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Richard D. deShazo, MD with thanks to the many colleagues from whom I have borrowed material.

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Presentation on theme: "Richard D. deShazo, MD with thanks to the many colleagues from whom I have borrowed material."— Presentation transcript:

1 Richard D. deShazo, MD with thanks to the many colleagues from whom I have borrowed material

2 What are the major diagnostic criteria for asthma? How can you separate asthma from COPD? What does a positive methacholine inhalation test mean?

3 3

4  FEV1 improvement of 12% and 200mL after beta-agonist is evidence for asthma according to ATS  Decreased FEV1/FVC ratio  20-39yr85%  40-59yr80%  60-80yr70%  Methacholine challenge – not diagnostic of asthma  + with 20% decrease FEV1 with <8-16mg/mL  True benefit is in negative predictive value 4

5 What test should be considered in a young asthmatic female with recurrent episodes of wheezing poorly responsive to bronchodilators?

6 Vocal Cord Dysfunction Is Not Asthma, But May Occur In Asthmatics Flow volume loops showing maximum inspiratory and expiratory flow- volume relationships in a patient with vocal cord dysfunction during asymptomatic (left) and symptomatic (right) periods. Note also the marked adduction of the vocal cords with severe reduction of the glottic aperture during a symptomatic period (right) of airway obstruction. 6

7  Female 2:1  Athletes  Bronchodilator non responsive  Many (up to 1/3) can have coexist asthma  Difficulty getting breath in 7

8 What is the likely diagnosis in a man with persistent cough, shortness of breath, dyspnea, and episodic wheeze that developed after working as a police officer in the 9/11 exposure area in NYC? A 22 year old with recent onset cough and wheeze works in a custom sailboat shop in Biloxi sealing the inside of boats with epoxy resins. What is the most likely low molecular weight chemical causing his symptoms?

9  Treatment – Time + corticosteroids  Occurs after a single inhalation of a caustic irritant in a non-asthmatic  Obstruction on PFT  (+) methalcholine inhalation challenge  Limited PFT-response to beta-agonists  Not “reactive airways disease” 9

10  Basically, asthma occurring because of exposure at work.  Testing difficult, so sometimes need to do pulmonary evaluation at the workplace. (Start with peak flows at work versus home).  Earlier you diagnose the better, because may lead to permanent asthma even when removed from environment. 10

11  Previous life-threatening asthma such as respiratory arrest  Hospitalization or ED visit for asthma within the last year  Use of 2 or more canisters of rescue inhaler/month  Poor perception of hypoxia or airway obstruction  Psychosocial disturbance 11

12 Components of Severity MildModerateSevere ImpairmentIntermittent----------------------- - ----Persistent--------------------------- - Symptoms<2 days/weeks>2 days/week but not daily >1 x/week but not nightly Throughout the day Nighttime awakenings <2 x/month3-4 x/month>1 x/week but not nightly Often 7 x/week SABA use for symptom control (not prevention of EIB) < 2 days/week>2 days/week but not more than 1 x/d DailySeveral times a day Interference with normal activity NoneMinor limitationSome limitationExtremely limited Lung functionNormal FEV1 between exacerbations FEV1 >80% of predicted FEV1/FVC normal FEV1 >80% of predicted FEV1/FVC normal FEV1 >60% but <80% of predicted FEV1/FVC reduced 5% FEV1 5% Risk Exacerbations (consider frequency and severity) 0-2/year>2/year 12

13  Rescue medicines  Short acting  agonists (SABA)  Anticholinergic  Controller medicines  Inhaled corticosteroids (ICS)  Long acting  agonists (LABA)  Leukotriene receptor antagonists (LTRA)  Combinations of ICS & LABA  Anti-IgE (omalizamab)  Others 13

14 14 Stacking Asthma Drugs

15 15 What Meds Should You Think About Adding On In Severe Asthma?

16  Vocal cord dysfunction  GERD  Sinusitis  Churg-Strauss syndrome  ABPA  Compliance/technique 16

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20  Churg-Strauss Vasculitis  Sinusitis, asthma, eosinophilia, vasculitis  Transient pulmonary infiltrates  Small vessel vasculitis  Neuropathies (mononeuritis multiplex)  MPO- ab/P-ANCA test (+) in about 50%  RX: steroids (cyclophosphamide/azathioprine in severe)  ? relationship to LTRA 20

21  Includes allergic bronchopulmonary aspergillosis (ABPA) and other allergic mycoses  Criteria  Asthma  Fleeting pulmonary infiltrates  High total IgE (>1000 ng/mL or 417 kU/L)  Positive immediate skin test (IgE) to fungus (A. fumigatus, etc.) or Positive RAST test  Elevated serum specific IgE to fungus (A. fumigatus, etc.)  CT with central bronchiectasis reflects chronic disease 21

22  Oral corticosteroids  Follow total serum IgE and CXR  No long term studies on Itraconazole as steroid sparing agent 22

23  No increase asthma but may have allergic – like dermatitis  Paramalignant syndrome  Peripheral blood eos count >1500 µL for at least 6 months  Absence of any other known cause of eosinophilia  Presumptive signs/symptoms of organ invasion by eosinophils  9:1 male:female  Systemic disease  Cardiac disease (thrombosis, fibrosis, necrosis) is the most common cause of death  FIP1L1/PDGFA fusion-gene present in some patients 23

24  Heart  Skin, Lungs, GI tract, Nervous System  Vascular thrombosis is a feared complication  Treatment  Steroids  Hydroxyurea  Interferon α  Gleevac (imatinib) - for FIP1L1-PDGFR α gene rearrangement 24

25 A 50 year old woman with asthma and atopic dermatitis has her 3 rd episode of food impaction. What findings should be sought from endoscopy to confirm the diagnosis?

26  Atopic individuals  Usually in upper tract  Food impaction in esophagus  Mucous furrowing and white specks on endoscopy  More than 12 eosinophils/HPF on biopsy  Food allergy in some  Treated with non-absorbable steroids 26

27 A 50 year old bird breeder has persistent shortness of breath, cough, and dyspnea. What should be done?

28  Fever, chills, malaise, cough, dyspnea  Acute, subacute, chronic  A systemic illness  Also known as “extrinsic allergic alveolitis”  Non allergic, not IgE mediated, no eosinophilia, no asthma except pigeon breeder’s (bird fancier’s) lung  T-cell mediated  CXR/CT scan abnormal: ground glass/diffuse alveolar pattern  PFT – restriction not obstruction except for pigeon breeders  Chronic  pulmonary fibrosis 28

29  Characterize chronicity looking for seasonal variation.  Ask for family history, indoor triggers and nasal itching.  Swollen nasal mucosa usually more blue that red.  Treat  If there is treatment failure, do allergy skin tests or RASTs to determine specific allergen and consider allergy shots.  Allergy shots are very effective for allergic rhinitis and insect anaphylaxis. 29

30  Nasal steroids – first line treatment  Antihistamines and leukotriene receptor antagonists – second line (about equal efficacy as second line agents)  To prevent symptoms on planned exposures, chromolyn or antihistamines work best  Allergen immunotherapy by subcutaneous or sublingual techniques 30

31  Vasomotor rhinitis  Rhinitis medicamentosa  Nasal manifestations of systemic disease:  Diabetes mellitus – mucor mycosis (nasal eschar; black crust)  Wegener’s granulomatosis (saddle nose)  Midline granuloma (saddle nose)  Relapsing polychondritis (saddle nose)  Sarcoidosis (bloody crusts)  Cystic fibrosis (nasal polyps)  CSF leak – check beta 2 transferrin (very specific) 31

32  Immunodeficiency  Cystic fibrosis  Aspirin – sensitive respiratory disease  Allergic fungal sinusitis  Anosmia is a big tip off for polyps 32

33 33 Acute & Chronic Sinusitis Ethmoid cell

34 A 30 year old nurse has a severe chronic dry and cracking dermatitis of both hands. What should you do first?

35 35 Irritant vs. Allergic Contact Dermatitis

36  36 AllergenCommon Route of Patient Exposure Nickel sulfateJewelry NeomycinTopical antibiotics Balsam of Peru (marker for fragrance allergy) Cologne or perfume Fragrance mixMost personal care products; products labeled “unscented” may contain masking fragrances Thimerosal (frequently positive, but rarely relevant) Eye-care products (eye drops, contact lens solutions); preservative in some vaccines Sodium gold thiosulfateJewelry; dental work Quaternium-15 (formaldehyde-releasing preservative) Personal care products FormaldehydePersonal care products BacitracinTopical antibiotic; may cross-react with neomycin Cobalt chlorideBlue paint; vitamin B12 (cyanocobalamin); may cross- react with other metals, including nickel MKSAP Most Frequently Positive Patch -Test Results in Allergic Contact Dermatitis

37 A 16 year old male with asthma has a chronic pruritic dermatitis in the flexural areas of his elbows and knees. What treatment would be best for this problem?

38 38 Atopic dermatitis with involvement of a flexural surface. The frequently involved anticubital and popliteal fossae. Atopic Dermatitis (Eczema) (1)

39  Increased susceptibility to  Staph aureus, HSV (eczema herpeticum), vaccinia  Differential diagnosis  Allergic contact dermatitis  Irritant contact dermatitis (gloves)  Cutaneous T – cell lymphoma An adult presenting with eczematous dermatitis with an erythrodermatous appearance by skin biopsy with genetic studies  Atopic dermatitis is associated with abnormalities in the filaggrin gene which encodes for filaggrin protein important in statium corneam moisturization 39

40  Skin hydration/moisturization (Aquaphor)  Medium strength topical corticosteroids and combinations (equal parts 0.1% triamcinolone and Aquaphor)  Oral antibiotics (Staph)  Antihistamines (control itch)  Topical calcineurin inhibitors (protopic/ elidel) 40

41 CauseHistoryPhysical Examination Findings Laboratory Findings Atopic dermatitisPersonal or family history of atopic dermatitis, allergic rhinitis, or asthma; preexisting skin lesions; severe pruritus LichenificationEosinophilia; elevated serum IgE level PsoriasisPersonal or family history of psoriasis; preexisting psoriatic skin lesions; history of corticosteroid withdrawal Nail pitting, oil droplet sign, onycholysis; psoriatic arthritis Skin biopsy may be diagnostic Cutaneous T-cell lymphoma/Sézary syndrome Slowly progressive; extreme, intractable pruritus Lymphadenopathy; painful, fissured keratoderma; alopecia; leonine facies Peripheral blood smear with >20% Sézary cells; clonal T-cell population in skin, blood, and/or lymph node Hypersensitivity drug eruption Onset 3-6 weeks after start of a new medication; most common causative medications are allopurinol, aromatic anticonvulsants, dapsone, NSAIDs, sulfonamides, lamotrigine Facial edema; cervical lymphadenopathy; fine scale around nose; hepatosplenomegaly Leukocytosis with eosinophilia or atypical lymphocytosis; elevated serum aminotransferases; elevated blood urea nitrogen or serum creatinine levels MKSAP Clinical Clues to the Diagnosis of Selected Causes of Erythroderma 41

42 42 Classification of Urticaria Acute urticaria (<6 weeks) Chronic urticaria (>6 weeks) Physical urticaria (pressure, cold, vibratory, etc.) Urticarial vasculitis Contact urticaria (touching a cat) Urticaria and angioedema as components of anaphylaxis

43 ClassificationCharacteristics Papular urticariaAssociated with insect bites Cholinergic urticariaOccurs in response to an increase in core body temperature; lesions present as small papules with a prominent surrounding erythematous flare Delayed pressure-induced urticariaPatients often experience systemic symptoms Exercise-induced urticariaOn a continuum with exercise- induced anaphylaxis; many patients only develop symptoms if primed by an ingested allergen prior to exercise Solar urticariaSome patients who appear to have solar urticaria prove to have lupus erythematosus or porphyria Cold urticariaAcquired cold urticaria is often related to an underlying infection, whereas familial cold urticaria represents an autoinflammatory syndrome Vibrational urticaria— 43 Classification of Physical Urticarias

44 44 Palpable purpura is a clinical sign of a small vessel (leukocytoclastic) vasculitis which may present as chronic urticaria.

45 A 68 year old female with chronic mylogenous leukemia presents with non itchy swelling of the lips and throat. What diagnostic tests would be most useful to diagnose acquired angioedema?

46  Allergic reactions and anaphylaxis  Idiopathic angioedema  Drug induced angioedema  Allergic contact dermatitis  Autoimmune conditions  Thyroid disorders  Superior vena cava syndrome and tumors  Cheilitis granulomatosa (Miescher’s cheilitis) and Melkersson-Rosenthal syndrome  Trichinosis  Low C-4 levels 46

47  Autosomal Dominant  Hiveless, itchless edema  Hereditary angioedema form more likely to have symptoms precipitated by trauma - dental visit, surgery, auto accident, menses, puberty  Visceral attacks may present as an acute abdomen with normal findings at surgery 47

48  Diagnosis of HAE 5  HAE 1 - low C4 1, 2 low C1 INH, low C1INH-F  HAE2 - low C4 1, 2 normal C1 INH, low C1INH-F  Acquired – low C4 1, 3 low C1 INH, low C1INH-F, low Clq  HAE3 – normal C4 4 normal C1 INH, normal C1INH-F, normal C1f level 1. Always low during attacks 2. SERPING1 gene abnormalities 3. Antibodies to C1INH 4. Abnormalities in Factor XII genes 5. Family members should be screened in all types 48

49  Associated with lymphoproliferative disorders  Have low C1q levels  Mechanism unclear  Low C1 of levels appear to reflect autoactivation  Anti – C1 esterase inhibitor antibodies have been described as well 49

50  C4 – is a great screening test, but is normal in HAE Type 3  Test for C1 esterase inhibitor level and function to discriminate between two hereditary types  Test C1q level for the acquired form  Genetic testing for HAE Type 3 50

51  On-demand treatment  Treatment of attacks with upper airways symptoms is mandatory  Acute attacks should be treated with  C1INH (plasma derived) Berinert  Escallantide (inhibits HMW Kininogen to bradykinen) Kalbitor  Icatibant (bradykinen recepptor antagonist) Firazyr  Intubation or trachestomy should be early in progressive airway obstruction  Antifibronolytics are not to be used  Procedural prophylaxis with procedures involving the upper airway is recommended without evidence  Pregnancy – C1INH advised  Long-term Prophylaxis  C1INH - immunizations, screens  Androgens – frequent screens for liver toxicity  Antifibrolytics not recommended 51

52  Purified (C1INHRP) or recombinant (rhC1INH) human C1 inhibitor (various products available worldwide)  Ecallantide, a kallikrein inhibitor (available only in the United States)  Icatibant, a bradykinin B2 receptor antagonist (available in the United States and the European Union) 52

53 What should be prescribed for a patient after treatment for acute anaphylaxis and how should it be used?

54  Usually starts with urticaria and itching but can present with syncope, hypotension, or erythoderma.  Most common causes are foods (peanut/ tree nut ingestion, etc.), insect stings, drug allergy (beta lactam)  Don’t forget latex, especially in medical spaces  No obvious trigger- think mastocytosis (check serum mast cell tryptase) or idiopathic anaphylaxis  All who have insect anaphylaxis patients require evaluation for venom immunotherapy 54

55  Hypotension - supine, IV (NS)  Respiratory distress - oxygen and albuterol, intubation  Epinephrine  1:1000.3-.5cc IM for adults (note this is NOT the 1:10,000 dilution - (1mg/10ml) on crash carts  Can repeat in 5 minutes  If on beta blocker and not responsive to epi, consider glucagon 1mg IM, IV, SC  Can start epi infusion if not responsive to IM epi  (1:10,000) 1-3 mg over 3 min, then 3-5mg over 3 minutes, then 4- 10ug/min infusion  All who recover must leave with an Epipen®.  Consider 1 mg of glucagon IV if on beta blocker 55

56  Cutaneous Mastocytosis  Have urticaria pigmentosa – reddish brown or tan macules. Darier’s sign  Indolent, benign course  Itching, burning skin, flushing, CNS symptoms 56

57  Major  Multifocal dense infiltrates of mast cells in bone marrow or extracutaneous organ  Minor  >25% mast cells are spindle-shaped or atypical  Presence of c-kit point mutation  Mast cells co-express Kit and CD2  Persistent serum tryptase >20ng/mL  Need one major and one minor, or 3 minor  Other:  Skin involvement improves prognosis  Insect stings may induce anaphylactoid reactions in patients with mastocytosis. 57

58 58 MKSAP Common Drug Reaction Patterns

59  “Red man syndrome” – pruritis and erythema of face, neck, upper torso, occasionally with hypotension  Non-immunologic release of histamine  Not IgE mediated  Rx: slow the infusion and pre-treat 59

60  Cause the “ hypersensitivity syndrome ”  Deficiency of epoxide hydrolase  Fever, maculopapular rash, generalized lymphadenopathy  Node bx resembles Hodgkin’s  Phenytoin, carbamazepine, phenobarb  Can also cause DRESS : Drug reaction with eosinophilia and systemic symptoms. Rash, fever, multi-organ failure  ½ actually have eosinophilia 60

61 61

62 A 21 year old college student has recurrent sinupulmonary infections. What test in addition to HIV should be performed to exclude immunodeficiency?

63  T- cell (cellular immunity) – virus, fungi, protozoa, mycobacteria and other intracellular organisms  Humoral (antibody mediated immunity) – infection with extracellular pyogenic organisms  Haemophilus  Pneumococcus  Streptococcus  Increased infections  Recurrent respiratory infections  Multiple systems involved  Unusual organisms  Malabsorption  Big LNs or absent LNs 63

64  Decreased IgG, M, A, normal to increased IgE  Recurrent sinopulmonary infections  Lymph tissue present or enlarged  High incidence of autoimmune disease (22%)  Increased risk of adenocarcinoma and lymphomas  In addition to encapsulated organisms  Giardia, yersinia, H. pylori, and H. jejuni are common 64

65  Most common form of primary immunodeficiency (1:333)  IgA <5mg/dL (VERY LOW)  Most patients with IgA def. are NORMAL  Have increased risk of infections: collagen vascular, allergic, and GI disease, and malignancy  Can make anti-IgA antibodies  Leads to anaphylaxis with IgA containing blood products 65

66  Indicated for common variable immunodeficiency and specific antibody deficiency  Not indicated for IgA deficiency  Side effects  Never transmitted of HIV  Fever, chills, HA, muscle pain.  Aseptic meningitis  Renal failure (was due to osmotic load, not as common now), stroke, MI 66

67  C2 deficiency (most common) – sepsis, pneumonia, meningitis, pyogenic arthritis with Strep pneumo  C2 deficiency – increased risk of rheumatoid arthritis, SLE  Terminal Complement Components – Neisseria sp infections  Think about if recurrent meningitis or if unusual strain 67

68  Measles  Mumps  Oral Polio  Rubella  Varicella  MMR  Smallpox (vaccinia)  Flumist 68

69  Influenza and Yellow fever 69

70 What dietary pattern do bedbugs follow?

71 71 Bedbug Bites Often Occur in a Series “Breakfast, lunch, and dinner” Lesions are usually painless and appear as pruritic, urticarial-like papules. Dinner Lunch Breakfast

72 72 Bedbugs are nocturnal, hiding in cracks and crevices within 10 feet of a bed and especially on the cords of the mattress during the day and biting at night. Hungry Fed

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