Organizing pneumonia

ORGANIZING PNEUMONIA

BRONCHIOLITIS OBLITERANS ORGANISING PNEUMONIA

Presented by

Dr. Mohamed A/Aziz Omer Mustafa

Consultant chest physician

MD Re spirato ry me dicineSMSB

Idiopathic

BRONCHIOLITIS OBLITERANS ORGANISING PNEUMONIA (BOOP)

CRYPTOGENIC ORGANISING PNEUMONIA (COP)

SECONDRY ORGANIZING PNEUMONIA (SOP)

Terms

• Bronchiolitis

Bronchiolitis obliterans (BO)

Obliterative bronchiolitis (OB)

Constrictive bronchiolitis(CB)

• Bronchiolitis obliterans syndrome (BOS)

• Pneumonia

• Organizing pneumonia

• Idiopathic interstitial pneumonia ( diffuse alveolar damage)

Bronchiolitis

• Is an inflammatory reaction that follows damage to the bronchiolar epithelium of the small conducting airways

Subsequent healing leads to excessive proliferation of granulation tissue within the airway walls , lumen or both.

Depending on disease stage the repair process may cause narrowing and distortion of the small airway (constrictive bronchiolitis ) or complete obliteration ( bronchiolitis obliterans)

BRONCHIOLITIS OBLITERANS

• Rare form of non-reversible Obstructive lung disease

• Scarring

• Polyploidy obliteration of the lumen of the bronchioles without involving of the distal lung parenchyma.

• Infants - adenovirus

BRONCHIOLITIS OBLITERANSSYMPTOMS & SIGNS

• Dry cough

• SOB

• WHEEZES

• FEV1 16-21%

• Desaturation

BRONCHIOLITIS OBLITERANSCAUSES

• Viruses

• Collagen vascular diseases

• Transplant rejection

• Stevens-Jonson's syndrome

• Pneumocystis Juversi pneumonia

• Drug reaction

• Aspiration

• Bronchopulmonary dysplasia-prematurity

• Toxic fumes

BOS

a distinct clinical entity

occurring in the transplanted lung

involves the small airways primarily

should not be confused with BO or BOOP

PNEUMONIA - PNEUMONITIS

• Inflammatory condition of the lung affecting primarily the microscopic airspaces known as alveoli .

• Congestion , hepatization , resolution

• …. Viruses , bacteria ,fungi , parasites

• > 100 strains identified

• Mixed infection 45% children : 15% adults

• Isolation of causative agent 50% despite careful testing

Pneumonitis

• Certain drugs

• Chemical burns

• Autoimmune diseases

• Radiation

• Thermal injuries

HISTORY• HIPPOCRATES 460-370 BC

• 12 November world pneumonia day

• 450 million /year

• 4 million deaths /year

• 7% world’s total deaths

• Greatest < 5 >75 yrs

• 8th leading death in the states 2009

PNEUMONIA IMAGING

• CXR does not always match the severity of pneumonia

• Not reliably separate between bacterial infection and viral infection

• CXR typically clear within one month

Pneumonic patch

Interstitial lung disease

• Is a class of diffuse lung diseases .

• It is a common pathological response of the lung to a wide variety of known & unknown cause .

• It is often referred to interstitial pneumonia , IP or interstitial lung disease ILD

• Until 1960 IPF remained largely a clinical diagnosis considered as a single entity

• Has been felt to be a terminal disease with poor prognosis

because of lack of response to immunosuppressive therapy only 30% respond

• only 30% histopathology done

ILD

Several acute and chronic lung disorders with variable degrees of pulmonary fibrosis ,variable degree of disease progression & variable response to treatment.

Retrospective review of clinical & histopathology data allows IPF to be classified into distinct subsets that differ in clinical course , prognosis & response to treatment .

ILD

• NSIP , LIP , DIP ,RBI lung disease , UIP , OP

• In some ILDs , the small airways- the respiratory and terminal bronchioles- are primarily affected .

respiratory bronchiolitis-associated interstitial lung disease (RBILD)

idiopathic , smoking related in contrast to BO .

Known factors or insults lead toInterstitial lung disease

• Drugs : antineoplastic (bleomycin , methotrexate )

: antimicrobial (nitrofrantoin , penicillin )

:amidorone

: hydroclorothiazide

• Hypersensitivity pneumonia

Farmer’s lung , pigeon breeder’s lung

. Dusts:

Asbestos , silica

Viral agents … mycoplasma pneumonia

. CD

ILD

.

It is important to differentiate terms used to define clinical entities and terms describing the pathologic lesions of these disorders .

Clinical syndromes IPF,CFA ,idiopathic BOOP,COP,BO,BOS ,Hamman-Rich syndrome .

Pathological:UIP , LIP , giant cell interstitial pneumonitis , diffuse alveolar damage, non classifiable interstitial pneumonia , BOOP .

D\DILD

• Clinical setting:-

• First: requires consideration of a variety of other pathological processes that mimic the interstitial disorders in appropriate clinical setting

• Second: Physician should be aware of at least 150 other clinical entities & situations associated with ILD

• Third: Owing the broad D\D & the availability of various evolving invasive & non-invasive diagnostic techniques, the best approach to use is to establish a specific diagnosis which is frequently difficult to determine

• Fourth: In a significant proportion of patients a conclusive cause can't be ascertained even when the most invasive diagnostic pathways are taken.

• Finally , even when a specific diagnosis is made, an effective therapeutic regimen is not available for many patients with ILD

????????BOOP

Bronchiolitis obliterans organizing pneumonia

Is a type of diffuse INTERSTITIAL LUNG DISEASE that affects the distal bronchioles , respiratory bronchioles , alveolar ducts and alveolar wall.

Type of lung injury and repair

Failure of resolution of acute pneumonia

The primary area of injury is within the alveolar wall.

BOOP Hx

• As a distinct clinical entity 1st described by Davison in 1983

• Gary Epler 1985 gave the name BOOP

• Inflammation of the bronchioles & surrounding tissue in the lung.

• Non infectious- infectious

• Complication of an existing chronic inflammatory disease such as RA

• Side effect of certain medication such as amidarone

• M\F 1:1

• Age 50-60 \ 20-80

• No relation to smoking

Organizing pneumonia

• Refers to unresolved pneumonia in which alveolar exudates persists & eventually undergo fibrosis .

. presence of buds of granulation tissue progressing from fibrin exudates to loose collagen containing fibroblast.

Accessory finding in other inflammatory diseases such as vasculitis

. It may also be a feature of organizing stage of ARDS

• A complication of an existing chronic inflammatory disease

or a side effect of certain medication .

• organizing Pneumonia of determined causes

• organizing Pneumonia of unknown cause occurring in a specific context causes

• Cryptogenic organizing pneumonia

Undetermined causes of organizing Pneumonia

• RA , Sjorgen syndrome---------- SLE ,SS

• Sweet”s disease

• HCV thyroiditis

• Crhons disease…..UC

• Behcets”s …. Wegener”s granulomatosis ….PN

• Polymyalgia Rheumatica

• Myelodysplasia

• Leukemia ….. Cancer

• Mesangiocapillary GN

• Chronic rejection …. Graft versus host disease

Determined causes of organizing Pneumonia

• Infection

• Drugs

• Radiation

• It was long been known that radiation may induce radiation pneumonitis

• However a syndrome similar to COP has been identified recently

• It differs strikingly from usual radiation pneumonitis in that the pulmonary infiltrate occur or migrate outside the radiation fields add respond good to steroids.

• Interestingly, unilateral breast irradiation, whether or not it results in pneumonitis , has been shown to induce bilateral lymphocytic alveolitis with activated CD4+Tcells

Infectious causes of organising pneumonia

• Bacteria Chlamydia pneumoniae

Coxiella burnetii

Legionella pneumophila

Mycoplasma pneumoniae

Nocardia asteroides

Pseudomonas aeruginosa

Serratia marcescens

Staphylococcus aureus

Streptococcus group B (newborn treated by extracorporeal oxygenation)

Streptococcus pneumoniae

Infectious causes of organising pneumoniacont…..

• Viruses Herpes virus

Human immunodeficiency virus

Influenza virus

Parainfluenza virus

• Parasites Plasmodium vivax

• Fungi Cryptococcus neoformans

Penicillium janthinellum

Pneumocystis carinii (in AIDS )

Clinical approach

• Medical Hx , environmental factors, occupational exposures, medication & drug usage, FH

• Age , smoking, sex

• Onset & Duration of symptoms

• Rate of disease progression

• Extra pulmonary symptoms

Clinical approach …cont.

• Acquired immunodeficiency & transplant

• Diffuse neoplasia

• CHF

• Pulmonary vascular disorder

• CBC, ESR, LFT, electrolytes, RFT

BOOP diagnosis

• Diagnosis is suspected after no response to multiple antibiotics & blood & sputum cultures are –ve for organisms

. Clinical picture & radiological images resemble infectious pneumonia

• CXR similar to an extensive pneumonia wide spread white patches

small nodular opacities 50% & large nodules in 15%

• Preservation of normal lung volume

• Migrate

• Persists or progresses

BOOP – CT

• CT may be used to confirm the diagnosis ( findings are typical enough to allow the doctor to make the diagnosis without ordering additional tests)

• …airspace consolidation with airbronchogram , lower zone predominance , sub pleural or peribronchial distribution 50%.

• Ground glass appearance in most pt , honeycombing in some

• Diffuse bilateral infiltratation

• as a solitary focal lesion associated usually with subacute or chronic inflammatory illness, it occurs in the upper lobes and may cavitate.

• .

BOOP - histopathology• Confirmation

• Broncoscopic biopsy !

• VATS

• Histology: presence of polyploidy plugs of loose organizing connective tissue

( Masson bodies ) within the alveolar ducts , alveoli , bronchioles .

• Intraluminal polyps in respiratory bronchioles , alveolar ducts , alveolar spaces .

• Accompanied by organizing pneumonia in the more distant parenchyma ( proliferative bronchitis)

Diagnosis of exclusion• COP is a diagnosis of exclusion .

• Once confirmation done , physician has to rule out :-

Chronic esinophilic pneumonia

Hypersensitivity pneumonia

Bacterial …. Viral

CD

Drug reaction

TREATMENT

• Spontaneous

• Macroloids

• Steroids

• Cytotoxic drugs

• Transforming growth factor , pirfenidone

Typical imaging pattern of organising pneumonia with patchy alveolar opacities on chest radiograph.

Cordier J Thorax 2000;55:318-328

Copyright © BMJ Publishing Group Ltd & British Thoracic Society. All rights reserved.

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Organizing pneumonia

Health & Medicine

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