Bronchoalveolar lavage and response to cyclophosphamide in scleroderma alveolitis.
OBJECTIVE
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.
Colaci
M
M
Rheumatology Unit, University of Modena and Reggio Emilia Medical School, Modena Polyclinic, Modena, Italy.
Sebastiani
M
M
Giuggioli
D
D
Manfredi
A
A
Spagnolo
P
P
Luppi
F
F
Richeldi
L
L
Ferri
Clodoveo
C
D000328:Adult; D018501:Antirheumatic Agents / Q000627:therapeutic use; D018893:Bronchoalveolar Lavage; D003520:Cyclophosphamide / Q000627:therapeutic use*; D005260:Female; D006801:Humans; D008168:Lung / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D011658:Pulmonary Fibrosis / Q000150:complications / Q000503:physiopathology / Q000628:therapy*; D012189:Retrospective Studies; D012595:Scleroderma, Systemic / Q000150:complications / Q000503:physiopathology / Q000628:therapy*; D012720:Severity of Illness Index; D016896:Treatment Outcome; D014797:Vital Capacity
D018501:Antirheumatic Agents; D003520:Cyclophosphamide