Idiopathic Pulmonary Fibrosis (IPF) Workup: Laboratory Studies, Imaging Studies, Other Tests
Drug-Induced Interstitial Lung Disease Linked To Cancer And CV Drugs
Cancer and cardiovascular (CV) drugs may be linked to a large proportion of drug-induced interstitial lung disease (ILD) and other diffuse lung disease cases, according to findings of a French study reported in the European Respiratory Journal.
The incidence of drug-induced lung diseases is low and typically becomes known through adverse drug reaction (ADR) reports made after drugs have been marketed. Investigators in France conducted a descriptive and retrospective study to identify the epidemiology of suspected medications and clinical presentations of drug-induced diffuse lung diseases over a 37-year period using the French Pharmacovigilance Database (FPVD).
ADRs in the FPVD are coded using the Medical Dictionary for Regulatory Activities (MedDRA) classification. The current analysis included adult cases involving ADRs registered in the FPVD from January 1, 1985, to April 1, 2022, coded with the MedDRA High Level Group Term "lower respiratory tract disorders (excluding obstruction and infection)."
A total of 7234 cases were included for analysis, comprising 7658 ADRs and 13,059 suspected drugs, with 13,836 sets of drug-ADR combinations. About 54.4% of patients were male, the median age was 69 years, and the proportion of serious cases was 96.7%.
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Our nationwide study confirms the importance, frequency, and severity of drug-induced diffuse lung diseases. It is essential that it be considered a potential diagnosis so that the implicated medication can be discontinued as quickly and safely as possible.
Amiodarone was the most suspected drug until 2020. From 2021 until the end of the study, the most commonly suspected agents were COVID-19 vaccines, followed by amiodarone and immune checkpoint inhibitors (ICIs).
Anti-cancer/anti-neoplastic drugs (n=4024) were the leading pharmaceutical class associated with ADRs, representing 31.2% of all suspected drugs and found in 2790 cases (38.6%). In this group, chemotherapy agents such as bleomycin and gemcitabine, as well as ICIs were the most frequently reported drugs.
Cardiovascular drugs were the second most common drug class associated with ADRs (n=3751), accounting for 29.1% of all suspected drugs, occurring in 2506 cases (34.6%). Amiodarone was the most common drug in this class and the most frequently reported drug (1290 reports, 10.0% of all suspected drugs) and occurred in 17.8% of all reported cases. Methotrexate had the second highest number of reports (n=401), representing 3.1% of all suspected drugs.
The 7658 coded ADRs were related to 41 MedDRA preferred terms (PTs) for different clinical and imaging patterns that fell under 3 MEDdra High Level Terms: parenchymal lung disorders (ie, ILDs and diffuse lung diseases) not elsewhere classified (69.0%), pulmonary edema (21.5%), and lower respiratory tract inflammatory and immunologic conditions (9.4%). The most common PTs associated with ADRs were "ILD" (51.0%), "pulmonary edema" (acute/non-acute) (15.6%) and "pulmonary fibrosis" (10.5%).
Of the 7234 cases, 34.9% of patients recovered without sequelae, 17.7% were recovering at notification, 22.5% had not recovered at the most recent follow-up, and 4.7% recovered with sequelae. A total of 962 patients (13.3%) died, and the outcome of the ADR was not specified in 6.9%.
Amiodarone was the most frequently found drug in cases with a fatal outcome: 201 cases were fatal, including 39 fatal cases considered as "at least plausible."
Limitations include under-reporting bias and notoriety bias. In addition, the research was conducted during a long period in which the coding of drugs changed.
"Our nationwide study confirms the importance, frequency, and severity of drug-induced diffuse lung diseases," the study authors stated. "It is essential that it be considered a potential diagnosis so that the implicated medication can be discontinued as quickly and safely as possible."
Pulmonary Fibrosis And Interstitial Lung Disease
MissionThe Program for Pulmonary Fibrosis and Interstitial Lung Disease's mission is to provide state of the art evaluation and treatment for patients with interstitial lung diseases (ILD). The University of Alabama at Birmingham ILD program is designated as a Pulmonary Fibrosis (PF) Foundation Care Center network site.
ObjectivesPulmonary fibrosis or interstitial lung diseases are chronic, progressive, lung disease characterized by disabling shortness of breath, declining exercise capacity and extensive formation of scar tissue (fibrosis) in the lung. There are several different types/causes of pulmonary fibrosis and are difficult to diagnose and treat. This program exists to better understand such illnesses, train physicians to more completely treat such illnesses, and to drive the creation of superior treatment options.
Our entire team at the ILD clinic is dedicated to improving the lives of our patients with ILD through patient care, education and research. Our multidisciplinary team combines the skills of pulmonologists, thoracic radiologists and thoracic pathologists, all of whom have specific expertise in ILD and meet weekly to discuss new patients evaluated in ILD clinic. The team also works closely with subspecialists, including the rheumatologists and lung transplant team.
Pulmonary Fibrosis/Interstitial Lung Disease Program Contact Information
UAB Interstitial Lung Disease ProgramTHT 422, 1900 University BoulevardBirmingham, AL 35294-0006Phone: (205) 934-7557Fax: (205) 934-6229
Contacting the Program for Pulmonary Fibrosis and Interstitial Lung DiseaseTo refer a patient to this program, please contact our office administrator at (205) 934-7557 from 8 AM-5 PM, CT, Monday-Friday. In order to expedite the scheduling and the evaluation process, please forward the following documents to our office as soon as possible:
1. Last clinic note or referral letter2. Pulmonary function tests (PFTs)3. Chest X-rays and CT scans - upload films to a CD and attach reports4. Lung biopsy slides and reports, if available.
After the appointment is scheduled, your patient will receive a package containing a schedule of tests and appointments, along with the "UAB ILD Program New Patient Questionnaire," maps, and information on lodging in the Kirklin Clinic area. After the clinic visit and once all data has been reviewed, including any prior biopsies, you will receive either a letter from one of our physicians or a copy the patient's clinic visit note.
Clinical TrialsThe UAB ILD Center offers clinical trial enrollment for patients with idiopathic pulmonary fibrosis and other forms of diffuse parenchymal lung disease as part of a multidisciplinary and integrated approach to treatment. Please call Ms. Tonja Meadows at 205- 934-6475 for more information.
Modelling Autoimmune Lung Disease
Cite this articleMinton, K. Modelling autoimmune lung disease. Nat Rev Immunol 10, 4 (2010). Https://doi.Org/10.1038/nri2694
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