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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 PMHAsthma since age 17ImmunotherapyPrednisone use since age 20Hx nasal polyp surgeryHx IVIG for HypogammaglobulinemiaOsteoporosis
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
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 <1:40• RA negative• Crypto Ag negative• Histoplasma Ag negative
More Laboratory Data
• Neutrophil Cytoplasmic
AB <1:16
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. O’Dell
Hospital Course
• Rx – O2, albuterol, ipratropium,Cefepime,
Azithromycin, Bactrim, SoluMedrol
Bronchoscopy 9/22 – bronchitis, mucous
Home on Prednisone 20 mg. BID
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
Pulmonary Eosinophilia - Causes
• 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 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
Other Causes of Pulmonary Eosinophilia
• Idiopathic Hypereosinophilic Syndrome
• Idiopathic Lung Diseases
• Neoplasms
• Nonhelminthic Infections – Cocci and rarely Tuberculosis
Differential diagnosis for peripheral, bilateral airspace disease
• 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– 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
• Vasculitis: Churg-Strauss syndrome
drug toxicity• Asthmatic bronchitis:
asthmachronic eosinophilic
pneumoniaallergic bronchopulmonary fungal disease
• Infectious agents:fungusparasites
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
Approximately 10% of systemic vasculitis patients. No gender predominance Median age –50, but may appear in late 30’s Uncommon after 65
CHURG-STRAUSS SYNDROMEETIOLOGY
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 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
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, MI’s (50% of deaths)
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)
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
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
CHURG-STRAUSS SYNDROMEPATHOLOGY
Eosinophilic Infiltrates Extensive Necrosis Eosinophilic Giant Cell Vasculitis, small arteries
and veins Interstitial and perivascular granulomas Eosinophilic Lymphadenopathy
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
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
ACUTE EOSINOPHILIC PNEUMONIA
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)