title: Long-term efficacy of B cell depletion therapy on lung and skin involvement in diffuse systemic sclerosis.
abstract: OBJECTIVE
  To assess the long-term efficacy and safety of single and multiple courses of rituximab therapy in systemic sclerosis (SSc) patients with and without lung disease.
  
  
  METHODS
  A total of 20 SSc patients with a diffuse disease were treated with rituximab. At baseline and during follow-up the lung involvement was evaluated with pulmonary function tests (FVC and DLCO) and with lung high-resolution computed tomography (HRCT).
  
  
  RESULTS
  The skin score, activity, and severity indices improved significantly after 12 months and at final follow-up compared to baseline. After 12 months, there was a significant increase of FVC and TLC compared to baseline (p = 0.024 and p = 0.005, respectively), while the mean DLCO value remained stable. Considering the last available follow-up in six patients with restrictive lung disease at baseline, two patients (33.3%) experienced an increase of more than 10% of FVC, one patient had a decrease of FVC >10%, while in three patients FVC remained stable (50%). After the mean follow-up of 48.5 ± 20.4 months, among the patients with normal lung parameters at baseline, FVC remained stable in 12 (85.7%) and in one patient (14.3%) it increased by more than 10%. At the final follow-up, the alveolar and interstitial HRCT scores remained stable in more than 80% of patients, both in patients with and without restrictive lung disease at baseline.
  
  
  CONCLUSIONS
  Anti-CD20 B cell depletion therapy is effective on skin involvement but seems also to preserve the pulmonary function, as supported by a stable or improved FVC and stable interstitial score, suggesting a possible role of rituximab as a modifying therapy overall in early diffuse SSc.
authors.affiliation: Department of Rheumatology, Institute of Rheumatology and Affine Sciences, Catholic University of the Sacred Heart, Rome, Italy.
authors.forename: Silvia L
authors.initials: SL
authors.lastname: Bosello
authors.affiliation: Department of Rheumatology, Institute of Rheumatology and Affine Sciences, Catholic University of the Sacred Heart, Rome, Italy.
authors.forename: Giacomo
authors.initials: G
authors.lastname: De Luca
authors.affiliation: Department of Rheumatology, Institute of Rheumatology and Affine Sciences, Catholic University of the Sacred Heart, Rome, Italy.
authors.forename: Manuela
authors.initials: M
authors.lastname: Rucco
authors.affiliation: Department of Rheumatology, Institute of Rheumatology and Affine Sciences, Catholic University of the Sacred Heart, Rome, Italy.
authors.forename: Giorgia
authors.initials: G
authors.lastname: Berardi
authors.affiliation: Institute of Radiology, Catholic University of the Sacred Heart, Rome, Italy.
authors.forename: Matteo
authors.initials: M
authors.lastname: Falcione
authors.affiliation: Institute of Radiology, Catholic University of the Sacred Heart, Rome, Italy.
authors.forename: Francesco Maria
authors.initials: FM
authors.lastname: Danza
authors.affiliation: Institute of Radiology, Catholic University of the Sacred Heart, Rome, Italy.
authors.forename: Tommaso
authors.initials: T
authors.lastname: Pirronti
authors.affiliation: Department of Rheumatology, Institute of Rheumatology and Affine Sciences, Catholic University of the Sacred Heart, Rome, Italy. Electronic address: gf.ferraccioli@rm.unicatt.it.
authors.forename: Gianfranco
authors.initials: G
authors.lastname: Ferraccioli
mesh_terms: D000328:Adult; D058846:Antibodies, Monoclonal, Murine-Derived / Q000494:pharmacology / Q000627:therapeutic use*; D018951:Antigens, CD20 / Q000187:drug effects / Q000276:immunology*; D001402:B-Lymphocytes / Q000187:drug effects / Q000276:immunology / Q000473:pathology*; D003520:Cyclophosphamide / Q000494:pharmacology / Q000627:therapeutic use; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008168:Lung / Q000187:drug effects / Q000473:pathology* / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D012129:Respiratory Function Tests; D000069283:Rituximab; D012595:Scleroderma, Systemic / Q000000981:diagnostic imaging / Q000188:drug therapy* / Q000503:physiopathology; D012720:Severity of Illness Index; D012867:Skin / Q000187:drug effects / Q000473:pathology*; D013997:Time Factors; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome
chemical_list: D058846:Antibodies, Monoclonal, Murine-Derived; D018951:Antigens, CD20; D000069283:Rituximab; D003520:Cyclophosphamide
keywords: B cells; CD20 depletion therapy; Lung involvement; Skin fibrosis; Systemic sclerosis