Ministerul Sanatatii al Republicii Moldova

IP Universitatea de stat de Medicina si Farmacie

N. Testemiteanu

Disciplina “Pneumologie”
Fibroza idiopatica pulmonara
Fibroza pulmonara idiopatica (FPI) sau alveolita
fibrozanta criptogenica, sau pneumonita interstitiala
idiopatica-este o forma specifica a PIF cronice si
asociata la biopsia pulmonara cu manifestarea
histologica de pneumonita interstitiala comuna de
etiologie necunoscuta.
 Factori asociati cu FPI:

•Tabagismul
•Factori ocupationali (fermierii, coaforii, lucratorii la cariere de
piatra, industria avicola, legumicultori, metalurgisti)
•Virusi ( Epstein-Barr, gripaA, paragripa 1 si 3, VHC, HIV-1,
Herepsvirus-6)
•Factorul genetic (proteina C-surfactant seric, scurtarea telomerilor-
pierderea celulelor epiteliale, TNF-a, receptorul C1, IL-1).
FPI-patologie epitelial-
fibroblastica
 Morfopatologie

•Heterogenitatea
•Focare de fibroblasti
•“fagure de miere”
histopatologic

Hematoxolin-eozina, distributie in mozaic a chisturilor

Idiopathic Pulmonary Fibrosis (IPF) Workup
https://emedicine.medscape.com/
 Tabloul Clinic
•Debut insidios, varsta 50-70 ani, cu precadere barbati
•Dispnee progresiva
•Tusea seaca/ cu min. de sputa, paroxistica, refractara la antitusive
•Hipocratism digital
•In stadii tardive ICD
•Initial crepitatie la baze , ulterior “plaman de celofan”
 Examenul paraclinic

1. Radiografia toracica-s. interstitial:

• Opacitati liniare, reticulare neregulate, cortical bilateral
• Localizare bazala si periferica
• Modificari in “fagure de miere” subpleural si zonele inferioare
• Micsorarea V pulmonar
• Limita cordului de aspect neregulat
2. HRCT-diagnostic precoce:
• Opacitati liniare neregulate 80%
• Opacitati in sticla mata 75%
• “fagure de miere” 95%-!!! Cartea de vizita in stadiul avansat
• Bronsiectazii de tractiune si bronsioloectaze 50%
• Ganglioni mediastinali usor mariti (<2cm)
Chest radiograph of a patient with idiopathic pulmonary fibrosis showing bilateral lower lobe
reticular opacities (red circles)
https://emedicine.medscape.com/
Classic subpleural honeycombing (red circle) in a patient with a diagnosis of idiopathic
pulmonary fibrosis.
https://emedicine.medscape.com
A patient with IPF and a
confirmed histologic
diagnosis of usual
interstitial pneumonia.
Note the reticular
opacities (red circle)
distributed in both lung
bases and the minimal
ground-glass opacities
(blue circle)
https://
emedicine.medscape.com
Age: 80 years
Gender: Female
Dyspnea and reduced
exercise tolerance
Conclusion: Advanced
pulmonary fibrosis
with widespread honey
combing, inter and
intralobular septal
thickening and traction
bronchiectasis.
https://
radiopaedia.org/
 Teste functionale- disfunctie restrictiva
•CPT, CV,VR scazute cu VEMS/CV pastrat, DLCO cu 30-50%

Reducere moderată a capacității vitale totale și forțate (aproximativ 50% din cea prevăzută). Este
prezentă și o ușoară reducere a fluxului expirator forțat.
Pulmonary Function Tests in Idiopathic Pulmonary Fibrosis
•Hemoleucograma-N, doar in infectii concomitente neutrofilie.
•Biopsia pulmonara chirurgicala-prelevare din zona de hotar conform
HRCT
•Bronhoscopia cu LBA-neutrofile
 Criterii de diagnostic

Major criteria
•exclusion of other known causes of interstitial lung disease (e.g. toxic effects
of certain drugs, environmental exposures, connective tissue diseases)
•abnormal results of pulmonary function studies, including evidence of
restriction (reduced vital capacity, often with an increased FEV1/FVC ratio)
and impaired gas exchange.
•bibasilar reticular abnormalities with minimal ground-glass opacities at high-
resolution CT: definite UIP pattern on HRCT chest 
•transbronchial lung biopsy or bronchoalveolar lavage shows no features to
support an alternative diagnosis
Minor criteria
•age >50 years
•insidious onset of otherwise unexplained dyspnea on exertion
•duration of illness >3 months
•bibasilar inspiratory crackles.
 Bibliografie

•V. Botnaru “Pneumologie” pag.543-558

•V. Botnaru “Semiologia radiologica a toracelui” pag. 165-169
•V. Botnaru “Medicina interna Breviar”
•Botnaru Victor Imagistica toracica in cazuri clinice comentate.
•Botnaru Victor Pneumologie in tabele si cazuri clinice
•Idiopathic Pulmonary Fibrosis (IPF) https://emedicine.medscape.com
•Idiopathic pulmonary fibrosis https://radiopaedia.org/
•Pulmonary Function Tests in Idiopathic Pulmonary Fibrosis
https://emedicine.medscape.com
•Идиопатический легочный фиброз:диагностика и лечение
https://studfile.net/