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Kombine pulmoner fibrozis ve amfizem (KPFA) sendromu egzersiz dispnesi, düşük karbon monoksit difüzyon kapasitesi (DLCO) ve korunmuş akciğer hacimlerinin yanı sıra üst loblarda amfizem, alt loblarda fibrozise ait radyolojik bulgularla karakterizedir. Sigara içicisi olan 67 ve 62 yaşlarında iki erkek hasta efor dispnesi ve nonprodüktif öksürük yakınmalarıyla kliniğimize başvurdu. Bu olgularda şiddetli desatürasyona bağlı düşük efor kapasitesi, bozulmuş DLCO ve sendrom ile uyumlu radyolojik bulgularının varlığı nedeniyle KPFA sendromu tanısı kondu. Olgularin birinde ayrica fibrotik akciğer alanı üzerinde gelişen evre 4 pulmoner adenokarsinom da saptandı. Antifibrotik (pirfenidone/nintedanib) tedavi almadan, palyatif radyoterapi ve kemoterapi alan olgumuz ciddi solunum yetmezliği nedeniyle 14 ay sonunda kaybedildi. Diğer olgumuza akciğer trasplantasyonu önerisi yapıldı. Başlanan pirfenidon tedavisi ile fonksiyonel ve radyolojik stabil olan hasta halen poliklinik takibimiz altındadır. Sonuç olarak, sigara öyküsü ile birlikte mikst tipte solunum fonksiyon testi bozukluğu, şiddetli gaz değişim bozukluğu ve radyolojik olarak amfizem ve fibrozis varlığı tespit edilen hastalarda KPFA tanısı akılda tutulmalıdır. Hastalar akciğer kanseri gelişimi açısından takip edilmeli ve uygun palyatif tedavilerin yanisira mutlaka akciğer transplantasyonu önerilmelidir.
Combined lung fibrosis and emphysema (CPFA) syndrome is characterized by exertional dyspnea, low carbon monoxide diffusion capacity (DLCO), preserved lung volumes, as well as radiological findings of emphysema in the upper lobes and fibrosis in the lower lobes. Two male smoker patients, aged 67 and 62, admitted to our clinic with complaints of dyspnea on effort and nonproductive cough. These cases were diagnosed as CPFA syndrome because they showed low effort capacities due to severe desaturation, impaired DLCO and the presence of radiological findings compatible with the syndrome. Stage 4 pulmonary adenocarcinoma which was developed on the fibrotic lung area was also detected in one of the cases. He received palliative radiotherapy and 4 cycles of chemotherapy, but not antifibrotic treatment (pirfenidone/nintedanib) for pulmonary fibrosis, and died after 14 months due to severe respiratory failure. In the other case, lung transplantation has been proposed. With the onset of pirfenidone therapy, his pulmonary functions and radiological findings are stable. He is still under our outpatient follow-up. In conclusion, the diagnosis of CPFA should be kept in mind in smoker patients with mixed-type pulmonary function test disorder, level gas exchange disorder, and radiological evidence of emphysema and fibrosis. Patients should be followed up for the development of lung cancer, and lung transplantation should be recommended in addition to appropriate palliative care.
Combined pulmonary fibrosis and emphysema (CPFA) syndrome is characterized by exertional dyspnea, low carbon monoxide diffusion capacity (DLCO), preserved lung volumes, as well as radiological findings of emphysema in the upper lobes and fibrosis in the lower lobes. Two male smoker patients, aged 67 and 62, admitted to our clinic with complaints of dyspnea on effort and nonproductive cough. These cases were diagnosed as CPFA syndrome because they showed low effort capacities due to severe desaturation, impaired DLCO and presence of radiological findings compatible with the syndrome. Stage 4 pulmonary adenocarcinoma which was developed on fibrotic lung area was also detected in one of the cases. He received palliative radiotherapy and 4 cycles of chemotherapy, but not antifibrotic treatment (pirfenidone/nintedanib) for pulmonary fibrosis, and died after 14 months due to severe respiratory failure. In the other case, lung transplantation has been proposed. With the onset of pirfenidone therapy, his pulmonary functions and radiological findings are stable. He is still under our outpatient follow-up. In conclusion, the diagnosis of CPFA should be kept in mind in smoker patients with mixed-type pulmonary function test impairment, severe gas exchange disorder, and radiologic evidence of emphysema and fibrosis. Patients should be followed up for development of lung cancer, and lung transplantation should be recommended in addition to appropriate palliative care.
Alan : Sağlık Bilimleri
Dergi Türü : Uluslararası
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