Idiopathic Pulmonary Fibrosis (IPF) is an interstitial lung disease of unknown etiology
expressed by progressive fibrosis and that affects mainly smokers of advanced age. Given the
lack of national epidemiological studies as a result of its complex diagnosis, it is important to
develop studies about the profile of the disease so its diagnostic approach is disseminated. The
objective of this study was to describe the clinical, epidemiological, tomographic,
histopathological and therapeutic profile of patients diagnosed with idiopathic pulmonary
fibrosis treated at the Medical Clinic for Rare Pulmonary Diseases of the João de Barros Barreto
University Hospital (HUJBB). This is a descriptive, observational, cross-sectional,
retrospective and quali-quantitative study based in 12 patients diagnosed with IPF, defined
according to the Fleischner Society's 2018 criteria, treated at HUJBB between 2017 and 2021.
The data collection was performed via active search of paper-based and electronic medical
records available in the institution's platform. It was found that 58.3% of the patients were male
and the mean age of the sample was 73.8 ±8.2. There was a predominance of mixed race
(91.7%), an equivalence between individuals born in the capital and the upstate (both 41.7%)
and a majority of individuals with bachelor’s degree (33.3%). Clinical evaluation data showed
as the most frequent findings: inspiratory fine crackles, cough, expectoration and coarse
crackles. As for the modified dyspnea scale, the largest number of patients were classified in
category 3. The mean peripheral oxygen saturation in room air was 94.6% ±2.9, while the one
of patients under supplemental oxygen was 87.5% ±4.9. The most observed comorbid condition
was systemic arterial hypertension (50%); smoking history was positive in 66.7% of patients,
with a mean smoking history in pack-years of 36.8 ±33.3; and history of acute exacerbations
and hospitalizations were present in the medical records of 33.3% of the patients. The most
frequent UIP pattern on computed tomography was typical UIP (50%), being associated with a
greater number of clinical manifestations and greater amount of smokers in this sample. On
biopsy, the definitive UIP pattern predominated (75%). The restrictive ventilatory pattern was
the most frequent; the mean forced vital capacity was 75.36% ±20.2; distance walked in six
minutes was 319 meters ±15.6 and oxygen saturation at the end of the exam was 94% ±2.8. All
patients had indications for antifibrotic use, most of them of nintedanib (66.7%), also being the
drug most related to adverse effects. The majority (66.7%) of the patients had a record of
difficulty in obtaining medication and only 16.7% reported having undergone physical therapy.
It was concluded that, in general, the profile of patients diagnosed with IPF included in the study corroborated findings in the literature and highlighted the need for studies to better
characterize the disease in Brazil and in the state of Pará.
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