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<strong>OP</strong>- C<strong>OP</strong>- BO<strong>OP</strong><br />

Σ Α Παπίρης


Organizing pneumonia is<br />

a non-specific<br />

response to<br />

various<br />

forms of lung injury and is<br />

the pathological hallmark<br />

of the distinct<br />

clinical entity termed<br />

cryptogenic organizing<br />

pneumonia


Organizing pneumonia is one of the<br />

main reparative reactions<br />

to acute injury by the lung and<br />

reflects the incomplete<br />

resolution of inflammation within<br />

the alveoli, and to a lesser extent the<br />

distal bronchioles


<strong>OP</strong>- C<strong>OP</strong>- BO<strong>OP</strong><br />

• Organising pneumonia of determined<br />

cause<br />

• Organising pneumonia of undetermined<br />

cause but occurring in a specific and<br />

relevant context<br />

• Cryptogenic (idiopathic) organising<br />

pneumonia


Causes of organizing pneumonia and associations with other conditions


The “typical”” C<strong>OP</strong> syndrome<br />

Flu-likelike illness accompanied with fever,<br />

non-productive<br />

cough, malaise, anorexia and weight<br />

loss.<br />

Dyspnoea is common but usually mild and evident<br />

only on exertion.<br />

Less common symptoms are bronchorrhoea, haemoptysis<br />

(but<br />

severe haemoptysis is exceedingly rare),<br />

chest pain,<br />

arthralgia and night sweats.


<strong>OP</strong> – C<strong>OP</strong> - BO<strong>OP</strong><br />

Lange, a German pathologist, first described the pathological findings of<br />

BO<strong>OP</strong> in two autopsies in 1901<br />

• Davison AG, Heard BE, McAllister WAC, and Turner-<br />

Warwick ME. Cryptogenic organizing pneumonitis.<br />

Q J Med 1983; 52: 382-394<br />

394<br />

• Epler GR, Colby TV, McLoud TC, and Gaensler EA.<br />

Bronchiolitis obliterans organizing pneumonia.<br />

N Engl J Med 1985; 312: 152-158<br />

158


Liebow &<br />

Carrington 1969<br />

Katzenstein<br />

1997<br />

Müller & Colby<br />

1997<br />

ATS / ERS 2002<br />

UIP<br />

UIP<br />

UIP<br />

UIP<br />

DIP<br />

DIP / RBILD<br />

DIP<br />

DIP /RBILD<br />

BIP<br />

BO<strong>OP</strong><br />

C<strong>OP</strong><br />

AIP<br />

AIP<br />

AIP<br />

NSIP<br />

NSIP<br />

NSIP<br />

LIP<br />

LIP<br />

GCIP


BO<strong>OP</strong> (C<strong>OP</strong>)<br />

Κατάταξη των διάμεσων παρεγχυματικών<br />

νοσημάτων πνεύμονα<br />

Διάχυτα Παρεγχυματικά Νοσήματα Πνεύμονα<br />

Φάρμακα, Αυτοανοσία,<br />

Επαγγελματικά, Λοιμώδη,<br />

Κακοήθη κλπ<br />

ΙΔΠς<br />

Κοκκιωματώσεις<br />

Άλλα σπάνια<br />

IPF 55%<br />

Άλλες<br />

DIP 15%<br />

RBILD<br />

AIP


BO<strong>OP</strong> Επιδημιολογικά δεδομένα<br />

• 6-77 ασθενείς /100.000 εισαγωγές σε μεγάλα νοσοκομεία<br />

• Μη καπνιστές / καπνιστές: 2 / 1<br />

• Ομοιόμορφη κατανομή ανάμεσα στα 2 φύλα<br />

• Μέση ηλικία εμφάνισης 55 έτη<br />

Alasaly K, et al. Medicine 1995; 74: 201-211<br />

211


Organizing pneumonia:<br />

the many morphological faces


Cordier et al. divided patients with BO<strong>OP</strong> in to two groups based on<br />

radiographic patterns and used this classification to predict outcome<br />

in each group.<br />

Patients with localized disease were rarely symptomatic and had<br />

overall a good prognosis, whereas patients with diffuse disease were<br />

more often symptomatic, required treatment with corticosteroids and<br />

had variable outcomes


Bronchiolitis obliterans organizing<br />

pneumonia in cancer<br />

A bar graph illustrating the relationship of radiographical patterns and the type of<br />

underlying malignancy in 43 patients with cancer and BO<strong>OP</strong>. Nodules (■), mass (▒)<br />

and infiltrate (□).


BO<strong>OP</strong> (C<strong>OP</strong>)<br />

treatment<br />

• Corticosteroid therapy is the most common treatment.<br />

• Complete clinical recovery, physiological<br />

improvement, and normalization of the chest film are<br />

seen in two thirds of patients<br />

• Approximately one-third<br />

demonstrate persistent<br />

disease. In general, clinical improvement is rapid,<br />

within several days or a few weeks. Occasionally,<br />

recovery is quite dramatic<br />

• Delay in treatment increases the risk of more severe<br />

disease and relapses


BO<strong>OP</strong> (C<strong>OP</strong>)<br />

treatment


BO<strong>OP</strong> (C<strong>OP</strong>)<br />

treatment<br />

•The<br />

therapy of choice is prednisone, , 0.75 mg/kg<br />

kg/day<br />

with gradual<br />

tapering of dosage in the following several months<br />

•Several<br />

day course of high-dose<br />

parenteral corticosteroid therapy may<br />

be needed in patients with rapidly progressive C<strong>OP</strong><br />

•The<br />

usual duration of corticosteroid therapy is 6 to 12 months<br />

•Relapse<br />

is seen in one-third<br />

of the patients if a short treatment course<br />

of less than 3 months of corticosteroid therapy is used<br />

•Prednisone<br />

can eventually be given on an alternate day dosage.<br />

•Some<br />

of these patients require low-dose<br />

maintenance prednisone<br />

therapy for several years to maintain stabilization with chronic<br />

symptoms


start<br />

with 0.75 mg/kg/day of prednisone for 4 weeks,<br />

then<br />

0.5 mg/kg/day for 6 weeks,<br />

then 20 mg/day for 6 weeks,<br />

then 5 mg/day for 6 weeks.


The<br />

occurrence of relapses in C<strong>OP</strong> could be<br />

regarded as a relatively benign and acceptable<br />

phenomenon, rather than a dangerous event<br />

requiring aggressive management.


1<br />

delayed treatment increases<br />

the risk of relapses;<br />

2<br />

mild cholestasis identifies a subgroup of<br />

patients with multiple relapses;<br />

3<br />

relapses do not affect outcome,<br />

and prolonged therapy to suppress<br />

relapses appears unnecessary;<br />

4<br />

a standardized treatment allows a<br />

reduction in steroid doses.


• Difffuse<br />

Panbronchiolitis<br />

• Cystic Fibrosis<br />

• Bronchiectasis<br />

• Chronic Sinusitis<br />

• Chronic otitis media<br />

• Nasal poliposis<br />

• Chronic Bronchitis<br />

• BO<strong>OP</strong>


While the use of<br />

macrolide therapy for treatment of C<strong>OP</strong>/BO<strong>OP</strong> is<br />

feasible, the decision to use it should be made on a<br />

case-by<br />

by-case basis.<br />

Before any definitive recommendations<br />

can be made, more information is needed,<br />

such as which patients are likely to respond to<br />

macrolide therapy, and the proper dosage and duration<br />

of macrolide therapy.

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