title: Long-term N-acetylcysteine therapy in systemic sclerosis interstitial lung disease: a retrospective study.
abstract: Systemic sclerosis (SSc) is associated with interstitial lung diseases. The primary endpoints of this study were changes between baseline and month 24 in single-breath carbon monoxide diffusing capacity (DLco). The secondary endpoints were: vital capacity (VC), forced expired volume in 1 sec (FEV1), total lung capacity (TLC), scores of high resolution computed tomography (HRCT) of the chest, number of adverse effects. In this study, we retrospectively investigated data from SSc patients who had undergone therapy with high-dose intravenous N-acetylcysteine (NAC) at a dosage of 15 mg/Kg/h for 5 consecutive hours every 14 days. After NAC therapy median values of DLco (69.5 vs 77.7%), VC (99 vs 101.3%) and TLC (93 vs 98.3%) significantly increased. We did not observe any significant changes from baseline in FEV1 value and HRTC score. The improvement in lung function was more evident in SSc patients without radiological signs of pulmonary fibrosis than in patients with pulmonary fibrosis. In SSc patients with mild-moderate pulmonary fibrosis intravenous NAC administration slows the rate of deterioration of DLco, VC and TLC. In conclusion, this retrospective study demonstrates that long-term therapy with intravenous NAC ameliorates pulmonary function tests in SSc patients.
authors: Sapienza University of Rome, Department of Clinical Medicine, Clinical Immunology Unit-Scleroderma Center, Rome, Italy.
authors: E
authors: E
authors: Rosato
authors: C
authors: C
authors: Rossi
authors: I
authors: I
authors: Molinaro
authors: A
authors: A
authors: Giovannetti
authors: S
authors: S
authors: Pisarri
authors: Felice
authors: F
authors: Salsano
mesh_terms: D000111:Acetylcysteine / Q000627:therapeutic use*; D000328:Adult; D000368:Aged; D000975:Antioxidants / Q000627:therapeutic use*; D002121:Calcium Channel Blockers / Q000627:therapeutic use; D023381:Endpoint Determination; D005260:Female; D005385:Fingers / Q000473:pathology; D006801:Humans; D008168:Lung / Q000503:physiopathology; D017563:Lung Diseases, Interstitial / Q000188:drug therapy* / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D009543:Nifedipine / Q000627:therapeutic use; D011658:Pulmonary Fibrosis / Q000473:pathology; D011928:Raynaud Disease / Q000188:drug therapy; D012129:Respiratory Function Tests; D012189:Retrospective Studies; D012595:Scleroderma, Systemic / Q000188:drug therapy* / Q000503:physiopathology; D014109:Total Lung Capacity; D016896:Treatment Outcome; D014456:Ulcer / Q000188:drug therapy / Q000473:pathology; D014797:Vital Capacity; D055815:Young Adult
chemical_list: D000975:Antioxidants; D002121:Calcium Channel Blockers; D009543:Nifedipine; D000111:Acetylcysteine