Eosinophilic Lung Disease Cases

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Eosinophilic Lung Disease Cases

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1. Eosinophilic Lung Disease Cases William Kelly, MD

3. Eosinophil – our friend and foe Two-lobed, polymorphonuclear leukocyte 12 to 15 um diameter Created by IL-3, Il-5 and GM-CSF Three granule types, largest made up of MBP (major basic protein), kills Parasites, tumor cells, respiratory epithelium Hypodense and normodense varieties

5. Eosinophil – our friend and foe Circulates <18 hours Chemotaxis complement, histamine, ECF-A, PAF, leukotrienes, lymphokines, tumor factos, IL-5 100-400x more in tissues than in blood

7. Eosinophil – competing theories Host defense Modulator of inflammation Tissue destroyer

8. Eosinophils: What’s Normal Blood EOS (#) = 50-250 per microliter Bronchoscopy (BAL) EOS Percentage (%) rather than absolute number Normal volunteers = < 1% ARDS usually none Increased (= >5%) five percent of the time Mostly PCP, drug-related, idiopathic

9. Classification Reeder and Goodrich, 1952 Pulmonary Infiltrates & Eosinophilia (PIE) Croften et al, 1952 Simple pulmonary eosinophilia (Loffler’s) Prolonged pulmonary eosinophilia Tropical eosinophilia Pulmonary eosinophilia with asthma Polyarteritis nodosa (PAN)

10. Classification Liebow and Carrington, 1969 Added Chronic eosinophilic pneumonia (CEP) McCarthy and Pepys, 1973 Either ABPA or “cryptogenic” Schatz et al, 1981 Back to “PIE”

11. Classification Airway Lung parenchyma – known systemic illness Lung parenchyma – idiopathic

12. Airway disorders Asthma Allergic Bronchopulmonary Aspergillosis Bronchocentric granulomatosis

13. Lung parenchyma known systemic illness Infections Bacterial, mycobacterial, fungal, parasites, AIDS (PCP) Interstitial lung disease IPF/UIP Sarcoid SLE

14. Lung parenchyma known systemic illness Hypereosinophilic syndrome Vasculitis (Churg-Strauss) Hodgkin’s disease Drug reactions Lung cancer

15. Lung parenchyma idiopathic Simple eosinophilic pneumonia (SEP, Loffler’s pneumonia) Chronic eosinophilic pneumonia (CEP) Acute eosinophilic pneumonia (AEP)

16. Case 1 23 y.o. male nonsmoker with over a year of nonproductive cough, wheezing, exertional dyspnea Personal and family history of allergies WBC 9400 with 6% eosinophils Refers himself to “U-SCAN” at the mall

18. Case 1 – you should next… Start oral steroids Order thick and thin smears of blood Obtain PFTs with bronchodilator Start diethyl-carbamazine

19. Case 1 – you should next… Start oral steroids Order thick and thin smears of blood Obtain PFTs with bronchodilator Start diethyl-carbamazine

20. Asthma Peripheral eosinophilia often noted “intrinsic” and “extrinsic” Proportional to airflow obstruction Eosinophil MBP results in airway shedding, may contribute to “creola bodies”

21. Case 2 44 y.o. female with hard-to-treat asthma, just weaned off oral steroids (again), presents with increased shortness of breath Treated with antibiotics over the years for pneumonias with radiographic clearing Coughs up “brown plugs” of mucous Sputum culture does not show fungus

22. Case 2 (cont’d) Peripheral eosinophils and IgE are increased Allergist gets “wheal and flare” when they prick the skin with aspergillus antigens Radiologist says CXR shows “tram-tracking” Chest CT is obtained…

24. Case 2 – you should next… Oral steroid “burst” for 5 days then Advair Oral steroids for months Itraconazole A and C B and C

25. Case 2 – you should next… Oral steroid “burst” for 5 days then Advair Oral steroids for months Itraconazole A and C B and C

26. ABPA Asthmatics (6%), Cystic fibrosis (10%) Aspergillus fumigatus Others= Candida albicans, Heliminthosporium, Curvularia lunata Proximal obstruction and bronchiectasis

27. ABPA Difficult asthma Blood eosinophilia Elevated IgE Total and specific Aspergillus skin prick Aspergillus precipitins Radiographic infiltrates Aspergillus in sputum Brown mucous plugs Arthus (Type III) skin reaction Radiographic clues “gloved-finger” “ring shadows” “tram-tracking”

28. ABPA Staging I Acute II Remission III Exacerbation IV Steroid-dependent V Fibrotic

29. ABPA treatment Oral corticosteroids Hi-dose for 1-4 weeks Change to QOD over next three months Slow taper over following three months Itraconazole* Surveillance CXR Q4 x 6, Q6 x 2, Q12 months IgE Q1 month

30. Case 3 54 y.o. male non-smoker presents with an abnormal CXR done for a physical exam On review he does complain of cough, malaise and occasional fevers PFTs show mild obstruction which normalize after bronchodilator

32. Case 3 (cont’d) Blood eosonophils are increased Serum RF and C-ANCA are negative Biopsy is performed No malignancy is found Special stains for AFB and fungus are negative You review the slides with the pathologist…

34. Case 3 – your diagnosis… Eosinophilic granuloma Smear negative, culture negative TB Bronchocentric granulomatosis Sarcoidosis

35. Case 3 – your diagnosis… Eosinophilic granuloma Smear negative, culture negative TB Bronchocentric granulomatosis Sarcoidosis

36. Bronchocentric granulomatosis Liebow 1973 Clinical and radiographic pattern variable Surgical biopsy required Inflammation, esosinophilia, Charcot-Leyden crystals NO extrabronchial granulomas Diagnosis of exclusion TB, fungal infection Wegener’s or Rheumatoid lung disease Aspiration

37. Bronchocentric granulomatosis 1/3 of patients Asthma, eosinophilia, fungal hyphae on bx, aspergillus in sputum “tissue-destructive” ABPA 2/3 of patients Neutrophils without asthma or fungi evident Corticosteroids can be effective

38. Case 4 32 y.o. male with night sweats, weight loss, fever, pruritis presents with a cold, pulseless foot CXR shows interstitial infiltrates and a small effusion CBC: WBC 12000, 56% eosinophils

39. Case 4 – which is FALSE… Cardiac involvement is the leading cause of mortality 50% will have a good response to steroids 40% have pulmonary involvement Venous thrombosis is more common than arterial

40. Case 4 – which is FALSE… Cardiac involvement is the leading cause of mortality 50% will have a good response to steroids 40% have pulmonary involvement Venous thrombosis is more common than arterial

41. Idiopathic Hypereosinophilic Syndrome (HES) “eosinophilic leukemia” Rare, often fatal EOS >1500/microliter for six months 7:1 male predominance Usually age 30s (oldest reported = 70) Blood EOS 30-70% Bronchoscopy (BAL) EOS up to 73%

42. Case 5 38 y.o. male with 8 year history of asthma, chronic rhinitis, presents with fever, malaise, weight loss Hypertension, mononeuritis multiplex noted Labs notable for anemia; increased EOS (9400), IGE and ESR; RF weakly positive; microscopic hematuria, Scr 0.8; P-ANCA is positive

44. Case 5 (cont’d) PFTs show obstruction Bronchoscopy reveals 32% eosinophils but cultures are negative for bacteria, AFB or fungus Thoracentesis reveals an exudate with increased eosinophils Biopsy is performed …

46. Case 5 – which is FALSE… 50% die in three months without treatment With treatment, mean survival has been reported as 9 years 50% of patients may be ANCA positive (usually perinuclear – P-ANCA) Unlike Wegener’s, pulmonary nodules do NOT tend to cavitate Treatment requires corticosteroids along with cytotoxic agents

47. Case 5 – which is FALSE… 50% die in three months without treatment With treatment, mean survival has been reported as 9 years 50% of patients may be ANCA positive (usually perinuclear – P-ANCA) Unlike Wegener’s, pulmonary nodules do NOT tend to cavitate Treatment requires corticosteroids along with cytotoxic agents

48. Churg-Strauss Syndrome Churg and Strauss - 1951 “Allergic angiitis and granulomatosis” Three phases Prodromal allergic rhinitis, asthma (years) Dramatic peripheral eosinophilia (months, yrs) Tissue infiltration like Loffler’s or CEP Systemic vasculitis

49. Churg-Strauss Syndrome Upper airway Rhinitis, polyps, sinusitis Skin (70%) Nodules, purpura, urticaria Neurologic Mono.Multiplex (66%) CNS (27%) Gastrointestinal Abd pain (59%) Diarrhea (33%) Bleeding (18%) Cardiac CHF (47%) Pericarditis (32%) HTN (29%) Renal (49%)

50. Churg-Strauss Syndrome Pathologic diagnosis Renal histology non-specific, often no granulomas TBBX probably inadequate Open lung biopsy = “gold standard” Treatment = steroids x 1 yr, relapse rare Pulse steroids or cytotoxic agents for failure

51. Churg-Strauss Syndrome differential diagnosis Wegener’s Nodules cavitate C-ANCA positive Histology different PAN Medium-sized vessels No lung involvement No eosinophilia CEP No granulomas Not “extra-pulmonary” Idiopathic hyper-eosinophilia syndrome No granulomas No vasculitis

52. Churg-Strauss Syndrome Associated with leukotriene antagonists Zafirlukast, montelukast Incidence = 1:20,000 Probably coincidental with steroid tapering of primary eosinophilic infiltrative disorder

53. Case 6 22 y.o. female referred to you for management of refractory asthma since returning from the Peace Corps in India consisting of nocturnal cough with wheezing and malaise Moist crackles and wheezes are auscultated, No adenopathy or hepatosplenomegaly PFTs show a mixed defect Serum Eosinophilia is 3400

55. Case 6 – which is FALSE… Mosquitoes spread this disease Bronchoscopy (BAL) may show 50% EOS Blood sampling is not helpful Tissue biopsy is required Treatment is diethyl-carbamazine

56. Case 6 – which is FALSE… Mosquitoes spread this disease Bronchoscopy (BAL) may show 50% eos Blood sampling is not helpful Tissue biopsy is required Treatment is diethyl-carbamazine

58. Tropical Pulmonary Eosinophilia Ancylostoma sp.* Ascaris sp.* Brugia Malayi Clonorchis sinesis Dicrofilaria immitis Echinococcus sp. Opisthorchiasis sp. Paragonimus westermani Schistosoma sp. Strongyloides steratocolis* Toxocara sp.* Trichinella spiralis Wuchereria bancrofti

59. Tropical Pulmonary Eosinophilia Ex: W. bancrofti GI symptoms predominate Lymphadenopathy common in children Blood EOS > 3000 Bronchoscopy (BAL) high EOS, IgE, IgG PFTS – usually restriction, 30% mixed

60. Case 7 32 y.o. from southern Texas, 31 weeks pregnant, with dry cough and mild SOB Dysuria two months ago, nitrofurantoin QD Tolerated same with last pregnancy PFTs show restriction Blood eosinophils are mildly elevated Abnormal CXR prompts shielded CT…

62. Case 7 – you recommend… Induce delivery or C-section Stop all medications Add oral steroids Start empiric anti-parasite medication, using one with best FDA pregnancy class Open lung biopsy

63. Case 7 – you recommend… Induce delivery or C-section Stop all medications Add oral steroids Start empiric anti-parasite medication, using one with best FDA pregnancy class Open lung biopsy

64. Drug reactions Common cause of pulmonary infiltrates and blood or BAL eosinophilia Multiple medications: Nitrofurantoin, Sulfasalazine, Phenytoin, Bleomycin, Tetracycline Treatment with dicontinuation of drug and possibly corticosteroids

65. Drug reactions (Ex. 1) Eosinophilic-myalgia syndrome (late 1980s) Contaminated L-tryptophan 50% of ingestions had myalgias, eosinophilia 50% of these had pulmonary involvement = cough, dyspnea, cxr infiltrates, effusions, muscle weakness IgE, CK normal, PFTs with restriction Histology with vasculitis, eosinophils

66. Drug reactions (Ex. 2) “Toxic oil syndrome” (1981-1982) 20,000 cases – 300 deaths in Spain “olive oil” – rapeseed oil with oleoanilide Fever, respiratory/GI distress, rash, adenopathy CXR – interstitial/alveolar infiltrates, Kerley B’s Steroids helpful acutely, others progressed to pulmonary fibrosis of hypertension

67. Case 8 54 y.o. female with history of atopy, seven months of slowly progressive cough, dyspnea, fever, malaise and weight loss PFTS consistent with asthma Blood shows increased IgE and eos CXR and CT are obtained…

71. Case 8 – you can tell her… Most cases resolve spontaneously It is unusual for women to get this It is unusual to get this at her age Her asthma was likely diagnosed within the past few years

72. Case 8 – you can tell her… Most cases resolve spontaneously It is unusual for women to get this It is unusual to get this at her age Her asthma was likely diagnosed within the past few years

73. Chronic Eosinophilic Pneumonia Christoforodis and Molnar 1960 (n=2) Carrington Peak incidence age - 50s Insidious onset – 7.7 months sxs pre dx Cough (90%), fever (87%), SOB (57%), weight loss (57%), asthma (50% - less than 5 yr duration) Peripheral EOS (>6%) = 88% BAL EOS usually >25% and may be 44%

74. CEP IgE elevated (66%) ESR, RF, immune complexes, thrombocytosis may be found Hypoxemia, PFTs with mild restriction Peripheral CXR infiltrates (63%) “photonegative of CHF” only 25%

75. CEP Unlike simple pulmonary eosinophilia (SPE, “Loffler’s pneumonia”) spontaneous resolution is rare (<10%) Steroids 40 mg/day – dramatic resolution Symptoms within 1-2 days CXR infiltrates within 10 days Relapse common in first 6 months

76. Case 9 39 y.o. male “crashes” in through the ER with Sao2 70s on room air and is intubated Per his wife he was in excellent health until the onset of the “flu” with fever to 104 and myalgias 3 days ago CXR shows only a mild interstitial infiltrate– in fact a CTPA gram is done to exclude PE- but infiltrates progress…

78. Case 9 (cont’d) CBC and blood eosinophils are normal Sputum/urine/blood cultures/HIV negative Thoracentesis- elevated pH and eosinophils Bronchoscopy (BAL) is done to exclude infection- 42% eosinophilia is noted

79. Case 9 – you know this differs from CEP because… Steroids are not effective Relapse is uncommon Bronchoscopy (BAL) shows EOS Peripheral blood EOS can be normal B and D

80. Case 9 – you know this differs from CEP because… Steroids are not effective Relapse is uncommon Bronchoscopy (BAL) shows EOS Peripheral blood EOS can be normal B and D

81. Acute Eosinophilic Pneumonia First described 1989 Unknown cause ? Inhaled antigen hypersensitivity Similar to some cases in CML patients Similar to some postoperative events Unlike other ARDS (BAL = neutrophils) Must exclude infection (esp. fungus)

82. Acute Eosinophilic Pneumonia Acute febrile illness < 5 days duration Hypoxemic respiratory failure Diffuse alveolar or mixed CXR infiltrates BAL EOS > 25% Absence of parasitic, fungal or other infxn Prompt/complete response to steroids Failure to relapse after steroids

83. Miscellaneous thoughts (1) Pleural fluid eosinophilia (PFE) Pneumothorax = most common Hemothorax (may take 1-2 weeks) Benign asbestos effusion Pulmonary embolism Parasites/ Fungus/ TB (rare) Drug induced Lymphoma/ Carcinoma (5-8%)

84. Miscellaneous thoughts (2) Eosinophilic Granuloma (EG) “Langerhans cell granulomatosis” 2-5 cases/million, whites, smokers (90%) Pneumothorax (6-20%) “stellate” (star-shaped) fibrosis 50% atypical lymphocytes, +OKT6, S100 NO eosinophilia Treatment = smoking cessation, steroids

85. Airway disorders Asthma Allergic Bronchopulmonary Aspergillosis Bronchocentric granulomatosis

86. Lung parenchyma known systemic illness Infections Bacterial, mycobacterial, fungal, parasites, AIDS (PCP) Interstitial lung disease IPF/UIP Sarcoid SLE

87. Lung parenchyma known systemic illness Hypereosinophilic syndrome Vasculitis (Churg-Strauss) Hodgkin’s disease Drug reactions Lung cancer

88. Lung parenchyma idiopathic Simple eosinophilic pneumonia (SEP, Loffler’s pneumonia) Chronic eosinophilic pneumonia (CEP) Acute eosinophilic pneumonia (AEP)

89. Questions ?

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