Een 75-jarige vrouw met inspanningsgebonden kortademigheid en desaturatie

A 75-year-old woman with a history of smoking was admitted to the hospital with dyspnea and desaturation on exertion. The clinical examination revealed bilateral crackles. The arterial blood gas revealed hypoxemia with an abnormally increased alveolar-arterial gradient. Pulmonary function testing demonstrated preserved lung volumes and a markedly decreased diffusing capacity. The high-resolution CT scan (HRCT) of the lung showed both emphysema and fibrosis. The emphysema occurred predominantly in both upper lobes, while fibrosis mostly occurs in both lower lobes. Pulmonary hypertension was disco­vered during the echocardiography. Based on these findings, the patient was diagnosed with combined pulmonary fibrosis and emphysema (CPFE). She was treated with moderate doses of oral steroids and long-term oxygen therapy. She died two months after admission due to respiratory insufficiency. CPFE is a rare cause of dyspnea. In 2005, it was described as a distinct clinical syndrome. It can occur isolated or in combination with a systemic disease. The condition is usually diagnosed with an HRCT with or without the addition of pathological findings on a lung biopsy. CPFE is often complicated by pulmonary hypertension, which is prognostically unfavorable. The pathogenesis is unknown but smoking remains the most important etiological factor. In addition to smoking cessation and supportive measures, the treatment possibilities are limited. It is important to include CPFE into the differential diagnosis of dyspnea on exertion because it is a syndrome with different therapeutic and prognostic implications when compared to fibrosis or chronic obstructive pulmonary disease (COPD)/emphysema alone