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Eosinophilic Pneumonia

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Eosinophilic Pneumonia Eosinophilic Pneumonia Or Churg-Strauss Syndrome? Case Report- J.N., 40 WF Admitted 9/19/03 9/24/03 for fever, congestion, dyspnea, chest ... – PowerPoint PPT presentation

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Title: Eosinophilic Pneumonia


1
Eosinophilic Pneumonia
2
Eosinophilic Pneumonia
  • Or
  • Churg-Strauss Syndrome?

3
Case Report- J.N., 40 WF
  • Admitted 9/19/03 9/24/03 for fever, congestion,
    dyspnea, chest tightness, abdominal cramps, and
    diarrhea
  • Extensive PMH
  • Asthma since age 17
  • Immunotherapy
  • Prednisone use since age 20
  • Hx nasal polyp surgery
  • Hx IVIG for Hypogammaglobulinemia
  • Osteoporosis

4
Initial Laboratory Eval
  • CXR, later CT scan Dr. Hamilton
  • Hgb 14.9
  • Hct 44.5
  • Platelets 412K
  • WBC 19.5 Neutrophils 62 Eosinophils
    31
  • ESR 50

5
Additional Laboratory Data
  • BNP 309
  • TSH 1.16
  • INR 1.2
  • IgA 243 (69-309)
  • IgE 711 (0-180)
  • IgG 1450 (613-1295)
  • IgM 207 (53-334)
  • IgG sub 1 597 (240-1118)
  • IgG sub 2 537 (124-549)
  • IgG sub 3 32 (21-134)
  • IgG sub 4 609 (7-89)
  • ANA lt140
  • RA negative
  • Crypto Ag negative
  • Histoplasma Ag negative

6
More Laboratory Data
  • Neutrophil Cytoplasmic
  • AB lt116
  • Blood cultures neg
  • Legionella Neg(urine)
  • Strept. Pneumo neg(urine)
  • Stool parasites neg
  • Fungal Serology neg
  • Bronchoscopy Data
  • AFB neg
  • Routine cult neg
  • Fungus-yeast, - crypto
  • Biopsy Dr. ODell

7
Hospital Course
  • Rx O2, albuterol, ipratropium,Cefepime,
  • Azithromycin, Bactrim, SoluMedrol
  • Bronchoscopy 9/22 bronchitis, mucous
  • Home on Prednisone 20 mg. BID

8
Later Outpatient Data
  • WBC (on 10/2) 10.4 with 1 Eos
  • P-ANCA neg
  • C_ANCA neg
  • Anti-myeloperoxidase neg
  • Anti-proteinase neg
  • Atypical ANCA neg

9
Pulmonary Eosinophilia - Causes
  • Drug and Toxin Induced
  • Helminthic and Fungal Infection
  • Acute Eosinophilic Pneumonia
  • Chronic Eosinophilic Pneumonia
  • Churg Strauss Syndrome
  • Others

10
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

11
Helminthic/Fungal Infection related
  • Transpulmonary larvae migration-Lofflers
  • Ascaris lumbricoides
  • Hookworm
  • Strongyloides stercoralis
  • Pulmonary Parenchymal Invasion
  • Helminths, e.g. Paragonimiasis
  • Heavy hematogenous seeding-Trichinosis,
    Strongyloidiasis, Schistosomiasis, Cutaneous and
    visceral larva migrans

12
Helminthic/Fungal Infection related
  • Tropical Pulmonary Eosinophilia
  • Wuchereria bancrofti
  • Brugia malayi
  • Allergic Broncho-Pulmonary Aspergillosis

13
Acute Eosinophilic Pneumonia
  • Acute, febrile, hypoxic, RF often, mechanical
    ventilation
  • Bx - DAD, hyaline membranes
  • Blood eosinophilia absent
  • HIV often

14
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

15
Churg Strauss SyndromeAllergic granulomatosis
and angiitis
  • Vasculitis
  • Sinusitis, asthma, blood eosinophilia
  • Lung, skin, cardiovascular, GI, nervous
  • Patchy opacities
  • Bx-eosinophilic infiltrates, eosinophilic
    vasculitis, necrotizing granulomas, and necrosis

16
Allergic Broncho-Pulmonary Aspergillosis
  • Come back January 14, 2004

17
Other Causes of Pulmonary Eosinophilia
  • Idiopathic Hypereosinophilic Syndrome
  • Idiopathic Lung Diseases
  • Neoplasms
  • Nonhelminthic Infections Cocci and rarely
    Tuberculosis

18
9/19/03
19
9/19/03
20
9/24/03
21
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22
Differential diagnosis for peripheral, bilateral
airspace disease
  • Eosinophilic pneumonia
  • BOOP
  • BAC
  • Sarcoid

23
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24
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25
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28
Eosinophilic Pneumonia
  • Eosinophils in alveolar spaces
    and/or interstitium
  • Variable organizing pneumonia
  • alveolar macrophages
  • granulomas
  • mild vascular inflammation

29
Etiology
  • Idiopathic
  • Chronic eosinophilic pneumonia
  • Acute eosinophilic pneumonia
  • Simple eosinophilic pneumonia (Loefflers)
  • Incidental eosinophilic pneumonia

30
Etiology
  • Secondary Eosinophilic Pneumonia
  • Infection parasites, fungi
  • Drugs
  • Immunologic asthma, allergic
    bronchopulmonary fungal disease, collagen
    vascular disease, Churg-Strauss syndrome
  • Systemic HIV, malignancy, idiopathic
    hypereosinophilia syndrome

31
Significant Histologic Findings
  • Vasculitis Churg-Strauss syndrome
  • drug toxicity
  • Asthmatic bronchitis
  • asthma
  • chronic eosinophilic pneumonia
  • allergic bronchopulmonary fungal
    disease
  • Infectious agents
  • fungus
  • parasites

32
Dr. Brodskys Presentation
33
CHURG-STRAUSS SYNDROME(ALLERGIC GRANULOMATOSIS
AND ANGITIS)
  • MULTI SYSTEM DISORDER
  • Allergic Rhinitis
  • Asthma
  • Peripheral Blood Eosinophilia
  • Lung involvement most common followed by skin
  • Cardiovascular, GI, CNS

34
CHURG-STRAUSS SYNDROME
  • Approximately 10 of systemic vasculitis
    patients.
  • No gender predominance
  • Median age 50, but may appear in late 30s
  • Uncommon after 65

35
CHURG-STRAUSS SYNDROMEETIOLOGY
  • Autoimmune Disorder
  • Allergic Features
  • Heightened T Cell Immunity
  • (Pulmonary angiocentric granulomatosis)
  • Altered humoral immunity (Hyperglobulinemia,
    ?IgE, ?RF)
  • Immune Complexes (vasculitis, ICs, P ANCA)
  • Rare complication with leukotriene receptor
    antogonists
  • Rare complication with free based cocaine

36
CHURG-STRAUSS SYNDOMECLINICAL FEATURES
  • Prodromal Phase-Second and Third decades-atopic
    disease, allergic rhinitis, asthma
  • Eosinopilic Phase-Eosinophilia, infiltration of
    multiple organs-lung, GI tract.
  • Vasculitic Phase-Third and Fourth Decades-life
    threatening systemic vasculitis medium and small
    vessels. Constitutional complaints

37
CHURG-STRAUSS SYNDROMECLINICAL FEATURES
  • Asthma-precedes vasculitis by 8-10 years Usually
    chronic, severe, steroid dependent.
  • Nasal and Sinus Disease nasal obstruction,
    recurrent sinusitis, nasal polyposis, chronic
    otitis
  • Skin disease-sub Q nodules extensor surfaces,
    hands, legs. Palpable purpura, nodules (67)
  • Cardiovascular Disease-pericarditis, CHF, MIs
    (50 of deaths)

38
CHURG-STRAUSS SYNDROMECLINICAL FEATURES
  • Neurologic Disease-peripheral neuropathy
    mononeuritis multiplex, strokes (75)
  • Renal Disease-focal segmental GN with crescents,
    necrosis, P ANCA (80)
  • GI Disease-Abdominal pain, diarrhea, GI Bleeding,
    Colitis (59)
  • Muscoloskeletol disease-Myalgias, migiatory
    polyarthralgias, arthritis (uncommon)

39
CHURG-STRAUSS SYNDROME LABORATORY FEATURES
  • Eosinophilia 5,000-9,000
  • NC/NC Anemia
  • ?ESR
  • ?IgE
  • Circulating Immune Complexes
  • Hyperglobulinemia
  • RF
  • P ANCA
  • ?IL2R
  • BAL 33 Eos

40
CHURG-STRAUSS SYNDROMERADIOGRAPHIC FEATURES
  • Transient patchy opcacities (75) without lobar
    or segmental distribution
  • Axillary, peripheral distribution
  • Diffuse Interstitial/Miliary pattern
  • Pulmonary Hemorrhage
  • Nodular Disease
  • Pleural effusions (exudative, Eos) (30)
  • Pulmonary arteries enlarged, vasculitis sign

41
CHURG-STRAUSS SYNDROMEPATHOLOGY
  • Eosinophilic Infiltrates
  • Extensive Necrosis
  • Eosinophilic Giant Cell Vasculitis, small
    arteries and veins
  • Interstitial and perivascular granulomas
  • Eosinophilic Lymphadenopathy

42
CHURG-STRAUSS SYNDROMETREATMENT
  • Corticosteroids 0.5 to 1.5 mg/kg for 6-12 wks
  • Monitor ESR, EOS, CXR
  • Late relapses uncommon 70 5 yr survival
  • Inhaled Steroids
  • CTX, AZA, IVIG
  • Poorer Prognosis Cardiac Failure or MI,
    Cerebral hemorrhage, Renal Failure, GI bleed

43
CHURG-STRAUSS SYNDROMEACR CLASSIFICATION CRITERIA
  • Asthma
  • Eosinophilia 10 or greater
  • Mononeuritis multiplex or polyneuropathy
  • Migratory or transient pulmonary opacities
  • Paranasal sinus abnormalities
  • Bx evidence of eosinophilic vasculitis/tissue
    eosinophils

44
ACUTE EOSINOPHILIC PNEUMONIA
  • Acute febrile illness of short duration
  • Hypoxemic Respiratory Failure
  • Diffuse pulmonary opacities on CXR
  • BAL Eosinophilia gt25
  • Lung Bx eosinophilic infiltrates (Acute and/or
    organizing DAD/eosinophils)
  • Dx of exclusion (Drugs, Infections, Asthma,
    Atopic Disease)

45
  • AEP C-S S
  • HX Asthma, Sinusitis,
  • Diarrhea, Neuropathy
  • PE Mild to Moderate SOB
  • CXR Diffuse Infiltrates
  • LAB ? IGE
  • - P ANCA
  • BX Tissue Eos Tissue Eos
  • RX Steroids Steroids
  • Response
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