Centrilobular fibrosis (CLF): a distinct histological pattern in systemic sclerosis with interstitial lung disease (ILD) / Fibrose centrilobular (FCL): um padrão histológico pulmonar distinto em pacientes com esclerose sistêmica e doença intersticial pulmonar

AUTOR(ES)
DATA DE PUBLICAÇÃO

2007

RESUMO

Objectives: CLF is a new histological pattern of idiopathic ILD associated to esophageal reflux. We have investigated its presence in SSc with lung involvement. Methods: 28 SSc patients were submitted to open lung biopsy. The specimens were classified according to the new consensus classification of idiopathic interstitial pneumonia and to the diagnostic criteria for CLF. High Resolution Computer Tomography (HRCT), Pulmonary Function Tests (PFT), contrast esophagogram and/or upper digestive endoscopy were also performed. Main Results: In SSc, the NSIP (67.8%) and the centrilobular (75%) patterns were the most frequent and in the majority of the cases, they co-existed. All, except one patient with CLF had the characteristic bronchocentric distribution and this lesion was more extensive in those with isolated CLF (p=0.01). Likewise, the basophilic content was more frequent in patients with CLF and completely absent in NSIP group (p<0.001). The central distribution of lung involvement on HRCT was the most prevalent finding in patients with isolated CLF (57.14%) contrasting with the predominant peripheral pattern in the other groups (p=0.02). Moreover, a trend towards a patchy distribution on HRCT was observed for CLF group (85.71%) and CLF+NSIP group (71.43%) whereas 80% of the NSIP group had diffuse distribution (p=0.08). Esophageal abnormalities were almost a universal finding. Conclusions: This is the first report of centrilobular fibrosis in SSc patients with lung involvement. This new pattern has distinct histological and tomographic features. The identification of this subgroup of patients will certainly contribute for a more appropriate therapeutic approach.

ASSUNTO(S)

scleroderma systemic gastroesophageal reflux lung diseases interstitial/diagnosis pulmão/patologia refluxo gastroesofágico doenças pulmonares intersticiais/diagnóstico tomography x-ray computed lung/pathology tomografia computadorizada por raios x esclerose sistêmica

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