Eosinophilic Pneumonia

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Eosinophilic Pneumonia
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Eosinophilic Pneumonia

• Or
• Churg-Strauss Syndrome?

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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

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Initial Laboratory Eval

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

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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

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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

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Hospital Course

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

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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

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Pulmonary Eosinophilia - Causes

• Drug and Toxin Induced
• Helminthic and Fungal Infection
• Acute Eosinophilic Pneumonia
• Chronic Eosinophilic Pneumonia
• Churg Strauss Syndrome
• Others

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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

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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

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Helminthic/Fungal Infection related

• Tropical Pulmonary Eosinophilia
• Wuchereria bancrofti
• Brugia malayi
• Allergic Broncho-Pulmonary Aspergillosis

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Acute Eosinophilic Pneumonia

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

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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

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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

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Allergic Broncho-Pulmonary Aspergillosis

• Come back January 14, 2004

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Other Causes of Pulmonary Eosinophilia

• Idiopathic Hypereosinophilic Syndrome
• Idiopathic Lung Diseases
• Neoplasms
• Nonhelminthic Infections Cocci and rarely
  Tuberculosis

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9/19/03
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9/19/03
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9/24/03
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Differential diagnosis for peripheral, bilateral
airspace disease

• Eosinophilic pneumonia
• BOOP
• BAC
• Sarcoid

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Eosinophilic Pneumonia

• Eosinophils in alveolar spaces
  and/or interstitium
• Variable organizing pneumonia
• alveolar macrophages
• granulomas
• mild vascular inflammation

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Etiology

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

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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

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Significant Histologic Findings

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

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Dr. Brodskys Presentation
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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

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CHURG-STRAUSS SYNDROME

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

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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

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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

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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)

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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)

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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

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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

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CHURG-STRAUSS SYNDROMEPATHOLOGY

• Eosinophilic Infiltrates
• Extensive Necrosis
• Eosinophilic Giant Cell Vasculitis, small
  arteries and veins
• Interstitial and perivascular granulomas
• Eosinophilic Lymphadenopathy

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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

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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

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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)

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• 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