Eosinophilic and Hypersensitivity Pneumonias

Eosinophilic and Hypersensitivity Pneumonias

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Hypersensitivity Pneumonias. Wyatt E. Rousseau, MDMay 10, 2007. Eosinophilic Lung Disease. Peripheral blood eosinophilia with radiographic abnormalitiesLung tissue eosinophilia on TBBx or OLBxIncreased eosinophils in BAL. Pulmonary Eosinophilia - Causes. Drug and Toxin InducedHelminthic and Fungal InfectionAcute Eosinophilic PneumoniaChronic Eosinophilic PneumoniaChurg Strauss SyndromeOthers.
Eosinophilic and Hypersensitivity Pneumonias

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1. Eosinophilic and Hypersensitivity Pneumonias Wyatt E. Rousseau, MD

2. Hypersensitivity Pneumonias Wyatt E. Rousseau, MD May 10, 2007

3. Eosinophilic Lung Disease Peripheral blood eosinophilia with radiographic abnormalities Lung tissue eosinophilia on TBBx or OLBx Increased eosinophils in BAL

4. Pulmonary Eosinophilia - Causes Drug and Toxin Induced Helminthic and Fungal Infection Acute Eosinophilic Pneumonia Chronic Eosinophilic Pneumonia Churg ? Strauss Syndrome Others

5. Drug and Toxin Induced Eosinophilic Lung Disease Nitrofurantoin, Ampicillin, NSAIDs, Pentamidine Phenytoin, L-Tryptophan, Ranitidine, Trazadone Metals, Scorpion stings, Heroin, Cocaine, Dust, Smoke, Scotchguard, Sulfite exposure, Organic chemicals

6. Helminthic/Fungal Infection related Transpulmonary larvae migration-Loffler?s Ascaris lumbricoides Hookworm Strongyloides stercoralis Pulmonary Parenchymal Invasion Helminths, e.g. Paragonimiasis Heavy hematogenous seeding-Trichinosis, Strongyloidiasis, Schistosomiasis, Cutaneous and visceral larva migrans

7. Helminthic/Fungal Infection related Tropical Pulmonary Eosinophilia Wuchereria bancrofti Brugia malayi Allergic Broncho-Pulmonary Aspergillosis

8. ABPA Allergic Bronchopulmonary Aspergillosis

9. ABPA Complex hypersensitivity reaction in patients with asthma that occurs when bronchi become colonized by Aspergillus Repeated episodes of bronchial obstruction, inflammation, and mucoid impaction can lead to Bronchiectasis, Fibrosis, and respiratory compromise

10. ABPA Pathology Mucoid impaction of bronchi, eosinophilic pneumonia, bronchocentric granulomatosis Asthma Septated hyphae with dichotomous branching may be seen in mucous, but do not invade the mucosa Culture + in 2/3 of patients

11. ABPA Clinical Asthma Bronchial obstruction Fever, malaise Expectoration of brownish mucous plugs Eosinophilia Hemoptysis Wheezing +/-

12. ABPA Radiologic features Upper lobe infiltrates Atelectasis ?Tram lines? ?Parallel lines? Ring shadows ?Toothpaste shadows? ?Gloved finger shadows? Perihilar infiltrates may simulate adenopathy Cylindrical bronchiectasis

15. ABPA PFTs Airflow obstruction ? reduced FEV1 Air trapping ? increased RV Positive BD response in ? Mixed obst. and rest. if bronchiectasis and fibrosis present Reduced DLCO if bronchiectasis present

16. ABPA Diagnosis Hx Asthma Skin test reactivity to Aspergillus Ppt. serum antibodies to A. fumigatus Serum IgE > 1000 ng/ml Peripheral blood eosinophilia >500/mm3 Pulmonary infiltrates Central bronchiectasis Elevated IgE and IgG to A. fumigatus

17. ABPA Treatment Corticosteroids Inhaled steroids may help control symptoms of asthma but do not have documented efficacy in preventing acute episodes of ABPA Itraconazole

18. Acute Eosinophilic Pneumonia Acute, febrile, hypoxic, RF often, mechanical ventilation Bx - DAD, hyaline membranes Blood eosinophilia absent HIV often

19. Chronic Eosinophilic Pneumonia Subacute, cough, fever, dyspnea, wheeze, sweats Asthma precedes/accompanies in 50% CXR ??photographic negative? of CHF in less than 1/3. Occasional pleural effusion, cavitations Bx - Giant cells, BOOP often

22. Churg ? Strauss Syndrome Allergic granulomatosis and angiitis Vasculitis Sinusitis, asthma, blood eosinophilia Lung, skin, cardiovascular, GI, nervous systems Patchy opacities Bx-eosinophilic infiltrates, eosinophilic vasculitis, necrotizing granulomas, and necrosis

23. Other Causes of Pulmonary Eosinophilia Idiopathic Hypereosinophilic Syndrome Idiopathic Lung Diseases Neoplasms Nonhelminthic Infections ? Cocci and rarely Tuberculosis

24. Hypersensitivity Pneumonitis Extrinsic Allergic Alveolitis A syndrome characterized by diffuse inflammation of lung parenchyma and airways in response to inhalation of antigens to which the patient has been previously sensitized

25. HP ? Incidence and Mortality General population based cohort study from a UK primary care database: 271 cases from 1991-2003, approx. 0.9 per 100,000 person years. Mean age at diagnosis 57; male=female Increased risk of dying (hazard ratio 2.98) Less likely to smoke (OR 0.56) Solaymani-Dodaran M, West J, Smith C, Hubbard R. Extrinsic allergic alveolitis: incidence and mortality in the general population. QJM 2007; 100: 233-237.

26. Case Report BH, 50 y.o. WF NS, described three episodes in three weeks of SOB, chest tightness, and hemoptysis. Initial episode lasted 2 hours, asso. with fever and chills, T. to 101F. , and tsp. of bloody phlegm. PMH: hysterx 1984, on Premarin, Lexapro, Neurontin, trazadone, calcium, Fosamax. Parents both smoked, F d. lung CA, M d. emphysema and bronchiectasis, D with asthma. Pt. had had severe complications of arthroscopy-6 surgeries, 3 ? years of immobilization until TKR in 12/02, with

27. lymphedema, but had become quite physically fit with resting pulse usually 47. On PE, macular, papular rash over abdomen, chest, and neck. No nodes, clear chest, and left leg was not more swollen than right. She had had chest CT 2/10/04 considered normal, and Lung scan 2/12/04 suggesting airways disease with inhomogeneous perfusion and prolonged washout. I recommended some lab work including D-dimer, CRP, and a venous doppler. She declined; so I asked her to take a sputum cup to bring in if she coughed and I planned a

28. CTA if another episode. She called the next day after a worse episode of tightness, SOB, and hemoptysis. CTA 2/17/04 ?tree in bud bilateral and diffuse? suggesting infection or inflammation. Bronchoscopy 2/18/07 appeared normal, with biopsies revealing ?mild chronic inflammation.? March 4 received report of outpatient sputum growing an AFB, and I assumed MAC, and discussed Abx therapy. She then decided on VATS OLBx. (Bronch and OLBx cultures were both negative ultimately.)

29. Lung biopsy revealed chronic bronchiolitis/ hypersensitivity pneumonitis (extrinsic allergic alveolitis) with opinion from Mayo Clinic path arriving on 3/30/04. ?Hot tub lung due to MAC?. I recommended Prednisone 20 mg. daily, but she had major psychiatric complications for which Seroquel was added and prednisone reduced. X-ray, spirometry, and symptoms resolved. Prednisone tapered and stopped in late May. Recurrence of symptoms and rash in August/September.

30. HP -CT Findings Ground glass opacities Poorly defined centrilobular nodules Mosaic attenuation on inspiration Air trapping on expiration Reticulation (fibrosis) when chronic Silva CI, Churg A, Muller NL. Hypersensitivity pneumonitis: spectrum of high-resolution CT and pathologic findings. Am J Roentgenol 2007; 188: 334-44.

33. Patchy ground glass with air trappingPatchy ground glass with air trapping

41. Classification of HP Acute HP Subacute or Intermittent HP Chronic progressive HP

42. Acute HP Clinical-Abrupt Lab-ESR,IGG,RA,CRP,LDH, BAL lymphocytosis, restrictive physiology CXR-micronodular interstitial, but freq. normal-HRCT needed, but not absolute Histopathology-poorly formed, noncaseating interstitial granulomas, monos, giant cells

43. Subacute or Intermittent HP Clinical-gradual cough, dyspnea, fatigue, anorexia, wt. loss, malaise; tachypnea and rales Lab-BAL lymphocytosis, hypoxemia. Restrictive or mixed spiro, decreased DLCO CXR-nml, micronodular, reticular. HRCT micronodules, ground glass, emphysema, fibrosis Histopathology- noncaseating granulomas, bronchiolitis with or w/o organizing pneumonia

44. Chronic Progressive HP Clinical-insidious cough, dyspnea, fatigue, weight loss, clubbing Lab-not very helpful-BAL lymphocytosis but not crisp; restrictive, but usually mixed. DL always reduced, and exertional hypoxemia CXR-may be normal, but usually progressive fibrosis; emphysema often Histopathology-granulomatous pneumonia, BO with or w/o OP, fibrosis

45. HP Diagnosis Known exposure to antigen-History, environmental investigation, IgG antibodies Compatible clinical, radiographic,and physiology BAL lymphocytosis Positive inhalation challenge Histopathology

46. Clinical Prediction Exposure to known antigen Positive precipitating antibodies to that antigen Recurrent symptoms Inspiratory crackles Sx occurring 4-8 hours after exposure Weight loss Lacasse Y, for the HP Study Group. Clinical Diagnosis of Hypersensitivity Pneumonitis. Am J Respir Crit Care Med 2003; 168: 952-958.

47. Helpful Clues to Recognize HP Hx recurring pneumonias, esp. if some regularity Sx after moving to new house or job Pets, esp. birds, with patient or family Hx water damage to home or office Use of hot tub, sauna, or pool Others with similar sx or have left work for illness Pt feels better when away from home or office

48. HP Associated with Farming Moldy hay, grain, silage-Farmer?s lung Mold on pressed sugar cane-Bagassosis Tobacco plants Mushroom workers Potato riddlers Cheese washers

49. HP Associated with Ventilation and Water-Related Contamination Humidifier fever Unventilated shower Hot tub lung Sauna taker?s lung Lifeguard lung

50. HP Associated with Birds/Poultry Bird fancier?s lung Poultry worker?s lung Turkey handling disease Canary fancier?s lung Duck fever

51. HP Associated with Veterinary Work and Animal Handling Laboratory worker?s lung Pituitary snuff taker?s disease Furrier?s lung Bat lung Coptic lung (mummy handlers) Pearl oyster shell pneumonitis

52. HP Associated with Grain and Flour Processing Grain measurer?s lung Miller?s lung Malt worker?s disease

53. HP Associated with Milling and Construction Wood dust pneumonitis Sequoisis Maple bark disease Wood trimmer?s disease Suberosis (moldy cork) Composter?s lung Thatched-roof lung

54. HP Associated with Plastics, Painting, Electronics, Etc. Chemical HP ? Toluene diisocyanate Detergent worker?s lung Pauli?s reagent alveolitis Vineyard sprayer?s lung Bible printer?s lung Epoxy resin lung

55. HP in Textile Workers Byssinosis (brown lung) ? cotton ?Velvet? worker?s lung Upholstery fabric workers Lycoperdonosis (lycoperdon puffballs)

56. HP Treatment Antigen avoidance Corticosteroids ? 0.5-1 mg/kg daily Alternatives?

57. HP Prevention/Avoidance Reduction of antigen burden Design of facilities-moisture control Preventative maintenance, esp. HVAC Protective devices-filters, respirators

58. HP Prognosis Farmer?s lung ? most recover; 50% have chronic lung dis, but minor, usually emphysematous changes Bird fancier?s lung ? prognosis usually worse. Duration of exposure, older age, greater exposure, all tend toward worsened severity

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