title: Subcutaneous immunoglobulins for the treatment of a patient with antisynthetase syndrome and secondary chronic immunodeficiency after anti-CD20 treatment: a case report.
abstract: BACKGROUND
  Antisynthetase syndrome is a rare and debilitating multiorgan disease characterized by inflammatory myopathy, interstitial lung disease, cutaneous involvement, and frequent chronic inflammation of the joints. Standard treatments include corticosteroids and immunosuppressants. In some cases, treatment resistance may develop. Administration of immunoglobulins intravenously is recommended in patients with drug-resistant antisynthetase syndrome.
  
  
  METHODS
  Here, we describe the case of a 56-year-old woman of Algerian origin. She is the first case of a patient with multidrug-resistant antisynthetase syndrome featuring pulmonary involvement and arthropathy, and chronic secondary immune deficiency with recurrent infections, after anti-CD20 treatment, in which her primary antisynthetase syndrome-related symptoms and secondary immune deficiency were treated successfully with subcutaneous administration of immunoglobulin. The administration of immunoglobulin subcutaneously was introduced at a dose of 2 g/kg per month and was well tolerated. Clinical improvement was observed within 3 months of initiation of subcutaneous administration of immunoglobulin. After 22 months of treatment, she showed a significant improvement in terms of muscle strength, pulmonary involvement, arthralgia, and immunodeficiency. Her serum creatine phosphokinase and C-reactive protein levels remained normal. Finally, she was compliant and entirely satisfied with the treatment.
  
  
  CONCLUSIONS
  Taken together, these observations suggest that administration of immunoglobulin subcutaneously may be a useful therapeutic approach to tackle steroid-refractory antisynthetase syndrome while ensuring minimal side effects and improved treatment compliance. This treatment also allowed, in our case, for the regression of the chronic immunodeficiency secondary to rituximab treatment.
authors.affiliation: Department of Internal Medicine, Pitié-Salpetrière Hospital Group, 47-83 Boulevard de l'Hôpital, 75013, Paris, France. patrick.cherin@aphp.fr.
authors.forename: Patrick
authors.initials: P
authors.lastname: Cherin
authors.affiliation: Institut Galliera, 4 rue de Galliera, 75016, Paris, France.
authors.forename: Christophe
authors.initials: C
authors.lastname: de Jaeger
authors.affiliation: Octapharma France SAS, Boulogne Billancourt, France.
authors.forename: Jean-Charles
authors.initials: JC
authors.lastname: Crave
authors.affiliation: Octapharma France SAS, Boulogne Billancourt, France.
authors.forename: Jean-Christophe
authors.initials: JC
authors.lastname: Delain
authors.affiliation: ClinSearch, Malakoff, France.
authors.forename: Abir
authors.initials: A
authors.lastname: Tadmouri
authors.affiliation: Department of Internal Medicine, Pitié-Salpetrière Hospital Group, 47-83 Boulevard de l'Hôpital, 75013, Paris, France.
authors.forename: Zahir
authors.initials: Z
authors.lastname: Amoura
mesh_terms: D000361:Agammaglobulinemia / Q000150:complications; D005260:Female; D006801:Humans; D007136:Immunoglobulins / Q000008:administration & dosage*; D007153:Immunologic Deficiency Syndromes / Q000150:complications; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D017563:Lung Diseases, Interstitial / Q000150:complications / Q000000981:diagnostic imaging; D008279:Magnetic Resonance Imaging; D008727:Methotrexate / Q000627:therapeutic use; D008875:Middle Aged; D009220:Myositis / Q000150:complications / Q000000981:diagnostic imaging / Q000628:therapy*; D065566:Subcutaneous Absorption
chemical_list: D007136:Immunoglobulins; D007166:Immunosuppressive Agents; D008727:Methotrexate
keywords: Anti-Jo-1 antibody; Antisynthetase syndrome; Autoimmune disease; Case report; Myositis; Secondary immunodeficiency; Subcutaneous human immunoglobulin