Nailfold capillaroscopic findings in systemic sclerosis related lung fibrosis and in idiopathic lung fibrosis.

OBJECTIVE

Interstitial lung diseases (ILDs) are heterogeneous pathological conditions, which can be idiopathic or associated to other diseases, such as connective tissue diseases (CTD), especially systemic sclersosis (SSc). Both in primary and secondary forms of ILDs, fibroblastic and vascular anomalies are involved in the progressive structural destruction of lung parenchyma. Nailfold video-capillaroscopy (NFC) is a non-invasive diagnostic tool that permits detection of the main local microvascular alterations in SSc, which are an expression of the systemic vascular changes characteristic of this disease. The aim of this study is to record the range of capillaroscopic anomalies in patients with idiopathic pulmonary fibrosis (IPF) and to detect the main differences compared to patients with pulmonary fibrosis associated to SSc (SSc-PF).

METHODS

We performed NFC on 23 patients with PF secondary to systemic sclerosis (SSc-PF), 20 patients with IPF, and 22 patients with chronic obstructive pulmonary disease (COPD) and we analysed the differences in morphological capillaroscopic parameters between the study groups.

RESULTS

The main finding of this study was the detection of minor capillaroscopic alterations in patients affected by IPF compared to SSc-PF patients, the latter having typical capillary loop changes. Particularly, we found that in IPF patients capillary density was significantly reduced and neoangiogenic aspects was significantly greater compared to patients with COPD.

CONCLUSIONS

An altered nailfold capillaroscopic pattern in IPF, as observed in this study, seems to support the hypothesis that a systemic vascular dysfunction in these patients plays a role in the pathogenesis of the disease.