Title: Eosinophilic Pneumonia
1Eosinophilic Pneumonia
2Eosinophilic Pneumonia
- Or
- Churg-Strauss Syndrome?
3Case 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
4Initial 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
5Additional 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
6More 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
7Hospital Course
- Rx O2, albuterol, ipratropium,Cefepime,
- Azithromycin, Bactrim, SoluMedrol
- Bronchoscopy 9/22 bronchitis, mucous
- Home on Prednisone 20 mg. BID
8Later 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
9Pulmonary Eosinophilia - Causes
- Drug and Toxin Induced
- Helminthic and Fungal Infection
- Acute Eosinophilic Pneumonia
- Chronic Eosinophilic Pneumonia
- Churg Strauss Syndrome
- Others
10Drug 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
11Helminthic/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
12Helminthic/Fungal Infection related
- Tropical Pulmonary Eosinophilia
- Wuchereria bancrofti
- Brugia malayi
- Allergic Broncho-Pulmonary Aspergillosis
13Acute Eosinophilic Pneumonia
- Acute, febrile, hypoxic, RF often, mechanical
ventilation - Bx - DAD, hyaline membranes
- Blood eosinophilia absent
- HIV often
14Chronic 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
15Churg 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
16Allergic Broncho-Pulmonary Aspergillosis
- Come back January 14, 2004
17Other Causes of Pulmonary Eosinophilia
- Idiopathic Hypereosinophilic Syndrome
- Idiopathic Lung Diseases
- Neoplasms
- Nonhelminthic Infections Cocci and rarely
Tuberculosis
189/19/03
199/19/03
209/24/03
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22Differential diagnosis for peripheral, bilateral
airspace disease
- Eosinophilic pneumonia
- BOOP
- BAC
- Sarcoid
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28Eosinophilic Pneumonia
- Eosinophils in alveolar spaces
and/or interstitium - Variable organizing pneumonia
- alveolar macrophages
- granulomas
- mild vascular inflammation
29Etiology
- Idiopathic
- Chronic eosinophilic pneumonia
- Acute eosinophilic pneumonia
- Simple eosinophilic pneumonia (Loefflers)
- Incidental eosinophilic pneumonia
30Etiology
- 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
31Significant Histologic Findings
- Vasculitis Churg-Strauss syndrome
- drug toxicity
- Asthmatic bronchitis
- asthma
- chronic eosinophilic pneumonia
- allergic bronchopulmonary fungal
disease - Infectious agents
- fungus
- parasites
32Dr. Brodskys Presentation
33CHURG-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
34CHURG-STRAUSS SYNDROME
- Approximately 10 of systemic vasculitis
patients. - No gender predominance
- Median age 50, but may appear in late 30s
- Uncommon after 65
35CHURG-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
36CHURG-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
37CHURG-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)
38CHURG-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)
39CHURG-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
40CHURG-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
41CHURG-STRAUSS SYNDROMEPATHOLOGY
- Eosinophilic Infiltrates
- Extensive Necrosis
- Eosinophilic Giant Cell Vasculitis, small
arteries and veins - Interstitial and perivascular granulomas
- Eosinophilic Lymphadenopathy
42CHURG-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
43CHURG-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
44ACUTE 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