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Organizing PneumoniaCatherine Hathaway

AM Report8/20/08

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There are three typesSecondary Organizing Pneumonia: this iswhen an identified cause resulted in thepneumonia . Pneumococcal PNA, atypical bacteria, viral

infxns, fungi, parasites Typically thought that the OP occurs afteretiological agent gone and inflammationcontinues

Cryptogenic Organizing Pneumonia:Idiopathic Bronchiolitis Obliterans OrganizingPneumonia: Granulation tissue is present in thebronchiolar lumen

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Pathophysiology

Histopathology demonstrates intraalveolargranulation tissue with myofibroblasts andcollagen. OP is a histopathologic

diagnosis. Lung architecture is preserved Necrosis and granulomas are not present

3 phases of typical pneumonia congestion, hepatization, and then resolution. If resolution fails then OP develops when the

inflammatory exudate becomes fibrous. 3

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Pathophysiology cont.Epithelial injury, then necrosis and sloughing of pneumocytes leaving denuded alveoliFibrin deposition followed by infiltration of inflammatory cells.The final stage has maturation of these fibroticbuds with deposition of layers of collagen andfibroblasts/myofibroblasts. Re-epithelization of the alveoli preserving architecture.

This is not the case in IPF/UIP bc re-epithelization isabnormal.

No hyaline membranes form in contrast withDAD/ARDS (could be related to severity of initialinjury)

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Cordier, 2006

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Uptodate, COP 2008 Butterfly lesions when intraalveolar budsConnect throu h Kohn ores

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Epidemiology

Relatively rare, some case reports demonstrateincidence of 1.96/100,000 3 . A major teachinghospital had 6-7/100K admissions in one year. 5

50% are idiopathic.Secondary causes include: radiation, infections,drugs, inhalantsAssociated diseases: Connective tissue disorders,

inflammatory disease, sarcoidosis, lung cancer,CF, secondary amyloidosis, ITP, Evans SyndromeThere have been studies connecting working atmicrowave popcorn plants and BOOP

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Patient CharacteristicsNo sex, race predilectionTypically patients are in their 40s 70s,however it has been noted to occur inyoung children with cancerCigarette smoking is not associated as aprecipitating factor In fact has been noted to be 2x as common in

nonsmokers/ex smokers. 3

BMT recipients (for several reasonsincluding inflammation with GVHD,immune suppression and 2/2 infection,and chemotherapeutic drug exposure)

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Clinical Features

Cough, shortness of breath, low gradefevers, malaise Many times can mimic presentation of CAP

Typically symptoms have been present ~1moChest pain can often be a symptomespecially if the location is peripheralClubbing is not present typicallyOften patients (50%) will describe ainfluenza/febrile illness 3-4 months prior

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OP vs IPF/UIPOP: fibrosis and severe honeycombing areunusualBoth have a RESTRICTIVE pattern on PFTsTime course is typically shorter inpresentation for OP (IPF/UIP moreinsidious)OP associated fibrosis typically reversible Collagen type I is predominant in UIP and

Collagen type III is more common in OP. TypeIII is more susceptible to being brokendown/resorbed.

Intra-alveolar buds in OP have many capillaries

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Laboratory Values in OrganizingPneumonia

Typically nonspecific About 50% have leukocytosis Usually ESR and CRP are elevated (70-

80% of the time)

Autoantibodies are uncommon(unless concurrent OP in pt withCTD)

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Radiographic Findings

CXR will show infiltrates, typicallypatchy, peripheral, and can bemultifocal

CT: Ground glass opacity Air bronchograms

Peribronchial distributionRarely will you have effusions,pleural disease, or cavities.

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Diagnostic studiesBronchoscopy High lymphocyte counts (typically higher than

those found in patients with IPF) Lower numbers of macrophages but will be

foamy Proportionately higher PMNs and Eos thannormal patients

Mast cells Decreased CD4/CD8 ratio

FNA Often not helpful because dont getenough architectureOpen biopsy Typically required

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Other things in the DifferentialExclusion of infections very important to rule outTB, fungal infections as these can mimic theradiographic and clinical findings of OPBacterial PNADAD/ARDSHypersensitivity PneumonitisCEP (chronic eosinophilic pna)Pulmonary Dz associated with Connective TissueDzPulmonary involvement associated with IBDMalignancy (low grade lymphoma and BAC)Vasculitis

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Pulmonary involvement associatedwith IBD

Rare, but does occurTypically pleural in location but canappear as mass lesionsWill find granulomas on biopsy, nocaseation, absence of infectiousorganismsTreatment is typically with rituximabCan precede development of IBD sx

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Treatment for OP

Antibiotics not helpful as this isdysregulated healing/immune responseFew cases will resolve on their own, most

patients require treatmentRelapse is an issue (up to 30%) aftersteroids are tapered

If the cause is secondary then treatmentof the underlying cause is important (if possible)

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Treatment cont.

For focal OP can opt for resection aloneTreatment typically is steroids Prednisone 1-1.5 mg/kg daily for 4-8 wks 5 to 1

year 3 For very severe/progressive disease can use

high dose methylprednisolone 125-250mg ivq6 for 3-5 days initially

Other options for progession despitesteroids: Cyclophosphamide (with low dose prednisone) Macrolides (Data limited to a few case reports)

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PrognosisTypically good most patients respondwhile only 1/3 of patients will typicallyhave progressive disease despite steroidsMost patients if they improve will do so in

weeks to 3 monthsRelapse is a problem, often occurringwhen immunosuppression is tapered in13-58%

But will typically respond when steroidsincreasedRarely is this a fatal process

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SHAZAAM!!!

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Resources1. Clinical Bronchiolitis Obliterans in Workers at a Microwave-Popcorn Plant. Kreiss et al. NEJM 8/2002

volume 347:330-338.2. Epler GR, Colby TV, McLoud TC, et al. Bronchiolitis obliterans organizing pneumonia. N Engl J

Med . Jan 17 1985;312(3):152-83. Cordier JF. Cryptogenic organising pneumonia. Eur Respir J . 2006;28(422).4. Kwan, Ali. Bronchiolitis Obliterans Organizing Pneumonia. Emedicine. Last edited 8/2/07. Accessed

8/17/08.5. King T. Cryptogenic Organizing Pneumonia. Uptodate. April 24, 2008. Accessed 8/18/08.6. Coonar AS et al. Pulmonary involvement in inflammatory bowel disease. Ann Thorac Surg. 2007

Nov;84(5):1748-50.7. Krishnan S et al. Lung lesions in children with Crohn's disease presenting as nonresolving pneumonias

and response to infliximab therapy. Pediatrics. 2006 Apr;117(4):1440-3.