Objective: This study was performed to confirm the efficacy of a 6-month therapy with a formulation of N-acetylcysteine (NAC; 600 mg/day p.o.) on frequency and severity of exacerbations in patients suffering from chronic obstructive pulmonary disease (COPD). Methods: One hundred sixty-nine patients attending five Italian centres were recruited in an open, randomized, controlled study. The patients were randomly allocated to standard therapy plus NAC 600 mg once a day or standard therapy alone over a 6-month period. At baseline, medical history was evaluated, and physical examination was performed; occurrence and severity of exacerbations and side effects of NAC were analyzed after 3 and 6 months. Results: The results showed a decreased number of exacerbations (by 41%) in the group of patients treated with NAC and standard treatment: 46 patients had at least one exacerbation as compared with 63 patients of the group treated with standard therapy alone. Also the number of the patients with two or more exacerbations was lower in the NAC group (26%) than in the standard-therapy group (49%). The number of sick days was less (82) in the NAC group as compared with the standard-therapy group (155). There was a small but significant improvement in FEV₁ and MEF₅₀ in the NAC group. NAC once a day was well tolerated. There were no differences in the number of side effects reported in both groups. Conclusions: These data confirm results of previous studies which reported a reduction in the number of exacerbations in patients having moderate to severe COPD treated with the antioxidant NAC. Further, the once-daily formulation is well tolerated and is likely to improve patient compliance with the prescribed regimen.

Pneumonitis is emerging as one of the most unpredictable and potentially serious, adverse effects of treatment with MTX. Its prevalence in rheumatoid arthritis (RA) has been estimated from several retrospective and prospective studies to range from 0.3% to 18%. On the other hand, MTX-induced pneumonitis seems to be very rare in psoriatic arthritis (PsA). Our review of 194 RA patients and 38 PsA patients receiving MTX has identified four RA patients and one PsA patient with MTX-induced pneumonitis, giving a prevalence of 2.1% and 0.03%, respectively. Diagnosis was suggested by clinical history and radiographic findings, but the bronchoalveolar lavage plays an important role both in excluding infectious agents and in providing information for understanding the pathogenesis of lung injury. The presence of a lymphocyte alveolitis with a predominance of CD4+ T cells in 3 RA patients and CD8+ T cells with a concomitant increase in neutrophils in another case suggests that immunologically mediated reactions may be one damage mechanism in MTX-induced pneumonitis. Although risk factors for MTX-induced pulmonary toxicity are poorly understood, the presence in 3 out of 5 of our patients of pre-existing lung disease, represented by diffuse interstitial changes on chest X-ray, and mild bronchial asthma in two RA patients and by pulmonary silicosis in the patient with PsA may account for a predisposition to the development of MTX pneumonitis.

Eighteen non-smoking women suffering from primary Sjögren's syndrome (pSS) with previously documented alveolitis were re-examined, clinically and by pulmonary function tests (PFT), bronchoalveolar lavage (BAL), chest X-ray and high-resolution computed tomography (HRCT) after a 2 yr follow-up period. Longitudinal evaluation revealed unchanged PFT. The final BAL study showed a normal differential count in six of 14 patients with initial lymphocyte alveolitis, and a persistent alveolar lymphocytosis in the remaining eight patients, associated with an increased percentage of neutrophils in one of them. In four patients with initial mixed alveolitis, the BAL cell profile was unchanged 2 yr later. Five of 18 patients (28%) had abnormal HRCT, represented by isolated septal/subpleural lines in three patients, ground-glass opacities with irregular pleural margins in one patient, and ground-glass opacities associated with septal/subpleural lines in another. All these patients had abnormal BAL results with an increased proportion of both neutrophils and lymphocytes. The presence of alveolar neutrophils was associated with a significantly (P=0.005) greater mean rate of reduction of carbon monoxide diffusing capacity (DLCO) -- more than four times the normal rate of loss of DLCO. Chest X-ray, repeated at the end of the 2 yr follow-up period, showed parenchymal abnormalities in only one patient who had evidence of fibrosis on HRCT. This study provides evidence that lung involvement is not an uncommon extraglandular manifestation of pSS and that a BAL neutrophilia may play an important role in the pathogenesis of pulmonary disease in this autoimmune disorder.

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Background Interstitial pulmonary fibrosis (IPF) is a frequent manifestation in patients with systemic sclerosis (SSc). The severity of lung involvement may vary considerably depending on the underlying disease and it can be often the cause of death of these patients (1). Currently chest high resolution computed tomography (HRCT) is considered the “gold-standard” for the diagnosis and disease activity monitoring of IPF (2). The recent availability of open sources DICOM viewer softwares such as OsiriX (3) opens up a new research area for the rheumatologist to assess the IPF. Objectives To investigate the correlation between a open source (OsiriX) software and HRCT findings in the assessment of IPF in patients with SSc. Methods Sixty two patients with diagnosis of SSc were enrolled. All patients showed an IPF at HRCT which has been interpreted by two expert radiologist. Spirometric pulmonary function tests and DLco were also performed. Both radiologist indipendently performed an analysis of the HRCT findings of all patients in order to score the IPF. Successively they reached an agreement by a discussion to obtain a consensus for a single score. Afterwards, an experienced OsiriX user rheumatologist, independently and blinded to radiologists’ scoring, quantified the overall extent of IPF in all patients using Osirix, which is a multidimensional DICOM viewer software designed to display, interpret and analyze large sets of multidimensional and multimodality images with many tools that allow the user to perform post-processing algorithms. These results were compared with those obtained by the lectures of the radiologists. Results A total of 62 SSc patients were assessed. A highly significant correlation between radiologists’ scoring and OsiriX HRCT quantitative assessment of IPF was found (p≤0.0001). In particular there was a significant correlation between quantitative evaluation and visual scoring stratified according to overall extension and fibrosis (both p≤0.0001). No correlation was found between quantitative assessment and DLco values (p=0.73). Conclusions Our results provide a new working hypothesis in favour of the utility of OsiriX in perfoming an objective IPF evaluation which could be very helpful in assessing and monitoring SSc patients. References Tyndall et al. Causes and risk factors for death in systemic sclerosis: a study from the EULAR Scleroderma Trials and Research (EUSTAR) database. Ann Rheum Dis 2010; 69 (10):1809–15. Wells. High-resolution computed tomography and scleroderma lung disease. Rheumatology (Oxford) 2008; 47 (Suppl 5):v59-61. Rosset et al. OsiriX: an open-source software for navigating in multidimensional DICOM images. J Digit Imaging 2004;17 (3): 205-16. Disclosure of Interest None Declared