Portal de Periódicos da CAPES

Combined pulmonary fibrosis and emphysema syndrome in systemic sclerosis

2012; Wiley; Volume: 15; Issue: 5 Linguagem: Inglês

10.1111/j.1756-185x.2012.01747.x

1756-185X

C. Lubatti, S. Zeni, Francesca Ingegnoli,

Medical Imaging and Pathology Studies

Dear Editor, Combined pulmonary fibrosis and emphysema (CPFE) is a described syndrome observed in patients affected by connective tissue diseases (CTDs) and by smoking-induced chronic lung disease.1-3 We have observed two patients with systemic sclerosis (SSc) and pulmonary alterations consistent with CPFE. The first was a 67-year-old man with limited cutaneuos SSc (lcSSc) who had been a heavy smoker for more than 30 years. High-resolution computed tomography (HRCT) revealed diffuse subpleural linear thickening and centrilobular emphysema with large bullae in the apex, bronchiectasis and honeycomb lesions at the base. There were no other signs of disease activity. Five years later, he suddenly developed symmetric erosive polyarthritis, worsening HRCT lesions (subpleural, apical and intraparenchymal emphysematous bullae, diffuse honeycombing, multiple mediastinic lymph nodes, and subpleural interstitial thickening: Fig. 1a), reduced forced vital capacity (76%) and diffusion capacity of carbon monoxide (DLCO: 60%), and high systolic pulmonary artery pressure (sPAP) as revealed by Doppler echocardiography (36 mmHg). The second patient was a 70-year-old woman who rapidly developed diffuse cutaneous SSc (dcSSc) with neurological, esophageal and pulmonary involvement. Pulmonary function tests showed severely reduced DLCO (46%), and HRCT revealed diffuse interstitial reticulo-nodular alterations, multiple cysts and small mediastinic lymph nodes (Fig. 1b). Smoking, exposure to chemical substances, infection and rare neoplasms such as Langherhans cell histiocytosis were excluded. As they were found in our SSc patients at the time of diagnosis, interstitial fibrosis with diffuse pulmonary cystic lesions may be a not so uncommon manifestation of interstitial lung disease4 that requires specific investigations in order to exclude infections and cancer.5, 6 Our cases strengthen the hypothesis of Cottin et al.1 that CTDs may be a co-factor in the pathogenesis of CPFE. The presence of CPFE in SSc patients may increase the risk of severe visceral complications such as pulmonary hypertension and reduced alveolar diffusion capacity.1 Furthermore, in the absence of other signs of disease activity, pulmonary damage due to tobacco smoking may hamper the recognition of lung involvement due to SSc at the time of diagnosis.