these fruits if they are baked into a pie? True food allergy can kill! Pollen-Food Syndrome is generally just annoying If in doubt, prescribe epinephrine and refer your patient to an Allergy/Immunology specialist 5

6 Case #2 A 42 year old bicyclist is concerned about allergy to bees. He was biking in the Marin Headlands when a bee got caught under his helmet, and he was stung on the left temple. He kept riding to the trail exit but started to develop itching of the palms and soles. As he and his companion loaded their bicycles into the van he developed frank hives, shortness of breath and dizziness. His companion gave him a benadryl pill and they went to the emergency room, where he was given epinephrine, antihistamines, and corticosteroids. Over the next 2 hours his symptoms resolved, and he was discharged home with instructions to see his primary care physician. What is wrong with this scenario? A. He was not referred to an allergy/immunology specialist B. He was not administered a topical treatment for the sting, and might relapse C. He was not given a prescription for epinephrine D. The type of bee was not clearly identified Answer C. He was not given a prescription for epinephrine Why is appropriate management so important? Because the next sting could kill him! Even though answer A (send to an AI doc) is also correct, C is the best answer. Prior to discharge please prescribe and demonstrate how to use epinephrine 6

7 Epinephrine Prescription Example: EpiPen (adult) or (child) #2 Use as directed 2 Refills Image: AAAAI Food Allergy Teachings Slides 7

8 Distinguish local from systemic sting reactions Local reactions receive local treatment; they do not require allergen immunotherapy Systemic reactions can be deadly, but they are preventable Evolution of a Local Sting Reaction Images Wikipedia Systemic allergic reactions Itching of the palms and soles, or diffuse itching Hives Angioedema Abdominal pain or menstrual cramps Asthma or upper airway edema Cardiovascular collapse Reactions closer to the face and throat predispose to severe reactions 8

9 Honey Bee Apis mellifera Yellowjacket Vespula germanica Hymenoptera Paper Wasp Polistes dominulus Bumble Bee Bombus pascuorum Baldfaced Hornet Dolichovespula maculata Hornet Vespa crabro Images: cirrusimage.com, USDA, Wikipedia Honey Bee Stinger Image: Wikipedia Paper wasp and intact stinger Images: Wikipedia 9

10 Clinical Pearls Patients with a history of systemic reactions should carry an epinephrine injection system, and know how to use it Refer to AI for definitive diagnosis and treatment (skin testing and allergen immunotherapy) Hymenoptera anaphylaxis is highly treatable, and appropriate therapy can prevent death For practical preventive tips.. Practical preventive tips Avoid wearing brightly colored clothes or strong perfumes Exert caution at outdoor events where food is present Remove nests from around the house When working outdoors, wear long pants, long sleeve shirts, shoes and gloves Image: Wikipedia Case #3 A young father who has asthma and atopic dermatitis asks about whether his children will have the same problems. A discussion ensues about the genetics of these conditions, and the patient rightly wonders whether environmental exposures are important. 10

11 Which of the following early exposures is most associated with the prevention of atopic disease (atopic dermatitis, food allergy, allergic rhinitis, asthma)? A. Barn animals B. An older brother C. A household dog D. Dust mites in the pillow Answer: A Barn animals Early exposure to barn animals is strongly associated with less atopy Exposure to pets from infancy might also be helpful, as are older siblings Exposure to dust mites is associated with increased atopic conditions Von Mutius E. Proc Am Thorac Soc 2007; Vol 4 pp = Images: Wikipedia dogs 11

12 Image by K. Gundling Image by Claudio Matsuoka (Wikipedia) Prevention of atopic conditions Clear: -Infants should be breast fed -Early exposure to animals (especially barn animals) and older siblings is preventative -Allergen immunotherapy can prevent the development of new sensitization and asthma Unclear: What the pregnant mother should eat or avoid What the lactating mother should eat or avoid Whether an infant should be exposed to small amounts of common food allergens, or completely avoid common allergens 12

13 Case #4 A 58 year old man CC: problems with swelling, occurs intermittently, unpredictably; especially one side of the face or the other PMH: generally healthy Meds: none NKDA FH: sister with low thyroid problem SH: marketing consultant ROS: neg VS: nml PE: unremarkable Image: AAAAI Urt/Ang Teaching Slides Office Differential Diagnosis of Angioedema: Medication induced Allergy/anaphylaxis spectrum Hereditary angioedema Chronic urticaria/angioedema (autoimmune, infection, idiopathic) How do I work this up in a 15 minute visit? 13

14 Which question is most helpful to determine whether a work-up for hereditary angioedema is necessary? A. Has this happened before? B. Do you also have itchy red bumps? C. How long do the lesions last? D. Do you have intermittent abdominal pain? Answer: B Do you also have itchy red bumps? Urticaria is not a feature of hereditary angioedema or of any of the bradykinin mediated swelling disorders. Image: AAAAI Urt/Ang Teaching Slides 14

15 Image: K. Gundling Mast cell Degranulation Image: Wikipedia Why are the other answers incorrect? Swelling conditions from many etiologies can occur recurrently Lesions of acquired angioedema or vasculitic origin can occur in a similar time frame Abdominal pain, although characteristic of hereditary angioedema, can be non-specific, and occur with other causes of angioedema. 15

16 In this patient Medication Not on an ACE inhibitor Allergy/Anaphylaxis spectrum No obvious allergic triggers or changes in routine Hereditary angioedema Occurring first in his 50s; would be unusual Chronic urticaria/angioedema spectrum Is urticaria present? YES This makes chronic urticaria/angioedema the most likely cause. In the absence of a clear etiology, it is usually self-limited, and responsive to antihistamines. Sample regimens: Cetirizine 10 mg, bedtime, alone, or with Loratadine 10 mg, in the morning Hydroxyzine 25 mg, 4x/daily; may increase to 50 mg, 4x/daily (can be very sedating initially) Keep in mind, the new onset of isolated, persistent angioedema in an older person can be a hallmark of lymphoproliferative disease such as CLL, or other immune dysregulation. If there is no urticaria, and no other explanation of the angioedema, consider: -Thorough history and physical exam -Age appropriate cancer screening -Serum protein electrophoresis/immunofixation and refer to Allergy/Immunology for further work-up. 16

17 Shellfish allergy is a contraindication to receiving contrast dye A. True B. False Mast cell degranulation www-immuno.path.cam.ac.uk Answer B: False Food allergy to shellfish is due to hypersensitivity to tropomyosin, a muscle protein, not due to iodine. The food allergy to shellfish is IgE mediated immediate hypersensitivity. The reaction to contrast dye is caused by direct mast cell degranulation. It is the state of being atopic that puts one at higher risk for reactions to contrast dye. The more atopic, the higher the risk. Shellfish allergy increases the risk slightly in the same way as any other food allergy. 17

18 Which of the following medications is the best option for pain control in patients with Aspirin Exacerbated Respiratory Disease? A. Ibuprofen B. Oxycontin C. Extra strength acetaminophen D. Celecoxib Answer: D Celecoxib AERD: Asthma/chronic sinusitis/nasal polyposis/nsaid sensitivity Understand that COX-2 inhibitors are safe to use in patients who have allergy to NSAIDS (in spite of what is on the label) -Ibuprofen can cause severe reactions because of its COX-1 inhibition -Oxycontin should not be used when safer, nonnarcotic agents are available -High dose acetaminophen can have COX-1 inhibition effects Arthritis Rheum 2002; 46(8): Summary Pollen food syndrome is common in patients with pollen allergies, and can be diagnosed by ascertaining whether the symptoms disappear with cooking of the food Bee stings can be deadly. Provide and demonstrate epinephrine use and refer to an Allergy/Immunology specialist. Dirt is good for you! The differential diagnosis of angioedema is simplified by determining the presence of urticaria Shellfish allergy is NOT a contraindication to contrast dye. Celecoxib is safe to use for pain control in patients with AERD 18

19 Conditions seen by Allergy/Immunology Specialists Allergic rhinitis Anaphylaxis Angioedema Asthma Aspirin exacerbated respiratory disease Atopic dermatitis Conjunctivitis Drug adverse reactions Eosinophilic mucosal diseases Exercise induced asthma Eye allergy Food allergy Hives Immunotherapy Insect stings Mastocytosis Primary immunodeficiency diseases Recurrent infections Sinusitis Urticaria Vaccine questions 19