Objective: Idiopathic pulmonary fibrosis (IPF) is a progressive, fibrotic, and fatal lung disease associated with the inevitable loss of lung function. Pirfenidone, which has antifibrotic properties and has been used orally in recent years, slows down the progression of the disease and increases survival rates. However, photosensitive skin rash caused by absorbing ultraviolet rays is the most frequently encountered adverse effect in clinical practice.
Methods: Thirteen patients who were treated for IPF in the department of chest diseases between September 2018 and January 2022, used pirfenidone, and applied to the dermatology outpatient clinic due to rash were retrospectively examined. During this period, the number of patients receiving pirfenidone for IPF in chest diseases was fifty-six.
Results: In dermatological examination, scaly plaques on an erythematous background were common in seven patients, whereas lichenoid papules and plaques were dominant in six. In the histopathological evaluation of biopsies taken from the lesional skin area, the findings were consistent with superficial perivascular dermatitis in two, psoriasiform dermatitis in five, and lichenoid reaction pattern in six patients. When photosensitivity reactions occurred, pirfenidone treatment was continued in eleven patients at a reduced dose, and only two patients discontinued pirfenidone and switched to nintedanib therapy.
Conclusion: We aimed to show that photosensitivity reactions can be managed in the majority of patients without discontinuing pirfenidone, which plays a vital role in the treatment of IPF symptom control and survival by reducing the dose, using sun protection, and taking additional protective measures, and to provide further insight to clinicians in this regard.