title: Intravenous sodium thiosulfate for treating tumoral calcinosis associated with systemic disorders: Report of four cases.
abstract: Intravenous sodium thiosulfate (ivSTS) is a promising new therapeutic option for calciphylaxis related to end-stage renal disease. However, its effect on tumoral calcinosis (TC) complicating autoimmune connective-tissue diseases has been scarcely described. We report here 4 cases (3 adults and 1 child) of TC treated with ivSTS. TC was secondary to CREST syndrome, dermatomyositis (1 adult and 1 child) and systemic erythematous lupus and involved multiple sites in all cases. In all 4 patients, TC was responsible for joint pain, reduced mobility, inflammatory flares and skin fistulations. One patient experienced difficulty sitting due to the pain induced by calcified lesions on the buttock; another patient had major disability, moved only with wheelchair and was under opioid treatment for pain. For all patients, treatment with several medications before STS was unsuccessful. The 3 adults received at least 6 cycles of ivSTS (20g/d, 5 days/month) and the child received a daily infusion of 17g STS during 1 month then a 9-g/d infusion during 3 months. Two adults and the child showed clinical improvement with STS treatment and the third adult felt disappointed and stopped STS treatment after 6 months. The child also stopped STS after 6 months due to vomiting. In one patient, an intensive regimen of ivSTS (20g every 2 days) controlled recurrent flares and fistulations. Unfortunately, TC remained unchanged. Further studies are needed to decipher how STS modulates ectopic calcification, the optimal regimen and posology.
authors.affiliation: Pôle locomoteur, service de rhumatologie, centre Viggo Petersen, hôpital Lariboisière, AP-HP, 2, rue Ambroise-Paré, 75010 Paris, France.
authors.forename: Arthur
authors.initials: A
authors.lastname: Mageau
authors.affiliation: Service de pédiatrie, hôpital de la Mère et de l'Enfant, 8, avenue Dominique-Larrey, 87000 Limoges, France.
authors.forename: Vincent
authors.initials: V
authors.lastname: Guigonis
authors.affiliation: Service de pharmacie, hôpital de la Mère et de l'Enfant, 8, avenue Dominique-Larrey, 87000 Limoges, France.
authors.forename: Voa
authors.initials: V
authors.lastname: Ratzimbasafy
authors.affiliation: Pôle locomoteur, service de rhumatologie, centre Viggo Petersen, hôpital Lariboisière, AP-HP, 2, rue Ambroise-Paré, 75010 Paris, France; UFR de médecine, université Paris 7 Denis Diderot, Paris Cité Sorbonne, 16, rue Henri-Huchard, 75018 Paris, France.
authors.forename: Thomas
authors.initials: T
authors.lastname: Bardin
authors.affiliation: Pôle locomoteur, service de rhumatologie, centre Viggo Petersen, hôpital Lariboisière, AP-HP, 2, rue Ambroise-Paré, 75010 Paris, France; UFR de médecine, université Paris 7 Denis Diderot, Paris Cité Sorbonne, 16, rue Henri-Huchard, 75018 Paris, France.
authors.forename: Pascal
authors.initials: P
authors.lastname: Richette
authors.affiliation: Service de néphrologie et dialyse, Ramsay-Générale de Santé, clinique du Landy, 23, rue de Landy, 93400 Saint-Ouen, France.
authors.forename: Pablo
authors.initials: P
authors.lastname: Urena
authors.affiliation: Pôle locomoteur, service de rhumatologie, centre Viggo Petersen, hôpital Lariboisière, AP-HP, 2, rue Ambroise-Paré, 75010 Paris, France; UFR de médecine, université Paris 7 Denis Diderot, Paris Cité Sorbonne, 16, rue Henri-Huchard, 75018 Paris, France. Electronic address: korngea@yahoo.fr.
authors.forename: Hang-Korng
authors.initials: HK
authors.lastname: Ea
mesh_terms: D061605:Administration, Intravenous; D002114:Calcinosis / Q000000981:diagnostic imaging / Q000188:drug therapy* / Q000209:etiology; D002614:Chelating Agents / Q000008:administration & dosage*; D006801:Humans; D013885:Thiosulfates / Q000008:administration & dosage*
chemical_list: D002614:Chelating Agents; D013885:Thiosulfates; C017717:sodium thiosulfate
keywords: CREST syndrome; Calcification; Dermatomyositis; Sodium thiosulfate; Systemic lupus erythematosus; Tumoral calcinosis