title: Systemic capillary leak syndrome and autoimmune diseases: A case series.
abstract: Systemic capillary leak syndrome (Clarkson's disease) is a rare entity characterized by recurrent and unpredictable attacks of capillary leakage of plasma fluid and proteins throughout the endothelium. Some cases are secondary. We describe the rare association between secondary capillary leak syndrome (SCLS) and autoimmune diseases.
  
  
  
  We conducted a nationwide, retrospective, observational, and collaborative study throughout the hospital units of the Club des Rhumatismes et Inflammations network (CRI) between March and August 2015. Inclusion criteria were patients with (1) capillary leakage episodes characterized by edema and elevated hematocrit, low albumin count without proteinuria, or other cause of protein loss; and (2) definite autoimmune diseases according to international classification criteria.
  
  
  
  The clinical and biological data of five patients (three women) were reviewed. Median age was 43.2 (17-55) years. Four patients had Sjögren syndrome. One of them also fulfilled the criteria for systemic sclerosis (n = 1). The fifth patient had polymyositis. During the 37.2 months of median follow-up (5.4-201), we recorded a total of 24 attacks, yielding an attack incidence of 91/100 patient-years. Laboratory tests revealed that three patients had anti-SSA/Ro antibodies. Only one patient had a monoclonal blood component (IgGκ). Three patients needed ICU support; one died during a flare.
  
  
  
  We reported the first case series of a rare association of SCLS and autoimmune diseases, supporting the idea of some immune mediation in the pathogenesis of the former disease.
authors.affiliation: Service de Rhumatologie, Centre National de Référence des Maladies Autoimmunes Rares, Hôpitaux Universitaires de Strasbourg, Strasbourg, France; Service d'Immunologie Clinique et de Médecine Interne, Centre National de Référence des Maladies Autoimmunes Rares, Hôpitaux Universitaires de Strasbourg, 1, place de l'Hôpital 67091, Strasbourg, France; Fédération de médecine translationnelle de Strasbourg (FMTS), Université de Strasbourg, Strasbourg Cedex, Strasbourg, France. Electronic address: aurelien.guffroy@chru-strasbourg.fr.
authors.forename: Aurélien
authors.initials: A
authors.lastname: Guffroy
authors.affiliation: Service de Médecine Interne, Hôpitaux Universitaires de Strasbourg, Strasbourg, France; Fédération de médecine translationnelle de Strasbourg (FMTS), Université de Strasbourg, Strasbourg Cedex, Strasbourg, France.
authors.forename: Benjamin
authors.initials: B
authors.lastname: Dervieux
authors.affiliation: Service de Rhumatologie, Centre National de Référence des Maladies Autoimmunes Rares, Hôpitaux Universitaires de Strasbourg, Strasbourg, France; Fédération de médecine translationnelle de Strasbourg (FMTS), Université de Strasbourg, Strasbourg Cedex, Strasbourg, France.
authors.forename: Simon
authors.initials: S
authors.lastname: Gravier
authors.affiliation: Service de Médecine Interne, Hôpitaux Universitaires de Strasbourg, Strasbourg, France; Fédération de médecine translationnelle de Strasbourg (FMTS), Université de Strasbourg, Strasbourg Cedex, Strasbourg, France.
authors.forename: Camille
authors.initials: C
authors.lastname: Martinez
authors.affiliation: Service de Médecine Interne et d'Immunologie Clinique, CHU Nancy-Brabois, Nancy, France.
authors.forename: Joelle
authors.initials: J
authors.lastname: Deibener-Kaminsky
authors.affiliation: Service de Médecine Interne, Centre National de Référence des Maladies Autoimmunes, Hôpital Claude Huriez CHRU, Lille, France.
authors.forename: Eric
authors.initials: E
authors.lastname: Hachulla
authors.affiliation: Service de Médecine Interne, Hôpital Henry Mondor, Creteil, France.
authors.forename: Marc
authors.initials: M
authors.lastname: Michel
authors.affiliation: Service de Médecine Interne, Hôpitaux Universitaires de Strasbourg, Strasbourg, France; Fédération de médecine translationnelle de Strasbourg (FMTS), Université de Strasbourg, Strasbourg Cedex, Strasbourg, France.
authors.forename: Jean-Christophe
authors.initials: JC
authors.lastname: Weber
authors.affiliation: Service d'Immunologie Clinique et de Médecine Interne, Centre National de Référence des Maladies Autoimmunes Rares, Hôpitaux Universitaires de Strasbourg, 1, place de l'Hôpital 67091, Strasbourg, France; Fédération de médecine translationnelle de Strasbourg (FMTS), Université de Strasbourg, Strasbourg Cedex, Strasbourg, France.
authors.forename: Anne-Sophie
authors.initials: AS
authors.lastname: Korganow
authors.affiliation: Service de Rhumatologie, Centre National de Référence des Maladies Autoimmunes Rares, Hôpitaux Universitaires de Strasbourg, Strasbourg, France; Fédération de médecine translationnelle de Strasbourg (FMTS), Université de Strasbourg, Strasbourg Cedex, Strasbourg, France.
authors.forename: Laurent
authors.initials: L
authors.lastname: Arnaud
authors.affiliation: Service de Rhumatologie, Centre National de Référence des Maladies Autoimmunes Rares, Hôpitaux Universitaires de Strasbourg, Strasbourg, France; Fédération de médecine translationnelle de Strasbourg (FMTS), Université de Strasbourg, Strasbourg Cedex, Strasbourg, France.
authors.forename: Jacques-Eric
authors.initials: JE
authors.lastname: Gottenberg
authors.affiliation: Service de Rhumatologie, Centre National de Référence des Maladies Autoimmunes Rares, Hôpitaux Universitaires de Strasbourg, Strasbourg, France; Immunogénétique Moléculaire et Humaine, Centre de Recherche d'Immunologie et d'Hématologie, Faculté de Médecine, Hôpitaux Univesitaires de Strasbourg, Strasbourg, France; Fédération de médecine translationnelle de Strasbourg (FMTS), Université de Strasbourg, Strasbourg Cedex, Strasbourg, France.
authors.forename: Jean
authors.initials: J
authors.lastname: Sibilia
mesh_terms: D000293:Adolescent; D000328:Adult; D000974:Antibodies, Antinuclear / Q000276:immunology; D001327:Autoimmune Diseases / Q000150:complications / Q000276:immunology; D019559:Capillary Leak Syndrome / Q000150:complications*; D005260:Female; D005500:Follow-Up Studies; D005602:France; D006801:Humans; D008297:Male; D008875:Middle Aged; D017285:Polymyositis / Q000150:complications* / Q000276:immunology; D012189:Retrospective Studies; D012595:Scleroderma, Systemic / Q000150:complications* / Q000276:immunology; D012859:Sjogren's Syndrome / Q000150:complications* / Q000276:immunology; D055815:Young Adult
chemical_list: D000974:Antibodies, Antinuclear; C035356:SS-A antibodies
keywords: Anti-Ro/SSA, Sjögren; Autoimmunity; Capillary leak syndrome; Inflammatory myopathy; Systemic sclerosis