title: Successful Treatment of Lung Calciphylaxis With Sodium Thiosulfate in a Patient With Sickle Cell Disease: A Case Report.
abstract: Calciphylaxis is a small vessel vasculopathy, characterized by medial wall calcification that develops in a few patients with chronic renal failure. The prognosis of skin calciphylaxis has improved considerably since the introduction of sodium thiosulfate (STS), but it remains unclear whether this therapy is effective against organ lesions related to calciphylaxis. Pulmonary calciphylaxis is a usually fatal medical condition that may occur in association with skin involvement in patients with end-stage renal disease.We report here the case of a 49-year-old woman homozygous sickle cell disease patient on chronic hemodialysis with biopsy-proven systemic calciphylaxis involving the lungs and skin. On admission, ulcerative skin lesions on the lower limbs and bilateral pulmonary infiltrates on chest computerized tomography scan were the main clinical and radiological findings. Skin and bronchial biopsies demonstrated calciphylaxis lesions. The intravenous administration of STS in association with cinacalcet for 8 consecutive months led to a clear improvement in skin lesions and thoracic lesions on chest computerized tomography scan.This case suggests for the first time that organ lesions related to calciphylaxis, and particularly lung injury, are potentially reversible. This improvement probably resulted from the combination of 3 interventions (more frequent dialysis, cinacalcet, and STS), rather than the administration of STS alone.
authors.affiliation: From the Service de Néphrologie et Transplantation, Hôpitaux Universitaires Henri Mondor, Centre de référence maladie rare Syndrome Néphrotique Idiopathique, Institut Francilien de recherche en Néphrologie et Transplantation (IFRNT), AP-HP (Assistance Publique-Hôpitaux de Paris), Créteil, France (RA, CD, PR, GB, BB, CL, AD, TK, MM, DS, PG, PL, VA); Equipe 21, INSERM Unité 955 (RA, CD, PR, GB, BB, CL, AD, TK, MM, DS, PG, PL, VA); Centre de Dialyse des Nouvelles Eaux Vives, 97100 Basse-Terre, Guadeloupe (RD); Service d'Imagerie Médicale, Hôpitaux Universitaires Henri Mondor, APHP, UPEC, Créteil, France (J-FD); Service de Pneumologie, Hôpitaux Universitaires Henri Mondor, APHP, UPEC, Créteil, France (CC, BM); Centre de Référence des Syndromes Drépanocytaires Majeurs, Hôpitaux Universitaires Henri Mondor, APHP, UPEC, Créteil, France (PB); and Département de Pathologie, Hôpitaux Universitaires Henri Mondor, APHP, UPEC, Créteil, France (J-TVN), Créteil, France.
authors.forename: Romain
authors.initials: R
authors.lastname: Arrestier
authors.forename: Caroline
authors.initials: C
authors.lastname: Dudreuilh
authors.forename: Philippe
authors.initials: P
authors.lastname: Remy
authors.forename: Ghada
authors.initials: G
authors.lastname: Boulahia
authors.forename: Bouteina
authors.initials: B
authors.lastname: Bentaarit
authors.forename: Claire
authors.initials: C
authors.lastname: Leibler
authors.forename: Amir
authors.initials: A
authors.lastname: Adedjouma
authors.forename: Tomek
authors.initials: T
authors.lastname: Kofman
authors.forename: Marie
authors.initials: M
authors.lastname: Matignon
authors.forename: Dil
authors.initials: D
authors.lastname: Sahali
authors.forename: Roger
authors.initials: R
authors.lastname: Dufresne
authors.forename: Jean-Francois
authors.initials: JF
authors.lastname: Deux
authors.forename: Charlotte
authors.initials: C
authors.lastname: Colin
authors.forename: Philippe
authors.initials: P
authors.lastname: Grimbert
authors.forename: Philippe
authors.initials: P
authors.lastname: Lang
authors.forename: Pablo
authors.initials: P
authors.lastname: Bartolucci
authors.forename: Bernard
authors.initials: B
authors.lastname: Maitre
authors.forename: Jeanne
authors.initials: J
authors.lastname: Tran Van Nhieu
authors.forename: Vincent
authors.initials: V
authors.lastname: Audard
mesh_terms: D000755:Anemia, Sickle Cell / Q000150:complications; D000975:Antioxidants / Q000627:therapeutic use*; D002115:Calciphylaxis / Q000150:complications / Q000188:drug therapy*; D005260:Female; D006801:Humans; D008171:Lung Diseases / Q000150:complications / Q000188:drug therapy*; D008875:Middle Aged; D012074:Remission Induction; D013885:Thiosulfates / Q000627:therapeutic use*
chemical_list: D000975:Antioxidants; D013885:Thiosulfates; C017717:sodium thiosulfate