Pulmonary fibrosis

(Fr: fibrose pulmonaire). The background incidence of idiopathic pulmonary fibrosis (IPF) has to be taken into account for causality assessment. Imaging at PMID 23672718. The diagnosis of interstitial pulmonary fibrosis is raised in the presence of linear pulmonary opacities and architectural distortion on imaging incl. HRCT, restrictive lung dysfunction, low KCO, and hypoxemia obviating the need for a lung biopsy. Presentation can be chronic, subacute or rapidly progressive. Generally, the condition develops insidiously with dyspnea and bibasilar or diffuse reticular or streaky opacities. Honeycombing can develop with time in longterm survivors. DI-fibrosis may follow a classic episode of chemotherapy- or amiodarone-induced pulmonary toxicity. Separation of DI-fibrosis from idiopathic or CTD (viz. RA or scleroderma)-related fibrosis can be arduous (PMID 23791462). Patients may exacerbate and progress to ARDS. DI-fibrosis may develop late after completion of therapy with chemo agents, irradiation or amiodarone with no evidence of toxicity during treatment or in the meantime. DI-fibrosis rarely occurs as a complication of patterns Ia, b or c. Oxygen and irradiation can trigger the onset of or exacerbate DI-fibrosis. When DI-fibrosis develops during treatment with the offending agent, drug withdrawal may translate into some improvement. Costicosteroid therapy is indicated, but the results are unpredictable
Last update : 17/04/2017

Causative drugs


Tobacco smoke - Cigarette smoking

I.e I - Interstitial/parenchymal lung disease
I.e - Acute eosinophilic pneumonia (AEP)
I.g I - Interstitial/parenchymal lung disease
I.g - Pulmonary fibrosis
I.h I - Interstitial/parenchymal lung disease
I.h - Subclinical pulmonary opacities
I.j I - Interstitial/parenchymal lung disease
I.j - Exogenous lipoid pneumonia
I.ai I - Interstitial/parenchymal lung disease
I.ai - Pulmonary fibrosis and emphysema syndrome
IV.a IV - Airway involvement
IV.a - Bronchospasm - Asthma
IV.d IV - Airway involvement
IV.d - Cough (lone)
IV.g IV - Airway involvement
IV.g - Syncope - Cough syncope
IV.n IV - Airway involvement
IV.n - Obstructive airway dysfunction (see also IVc)
IX.q IX - Neuromuscular / CNS involvement - Disordered breathing during sleep
IX.q - Obstructive sleep apneas
X.m X - Systemic conditions, syndromes and reactions
X.m - Pulmonary and/or systemic vasculitis (ANCA status MPO, unknown or negative)
X.ac X - Systemic conditions, syndromes and reactions
X.ac - Anti-GBM antibody disease (Goodpasture-like) or flare of
X.ah X - Systemic conditions, syndromes and reactions
X.ah - Granulomatosis with polyangiitis (w/wo ANCA) or flare of
XI.m XI - Miscellaneous
XI.m - Pulmonary emphysema - Lung cysts or bullae
XI.ag XI - Miscellaneous
XI.ag - Vertebral compression fracture
XI.ah XI - Miscellaneous
XI.ah - Fainting - Syncope
XI.an XI - Miscellaneous
XI.an - Disrupted lung surfactant function
XIII.a XIII - Neoplastic conditions
XIII.a - Lung cancer
XIV.c XIV - Hemoglobinopathies-Abnormal hemoglobins states (acquired)
XIV.c - Carboxyhemoglobinemia
XV.k XV - Pulmonary pathology
XV.k - Path: Desquamative interstitial pneumonia (DIP-pattern)
XV.y XV - Pulmonary pathology
XV.y - Path: Respiratory bronchiolitis (RB-ILD-pattern)
XV.z XV - Pulmonary pathology
XV.z - Path: Chronic obliterative broncholitis (see also IVc, IVn)
XV.ay XV - Pulmonary pathology
XV.ay - Path: Smoking-related interstitial fibrosis (SRIF). Respiratory bronchiolitis with fibrosis
XV.bw XV - Pulmonary pathology
XV.bw - Path: Airspace enlargement with fibrosis
XVI.p XVI - Imaging
XVI.p - Imaging: Small irregular opacities - ILA - Diffuse reticulation
XVI.ac XVI - Imaging
XVI.ac - Imaging: A generalized increase in lung density
XVII.a XVII - Infections & related conditions
XVII.a - Pneumonia - Respiratory tract infection