Download Eosinophilic Pneumonia

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 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
Initial Laboratory Eval
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CXR, later CT scan – Dr. Hamilton
Hgb 14.9
Hct 44.5
Platelets 412K
WBC 19.5 Neutrophils 62%
Eosinophils 31%
• ESR 50
Additional Laboratory Data
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BNP 309
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TSH 1.16
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INR 1.2
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IgA 243 (69-309)
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IgE 711 (0-180)
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IgG 1450 (613-1295)
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IgM 207 (53-334)
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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 <1:40
RA negative
Crypto Ag negative
Histoplasma Ag negative
More Laboratory Data
• Neutrophil Cytoplasmic
• Bronchoscopy Data
AB <1:16
AFB neg
Blood cultures neg
Routine cult neg
Legionella Neg(urine)
Fungus-yeast, - crypto
Strept. Pneumo neg(urine)
Stool parasites neg
Biopsy – Dr. O’Dell
Fungal Serology neg
Hospital Course
• Rx – O2, albuterol, ipratropium,Cefepime,
Azithromycin, Bactrim, SoluMedrol
Bronchoscopy 9/22 – bronchitis, mucous
Home on Prednisone 20 mg. BID
Later Outpatient Data
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WBC (on 10/2) 10.4 with 1% Eos
P-ANCA neg
C_ANCA neg
Anti-myeloperoxidase neg
Anti-proteinase neg
Atypical ANCA neg
Pulmonary Eosinophilia Causes
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Drug and Toxin Induced
Helminthic and Fungal Infection
Acute Eosinophilic Pneumonia
Chronic Eosinophilic Pneumonia
Churg – Strauss Syndrome
Others
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
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
Helminthic/Fungal Infection
related
• Tropical Pulmonary Eosinophilia
Wuchereria bancrofti
Brugia malayi
Allergic Broncho-Pulmonary Aspergillosis
Acute Eosinophilic Pneumonia
• Acute, febrile, hypoxic, RF often,
mechanical ventilation
• Bx - DAD, hyaline membranes
• Blood eosinophilia absent
• HIV often
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
Churg – Strauss Syndrome
Allergic granulomatosis and angiitis
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Vasculitis
Sinusitis, asthma, blood eosinophilia
Lung, skin, cardiovascular, GI, nervous
Patchy opacities
Bx-eosinophilic infiltrates, eosinophilic
vasculitis, necrotizing granulomas, and
necrosis
Allergic Broncho-Pulmonary
Aspergillosis
Come back January 14, 2004
Other Causes of Pulmonary
Eosinophilia
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Idiopathic Hypereosinophilic Syndrome
Idiopathic Lung Diseases
Neoplasms
Nonhelminthic Infections – Cocci and
rarely Tuberculosis
9/19/03
9/19/03
9/24/03
Differential diagnosis for peripheral,
bilateral airspace disease
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Eosinophilic pneumonia
BOOP
BAC
Sarcoid
Eosinophilic Pneumonia
• Eosinophils in alveolar spaces
and/or interstitium
• Variable:organizing pneumonia
alveolar macrophages
granulomas
mild vascular inflammation
Etiology
Idiopathic
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Chronic eosinophilic pneumonia
Acute eosinophilic pneumonia
Simple eosinophilic pneumonia (Loeffler’s)
Incidental eosinophilic pneumonia
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
Significant Histologic Findings
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Vasculitis: Churg-Strauss syndrome
drug toxicity
Asthmatic bronchitis:
asthma
chronic eosinophilic
pneumonia
allergic bronchopulmonary
fungal disease
Infectious agents:
fungus
parasites
Dr. Brodsky’s Presentation
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
CHURG-STRAUSS SYNDROME
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Approximately 10% of systemic vasculitis patients.
No gender predominance
Median age –50, but may appear in late 30’s
Uncommon after 65
CHURG-STRAUSS SYNDROME
ETIOLOGY
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Autoimmune Disorder
Allergic Features
Heightened T Cell Immunity
(Pulmonary angiocentric granulomatosis)
Altered humoral immunity (Hyperglobulinemia,
IgE, RF)
Immune Complexes (vasculitis, IC’s, P ANCA)
Rare complication with leukotriene receptor
antogonists
Rare complication with free based cocaine
CHURG-STRAUSS SYNDOME
CLINICAL FEATURES
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Prodromal Phase-Second and Third decadesatopic 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
CHURG-STRAUSS SYNDROME
CLINICAL FEATURES
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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, MI’s
(50% of deaths)
CHURG-STRAUSS SYNDROME
CLINICAL FEATURES
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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)
CHURG-STRAUSS SYNDROME
LABORATORY FEATURES
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Eosinophilia –5,000-9,000
NC/NC Anemia
ESR
IgE
Circulating Immune Complexes
Hyperglobulinemia
+RF
P ANCA
IL2R
BAL – 33% Eos
CHURG-STRAUSS SYNDROME
RADIOGRAPHIC FEATURES
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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
CHURG-STRAUSS SYNDROME
PATHOLOGY
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Eosinophilic Infiltrates
Extensive Necrosis
Eosinophilic Giant Cell Vasculitis, small arteries
and veins
Interstitial and perivascular granulomas
Eosinophilic Lymphadenopathy
CHURG-STRAUSS SYNDROME
TREATMENT
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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
CHURG-STRAUSS SYNDROME
ACR CLASSIFICATION CRITERIA
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Asthma
Eosinophilia – 10% or greater
Mononeuritis multiplex or polyneuropathy
Migratory or transient pulmonary opacities
Paranasal sinus abnormalities
Bx evidence of eosinophilic vasculitis/tissue
eosinophils
ACUTE EOSINOPHILIC
PNEUMONIA
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Acute febrile illness of short duration
Hypoxemic Respiratory Failure
Diffuse pulmonary opacities on CXR
BAL Eosinophilia >25%
Lung Bx – eosinophilic infiltrates (Acute and/or
organizing DAD/eosinophils)
Dx of exclusion (Drugs, Infections, Asthma, Atopic
Disease)
AEP
C-S S
HX
Asthma, Sinusitis,
Diarrhea, Neuropathy
PE
Mild to Moderate SOB
CXR
Diffuse Infiltrates
LAB
 IGE
- P ANCA
BX
Tissue Eos
RX
+ Steroids
Response
Tissue Eos
+ Steroids