Antifibrotics in Non-Idiopathic Pulmonary Fibrosis Interstitial Lung Diseases
DOI:
https://doi.org/10.58931/crt.2025.1318Abstract
Case 1
A 73-year-old male with a history of seronegative rheumatoid arthritis associated interstitial lung disease (RA-ILD), showing a probable usual interstitial pneumonia pattern of fibrosis, was seen for follow up. He had initially been started on mycophenolate for treatment of his ILD and remained stable on this treatment for a period; however, he later experienced slowly worsening dyspnea and cough over time. His pulmonary function tests (PFTs) demonstrated a 9.5% relative and 6% absolute decline in forced vital capacity (FVC) and a 12% relative and 10% absolute decline in diffusion capacity for carbon monoxide (DLCO) over the past 20 months. A computed tomography (CT) scan of the thorax showed some new changes of mild honeycombing in the right lower lobe (Figure 1).
Case 2
A 50-year-old male developed dyspnea and cough after a COVID-19 infection. During the COVID-19 infection, his symptoms were mild and did not require treatment or hospitalization. A CT scan of the thorax demonstrated evidence of a fibrotic non-specific interstitial pneumonia pattern. Subsequent evaluation for new-onset ILD, included a surgical lung biopsy, which demonstrated organizing pneumonia with cicatricial changes.
The patient received a course of prednisone, which led to a significant improvement in his symptoms but no improvement in imaging or PFTs. After tapering off prednisone, he required another course due to re-emergence of symptoms. However, retreatment yielded no improvement in his symptoms, imaging, or PFTs. The patient’s case, imaging, and pathology were reviewed in a multidisciplinary discussion, resulting in a diagnosis of organizing pneumonia evolving toward a more fibrotic phenotype. In addition to his worsening symptoms, imaging revealed increased reticulation, and assessments showed a significant decline in his FVC and DLCO over time.
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