title: Sustained benefit from intravenous immunoglobulin therapy for gastrointestinal involvement in systemic sclerosis.
abstract: OBJECTIVE
  IVIG is known to confer significant benefit in rheumatologic conditions, including inflammatory myopathy. This study aimed to assess the efficacy of IVIG across different aspects of internal organ involvement in refractory active SSc, particularly the gastrointestinal (GI) system.
  
  
  METHODS
  SSc patients with overlap polymyositis who remained active and unresponsive to conventional disease-modifying agents and who subsequently received IVIG were identified. GI symptoms were assessed using validated questionnaires. The Medical Research Council Sum Score for muscle strength and modified Rodnan skin score (mRSS) were assessed. Serial measurements were undertaken at baseline prior to the first IVIG treatment and post-treatment in the most recent assessment.
  
  
  RESULTS
  Fifteen SSc patients were consecutively recruited into this observational study. The mean duration of IVIG treatment was 2.3 years, with treatment frequency ranging from every 6 weeks to 4 months. Compared with baseline, there was a significant reduction in gastro-oesophageal reflux frequency and intensity mean scores (P = 0.006 and P = 0.013, respectively). Significant improvement in the Gastrointestinal Tract (GIT) 2.0 score from a baseline mean score of 1.07 (s.d. 0.67) to 0.60 (0.46) (P = 0.002) was observed. There was regression in the markers of muscle disease with a reduction in the mean (s.d.) Medical Research Council sum score and the median creatine kinase level (P = 0.001 and P = 0.025, respectively). Significant amelioration of the mean basal modified Rodnan skin score from 21.5 (s.d. 13.8) to 10 (10.6) (P = 0.005) was observed.
  
  
  CONCLUSIONS
  IVIG may be a helpful adjunctive therapy in the amelioration of some key clinical aspects in refractory SSc. Sustained benefit from IVIG suggests a specific immunomodulatory effect on those with established SSc GI complications.
authors: Centre for Rheumatology and Connective Tissue Diseases, University College London Medical School, Royal Free Campus, London, UK, Division of Rheumatology, Department and Faculty of Medicine, University of Malaya, Kuala Lumpur, Malaysia and.
authors: Jasmin
authors: J
authors: Raja
authors: Centre for Rheumatology and Connective Tissue Diseases, University College London Medical School, Royal Free Campus, London, UK.
authors: Svetlana I
authors: SI
authors: Nihtyanova
authors: Centre for Gastroenterology, Royal Free Hospital, Pond Street, London, UK.
authors: Charles D
authors: CD
authors: Murray
authors: Centre for Rheumatology and Connective Tissue Diseases, University College London Medical School, Royal Free Campus, London, UK, c.denton@ucl.ac.uk.
authors: Christopher P
authors: CP
authors: Denton
authors: Centre for Rheumatology and Connective Tissue Diseases, University College London Medical School, Royal Free Campus, London, UK.
authors: Voon H
authors: VH
authors: Ong
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D005260:Female; D005500:Follow-Up Studies; D005767:Gastrointestinal Diseases / Q000188:drug therapy* / Q000209:etiology; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000008:administration & dosage*; D007155:Immunologic Factors / Q000008:administration & dosage; D007275:Injections, Intravenous; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D012595:Scleroderma, Systemic / Q000150:complications / Q000188:drug therapy*; D011795:Surveys and Questionnaires; D013997:Time Factors; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D016756:Immunoglobulins, Intravenous; D007155:Immunologic Factors
keywords: gastrointestinal manifestation; intravenous immunoglobulin; myositis; refractory; systemic sclerosis