Quantitative videocapillaroscopy correlates with functional respiratory parameters: a clue for vasculopathy as a pathogenic mechanism for lung injury in systemic sclerosis.

BACKGROUND: To determine whether lung involvement is related to microvascular perturbations, nailfold videocapillaroscopy (NVC) was performed in patients with systemic sclerosis (SSc). METHODS: A cross-sectional study was consecutively accomplished in 152 SSc patients. NVC, a pulmonary function test and echocardiography were undergone within a 3-month period. Finally, 134 patients with at least eight NVC (200× magnification) images were selected for quantitative and qualitative examinations. RESULTS: Patients with interstitial lung disease presented lower median capillary density (4.86/mm vs 5.88/mm, p = 0.005) and higher median of neoangiogenesis (0.56/mm vs 0.31/mm, p = 0.005). A higher quantity of neoangiogenesis capillaries was found in patients with pulmonary arterial hypertension (0.70/mm vs 0.33/mm, p = 0.008). Multivariate linear regression analysis established a correlation between neoangiogenesis and decreased forced vital capacity (FVC) (p < 0.001): for each capillary with neoangiogenesis visualized on average per 1 mm, FVC was 7.3% reduced. In qualitative NVC, a late pattern as defined by Cutolo was also associated with lower FVC (p = 0.018). The number of giant capillaries was associated with reduced diffusion capacity of the lung for carbon monoxide (DLCO) (p = 0.016); for each giant capillary per 1 mm, DLCO was 11.8% diminished. CONCLUSIONS: A good correlation was observed between distinctive quantitative and qualitative NVC features with lung functional parameters such as FVC and DLCO. It is suggested that vasculopathy could play a role in SSc lung involvement.

Good outcome of interstitial lung disease in patients with scleroderma associated to anti-PM/Scl antibody.

OBJECTIVE: The objective of this article was to establish the clinical course of interstitial lung disease (ILD) in scleroderma related to the presence of anti-PM/Scl antibody compared with anti-Scl-70 in a Spanish cohort. Furthermore, no study has thoroughly investigated the outcome of pulmonary function test in the first group of patients. METHODS: A total of 63 Spanish patients with scleroderma and ILD were selected in a retrospective observational study. Among them, 14 were positive for anti-PM/Scl antibodies and 49 for anti-Scl-70. Clinical assessments, including pulmonary function test, were collected. Variations equal or greater than 10% in forced vital capacity (FVC) were considered significant. Progression-free survival of disease was defined as the period of stable illness since pulmonary fibrosis diagnosis. RESULTS: Anti-Scl-70 patients had a higher frequency of diffuse SSc subset, peripheral vasculopathy, and gastrointestinal involvement. Inflammatory myopathy was associated to anti-PM/Scl antibody. Anti-PM/Scl patients presented more improvement in FVC during follow-up, 30.8% compared to a 7.1% in Scl-70 group (P = 0.04), with less worsening of this parameter (15.4% vs 52.4% in Scl-70 patients, P = 0.01), and secondary less frequency of severe restrictive pattern (FVC < 50%) (7.7% compared to 42.9% in the other group, P = 0.02). Regarding treatment, more anticalcineurinics were used in anti-PM/Scl patients, while cyclophosphamide and mycophenolate were mainly used in anti-Scl-70 patients. The progression-free survival of disease was higher in anti-PM/Scl patients, with 76% at 10 years from diagnosis of ILD against a 29% in the Scl-70 group. CONCLUSIONS: Several features and prognosis of ILD in SSc may be modified depending on the identified immunological profile.