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Bronchoalveolar lavage and response to cyclophosphamide in scleroderma alveolitis

Bronchoalveolar lavage and response to cyclophosphamide in scleroderma alveolitis
Bronchoalveolar lavage and response to cyclophosphamide in scleroderma alveolitis
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.
0300-9742
155-159
Colaci, M.
33ea2948-7ed7-44df-8ab2-d9a72a74da5c
Sebastiani, M.
3b77bb59-9a57-48c6-8b66-2b6522ca415b
Giuggioli, D.
87d728fd-223d-4ee6-8e80-afc5b4e64661
Manfredi, A.
dfc1819f-67ec-4173-b93d-749412771cfc
Spagnolo, P.
dfc6600e-bbca-4678-abc4-6feb243d4fbb
Luppi, F.
4a386f93-c228-4b1a-bd52-fcccf9fcb880
Richeldi, L.
47177d9c-731a-49a1-9cc6-4ac8f6bbbf26
Ferri, C.
bb2d4006-5c0d-4a57-a606-09c02d3bf009
Colaci, M.
33ea2948-7ed7-44df-8ab2-d9a72a74da5c
Sebastiani, M.
3b77bb59-9a57-48c6-8b66-2b6522ca415b
Giuggioli, D.
87d728fd-223d-4ee6-8e80-afc5b4e64661
Manfredi, A.
dfc1819f-67ec-4173-b93d-749412771cfc
Spagnolo, P.
dfc6600e-bbca-4678-abc4-6feb243d4fbb
Luppi, F.
4a386f93-c228-4b1a-bd52-fcccf9fcb880
Richeldi, L.
47177d9c-731a-49a1-9cc6-4ac8f6bbbf26
Ferri, C.
bb2d4006-5c0d-4a57-a606-09c02d3bf009

Colaci, M., Sebastiani, M., Giuggioli, D., Manfredi, A., Spagnolo, P., Luppi, F., Richeldi, L. and Ferri, C. (2010) Bronchoalveolar lavage and response to cyclophosphamide in scleroderma alveolitis. Scandinavian Journal of Rheumatology, 39 (2), 155-159. (doi:10.3109/03009740903270581). (PMID:20059446)

Record type: Article

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.

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Published date: March 2010
Organisations: Clinical & Experimental Sciences

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Local EPrints ID: 369029
URI: http://eprints.soton.ac.uk/id/eprint/369029
ISSN: 0300-9742
PURE UUID: e2ecd973-7509-492f-8eec-77a4155dc6a8

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Date deposited: 23 Sep 2014 12:13
Last modified: 14 Mar 2024 17:57

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Contributors

Author: M. Colaci
Author: M. Sebastiani
Author: D. Giuggioli
Author: A. Manfredi
Author: P. Spagnolo
Author: F. Luppi
Author: L. Richeldi
Author: C. Ferri

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