1460 The Journal of Rheumatology 2009; 36:7; doi:10.3899/jrheum.081212 Personal non-commercial use only. The Journal of Rheumatology Copyright © 2009. All rights reserved. Autologous Hematopoietic Stem Cell Transplant in Systemic Sclerosis: Quantitative High Resolution Computed Tomography of the Chest Scoring DAVID LAUNAY, ZORAMARJANOVIC, CEDRIC de BAZELAIRE, LAURAFLOREA, SARAH ZOHAR, HOMAHKESHTMAND,CHRISTOPHEDELIGNY,AXELLEdeRAIGNIAC,ATHOLU.WELLS, andDOMINIQUEFARGE ABSTRACT. Objective. Usinghighresolutioncomputedtomography(HRCT),toassessthelunginvolvementout- come after autologous hematopoietic stem cell transplant (AHSCT) in patients with scleroderma (systemic sclerosis, SSc). Methods. HCRT scans prospectively performed before (n = 9 patients) and after (n = 47) AHSCT were blindly reviewed by 2 independent investigators using the Wells score. Results. After a median 60 months’ followup, the overall disease extent score from HCRT scans decreasedfrom10(0–45)to4(0–36)(p=0.04)6monthsafterAHSCT,andthereafterincreasedup to36monthsandstabilized;themodifiedRodnanskinscorefell(p<0.05). Conclusion. The extent of SSc lung involvement on HRCT rapidly but transiently regressed after AHSCT. (First Release June 15 2009; J Rheumatol 2009;36:1460–3; doi:10.3899/jrheum.081212) Key Indexing Terms: SYSTEMICSCLEROSIS AUTOLOGOUSHEMATOPOIETICSTEMCELLTRANSPLANT LUNGINVOLVEMENT FIBROSINGALVEOLITIS HIGHRESOLUTIONCOMPUTEDTOMOGRAPHYCHESTSCAN From Service de Médecine Interne, Hôpital Claude-Huriez, Université Lille 2, Lille; Service d’Hématologie, Hôpital Hôtel-Dieu, APHP; Service de Radiologie, Service de Médecine Interne et Pathologie Vasculaire, and Département de Biostatistique et Infomatique Médicale, Hôpital Saint-Louis, APHP, Paris; Service de Médecine Interne, CHU, Fort-de-France, France; Interstitial Lung Disease Unit, Royal Brompton Hospital, London, UK; and Unité INSERM U976 France, Hôpital Saint-Louis, Paris, France. D. Launay, MD, Service de Médecine Interne, Hôpital Claude-Huriez, Université Lille 2; Z. Marjanovic, MD, Service d’Hématologie, Hôpital Hôtel-Dieu, APHP; C. de Bazelaire, MD, Service de Radiologie, Hôpital Saint-Louis, APHP; L. Florea, MD, Service de Médecine Interne et Pathologie Vasculaire, Hôpital Saint-Louis, APHP; S. Zohar, MD, Département de Biostatistique et Infomatique Médicale, Hôpital Saint-Louis, APHP; H. Keshtmand, MD, Service de Médecine Interne et Pathologie Vasculaire, Hôpital Saint-Louis, APHP; C. Deligny, MD, Service de Médecine Interne, CHU; A. de Raigniac, MD, Service de Médecine Interne et Pathologie Vasculaire, Hôpital Saint-Louis, APHP; A.U. Wells, MD, Interstitial Lung Disease Unit, Royal Brompton Hospital; D. Farge, MD, PhD, Service de Médecine Interne et Pathologie Vasculaire, and Unité INSERM U976 France, Hôpital Saint-Louis. Address reprint requests to Dr. D. Farge, Service de médecine interne et pathologie vasculaire, Hôpital Saint-Louis, INSERM U976, 1 avenue Claude-Vellefaux, 75010 Paris, France. E-mail: dominique.farge-bancel@sls.ap-hop-paris.fr Accepted for publication February 11, 2009. Pulmonary fibrosis (PF) occurring in 50% to 80% of patients with systemic sclerosis (SSc) is their leading cause of death 1-3 . In this context, cyclophosphamide (CYC) was shown to significantly improve patient’s functional status, skin score, and, albeit modestly, lung function 4 . In the past 10 years, several phase I-II studies 5-8 used high doses of CYCfollowedbyautologoushematopoieticperipheralstem cell transplant (AHSCT) 6 . The studies showed complete or partial remission with stable vital capacity (VC) and diffus- ingcapacityforcarbonmonoxide(DLCO)onlungfunction testsupto5yearsafterAHSCTintwo-thirdsofthepatients treated for severe diffuse SSc. High resolution computed tomography (HRCT) of the chest is the most sensitive and reproducible method to analyze PF associated with SSc (PF-SSc) 1,3 , but presently no data are available on HRCT afterAHSCT.Wedesignedthisstudywithaspecialfocuson HRCTanalysisofPF-SSCinasubgroupofpatientsinclud- ed in a single-institution pilot study as reported 5 for whom longterm followup evaluation was available afterAHSCT. MATERIALS AND METHODS Fifty-six HCRT scans (using sequential acquisition of 1-mm scans, spaced at 10 mm, intervals extending from the lung apices to below the costophrenic angles) were prospectively performed in 9 patients with SSc fulfilling the American College of Rheumatology preliminary diagnosis criteria,before(n=9)andafter(n=47)AHSCT.Allpatientswithdiffuse cutaneous and severe SSc plus early visceral involvement treated by AHSCTaspublished 5 andwithatleast1yearfollowupafterAHSCTwere analyzed. Before AHSCT and quarterly thereafter, repeated evaluations duringfollowupincluded:(1)skininvolvementusingthemodifiedRodnan skinscore(mRSS);(2)lungfunctionandinvolvementusingtheNewYork HeartAssociation (NYHA) functional class, pulmonary function tests, and HRCTscans. Two independent investigators (CDB and DL) blindly and randomly reviewed each HRCT using the Wells score 1 . HRCT were reviewed at 5 levels 1 .ToquantifytheextentandtheseverityofCTpatternsateachlevel, weanalyzed(A)thediseaseextentofinterstitiallunginvolvement,includ- ing both reticular pattern and ground-glass opacification, and (B) the coarseness of fibrosis as follows: 0, ground-glass opacification alone; 1, fine intralobular fibrosis; 2, microcystic reticular pattern comprising air www.jrheum.org Downloaded on October 24, 2022 from