Bronchoalveolar lavage and response to cyclophosphamide in scleroderma alveolitis

• Published in: Scandinavian Journal of Rheumatology Vol. 39; no. 2; pp. 155 - 159
• Main Authors: Colaci, M, Sebastiani, M, Giuggioli, D, Manfredi, A, Spagnolo, P, Luppi, F, Richeldi, L, Ferri, C
• Format: Journal Article
• Language: English
• Published: Colchester Informa UK Ltd 01-03-2010; Taylor & Francis; Informa
• Subjects: Adult; Antirheumatic Agents - therapeutic use; Biological and medical sciences; Bronchoalveolar Lavage; Cyclophosphamide - therapeutic use; Diseases of the osteoarticular system; Female; Humans; Lung - physiopathology; Male; Medical sciences; Middle Aged; Pulmonary Fibrosis - complications; Pulmonary Fibrosis - physiopathology; Pulmonary Fibrosis - therapy; Retrospective Studies; Sarcoidosis. Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. Vasculitis; Scleroderma, Systemic - complications; Scleroderma, Systemic - physiopathology; Scleroderma, Systemic - therapy; Severity of Illness Index; Treatment Outcome; Vital Capacity; Antineoplastic agent; Immunopathology; Connective tissue disease; Skin disease; Cyclophosphamide; Bronchoalveolar lavage; Systemic disease; Rheumatology; Autoimmune disease; Scleroderma
• ISSN: 0300-9742; 1502-7708; 1502-7732
• DOI: 10.3109/03009740903270581

Objectives: Systemic sclerosis (SSc) is characterized by abnormal fibrosis of the skin and internal organs, particularly the lungs. Recent reports have revealed a lack of correlation between bronchoalveolar lavage (BAL) variations and response to cyclophosphamide (CYC) in patients with scleroderma-related alveolitis. Our study aimed to evaluate whether the normalization of BAL cellularity correlates with long-term response to CYC.
Methods: We retrospectively studied 26 consecutive SSc patients with alveolitis diagnosed by BAL and treated with CYC therapy (cumulative dosage 26.5 ± 11.7 g; 21.1 ± 8.9 months of treatment). We evaluated high-resolution computed tomography (HRCT), forced vital capacity (FVC), and carbon monoxide diffusing capacity (DLCO) variations before and after CYC. Radiological and functional parameters were re-evaluated in 23 patients after 1-year follow-up.
Results: BAL cellularity normalized after CYC therapy in 12/26 (46.2%) patients (group 1), while it remained abnormal in 14/26 (53.8%) (group 2). FVC and DLCO of group 1 slightly increased after CYC (p = 0.014 and p = 0.07, respectively) and remained stable at follow-up, whereas in group 2 they did not change after CYC and at follow-up (p = not significant). Moreover, at the end of CYC, FVC and/or DLCO showed a clinical improvement/stabilization in all patients of group 1 versus 8/14 of group 2, while at the re-evaluation 1 year after completing CYC, 2/11 patients of group 1 worsened versus 5/12 of group 2. HRCT progression was observed in 1/11 of group 1 and 8/12 of group 2 (p = 0.009).
Conclusions: BAL fluid normalization after CYC therapy correlated with long-term response to treatment, contrary to what is observed in individuals with persistent alveolitis.