Idiopathic pulmonary fibrosis

Updated: July 4, 2024

Background

Epidemiology

Anatomy

Pathophysiology

Etiology

Genetics

Prognostic Factors

Clinical History

Physical Examination

Age group

Associated comorbidity

Associated activity

Acuity of presentation

Differential Diagnoses

Laboratory Studies

Imaging Studies

Procedures

Histologic Findings

Staging

Treatment Paradigm

by Stage

by Modality

Chemotherapy

Radiation Therapy

Surgical Interventions

Hormone Therapy

Immunotherapy

Hyperthermia

Photodynamic Therapy

Stem Cell Transplant

Targeted Therapy

Palliative Care

Medication

pirfenidone

1st week:

267 mg 3 times a day for 7 days
2nd week: 534 mg 3 times a day for the next 8 to 14 days
3rd week: 801 mg 3 times a day for day 15 and thereafter (do not exceed 2,403 mg/day)

pirfenidone

1st week:

267

3 times a day

days

2nd week: 534 mg 3 times a day for the next 8 to 14 days
3rd week: 801 mg 3 times a day for day 15 and thereafter (do not exceed 2,403 mg/day)

nintedanib

150

Orally 

twice a day

Interstitial Lung Diseases with a Chronic Fibrosing Progressive Phenotype
150 mg orally 2 times a day
Interstitial Lung Disease with associated Systemic Sclerosis
150 mg orally 2 times a day

Dose Adjustments

Dosage Modifications
Dose adjustments as a result of adverse reactions
the adverse reactions may need a dosage reduction or just a temporary interruption till the specificed adverse reaction get resolves.
Resume at 150 mg every 12 hours, or 100 mg every 12 hours, which can then be increased to the maximum dose (150 mg q12hr)
If 100 mg every 12 hours is not tolerated, the medication should be discontinued.
liver enzymes Elevated
AST/ALT levels more than 3 to 5 times the ULN without the signs for severe liver damage: Reduce or discontinue medication to 100 mg BID; after LFTs returns to normal, can reintroduce at 100 mg twice a day, which can be later increased to 150 mg twice a day.
AST/ALT levels more than 5times ULN or greater than 3times ULN with signs and symptoms for severe liver damage: Discontinue
Renal impairment
Mild and moderate: dosage adjustment is not necessary
ESRD or Severe (CrCl less than 30 mL/min): Not recommended Hepatic impairment
Mild (Child Pugh score A): 100 mg orally every 12hours
Moderate and severe (Child Pugh score B or C): usually Not recommended