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.

Bronchoalveolar lavage and response to cyclophosphamide in scleroderma alveolitis / Colaci, Michele; Sebastiani, Marco; Giuggioli, D.; Manfredi, Andreina Teresa; Spagnolo, Paolo; Luppi, Fabrizio; Richeldi, Luca; Ferri, Clodoveo. - In: SCANDINAVIAN JOURNAL OF RHEUMATOLOGY. - ISSN 0300-9742. - STAMPA. - 39:(2010), pp. 155-159. [10.3109/03009740903270581]

Bronchoalveolar lavage and response to cyclophosphamide in scleroderma alveolitis.

COLACI, Michele;SEBASTIANI, Marco;D. Giuggioli;MANFREDI, Andreina Teresa;SPAGNOLO, Paolo;LUPPI, Fabrizio;RICHELDI, Luca;FERRI, Clodoveo
2010

Abstract

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.
39
155
159
Bronchoalveolar lavage and response to cyclophosphamide in scleroderma alveolitis / Colaci, Michele; Sebastiani, Marco; Giuggioli, D.; Manfredi, Andreina Teresa; Spagnolo, Paolo; Luppi, Fabrizio; Richeldi, Luca; Ferri, Clodoveo. - In: SCANDINAVIAN JOURNAL OF RHEUMATOLOGY. - ISSN 0300-9742. - STAMPA. - 39:(2010), pp. 155-159. [10.3109/03009740903270581]
Colaci, Michele; Sebastiani, Marco; Giuggioli, D.; Manfredi, Andreina Teresa; Spagnolo, Paolo; Luppi, Fabrizio; Richeldi, Luca; Ferri, Clodoveo
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11380/646345
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