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The purpose of this study was to determine which high-resolution computed tomography (HRCT) features in patients with cystic fibrosis are most strongly associated with functional impairment as expressed by forced expiratory volume in one second (FEV1). Forty-seven patients with cystic fibrosis underwent chest HRCT and had pulmonary function tests. The HRCT examinations were evaluated for 11 features scored using a modification of Bhalla system and FEV1 was recorded as percentage of the predicted value. Univariate and multivariate correlations between HRCT scores and FEV1 were performed. The most common HRCT feature was bronchiectasis (98%) followed by atelectasis-consolidation (81%), bronchial wall thickening (77%), tree-in-bud sign (74%), mucous plugging (72%) and mosaic perfusion pattern (47%). On univariate analysis the following features correlated strongly with FEV1: bronchial wall thickening ( p

OBJECTIVE: To evaluate the safety and efficacy of monthly intravenous pulses of cyclophosphamide (CP) in combination with low or high doses of prednisolone in patients with systemic sclerosis (SSc) related interstitial lung disease (ILD) with FVC < 70% of predicted. METHODS: An open label, non-parallel arm study, performed in the rheumatology outpatient clinic of a university hospital. Twenty-eight patients with SSc related ILD were evaluated. Endpoint evaluations included the evolution of high resolution computed tomography, pulmonary function tests, skin involvement and dyspnea over 12 months. Patients were treated with monthly IV CP in combination with prednisolone at low (< 10 mg/day; n = 12) or high doses (1 mg/kg/day for 4 weeks, then reducing the prednisolone by 5 mg/day on alternating days each 2 weeks; n = 16). RESULTS: In the low dose steroid group, no improvement was seen for any endpoint at 6 and 12 months of followup. In the high dose steroid group, at 12 months there was significant improvement in the percentage of "ground glass" parenchymal lung involvement (-5.7%; p = 0.003), as well as in the percentage of predicted FVC (12.4%; p < 0.001), the percentage of predicted DLCO (7.3%; p = 0.029), the percentage of skin involvement (-5.4%; p = 0.01), and the severity of dyspnea (p = 0.012). Substantial improvement was seen as early as 6 months. One patient (low dose group) died from ILD. CONCLUSION: A combination of IV pulse CP with high doses of prednisolone shows promising efficacy in improving the clinical, physiological, and radiological evolution of SSc related ILD with reversal of the underlying alveolitis. J Rheumatol

Thirty-six patients with primary Sjogren's syndrome were evaluated for respiratory manifestations using clinical, roentgenologic, functional, and in five cases, histologic criteria. Twenty seven patients (75 percent) had evidence of respiratory involvement, usually occurring early in the course of the disease. Diffuse interstitial lung disease was most common (25 percent) followed by small airways disease (22 percent), desiccation of upper respiratory tract (17 percent) and large airways obstruction (8 percent). There was no patient with pleural effusion. Transbronchial lung biopsy of five patients studied revealed interstitial pathology ranging from dense lymphocytic infiltrates to interstitial fibrosis. Roentgenologic evaluation was often suggestive of interstitial lung disease but did not correlate with functional or histologic findings. Chest