title: Efficacy of subcutaneous immunoglobulins in primary immunodeficiency with Crohn's-like phenotype: report of a case.
abstract: Common variable immune deficiency (CVID) is the most frequent primary immunodeficiency in adults. In CVID, the prevalence of gastrointestinal manifestations ranges between 2 and 50% with a complication-related morbidity second only to that of the respiratory tract. In some cases, clinical and endoscopic features are undistinguishable from those of inflammatory bowel disease (IBD). We describe the case of a 28-year-old man in which a diagnosis of Crohn's disease was firstly suspected. Subsequently, a diagnosis of Crohn's-like disease in a patient with CVID was made and a replacement therapy with human normal immunoglobulin intravenously was started. Unfortunately, serum IgG levels remained below 2.0 g/l in pre-infusional controls with persistence of gastrointestinal symptoms and malnutrition despite anti-inflammatory therapy (mesalazine, corticosteroids). Then, the patient began treatment with human normal immunoglobulins administered subcutaneously. The follow-up visits showed a progressive increase in serum IgG. Moreover, the patient reported improvement of gastrointestinal symptoms with reduction of diarrhoea, and laboratory tests showed a progressive and significant improvement. We confirm that therapy with subcutaneously administered immunoglobulins is safe and effective. In addition, our observations indicate that, for patients with CVID and enteropathic complications, replacement therapy with subcutaneous IgG may be the treatment of choice.
authors.affiliation: Department of Clinical Medicine and Surgery, University of Naples Federico II, School of Medicine, Naples, Italy. marcosanges1974@libero.it.
authors.forename: M
authors.initials: M
authors.lastname: Sanges
authors.forename: G
authors.initials: G
authors.lastname: Spadaro
authors.forename: M
authors.initials: M
authors.lastname: Miniero
authors.forename: D
authors.initials: D
authors.lastname: Mattera
authors.forename: R
authors.initials: R
authors.lastname: Sollazzo
authors.forename: F P
authors.initials: FP
authors.lastname: D'Armiento
authors.forename: G D
authors.initials: GD
authors.lastname: De Palma
authors.forename: A
authors.initials: A
authors.lastname: Pecoraro
authors.forename: F
authors.initials: F
authors.lastname: Borrelli
authors.forename: A
authors.initials: A
authors.lastname: Genovese
authors.forename: A
authors.initials: A
authors.lastname: D'Arienzo
mesh_terms: D000328:Adult; D017074:Common Variable Immunodeficiency / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy*; D003424:Crohn Disease / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy*; D006801:Humans; D007136:Immunoglobulins / Q000008:administration & dosage*; D007279:Injections, Subcutaneous; D008297:Male; D010641:Phenotype
chemical_list: D007136:Immunoglobulins