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Intravenous immunoglobulins improve the function and ameliorate joint involvement in systemic sclerosis: a pilot study
  1. F Nacci1,
  2. A Righi1,
  3. M L Conforti1,
  4. I Miniati1,
  5. G Fiori1,
  6. D Martinovic3,
  7. D Melchiorre1,
  8. T Sapir2,3,
  9. M Blank2,3,
  10. Y Shoenfeld2,3,
  11. A Moggi Pignone4,
  12. M Matucci Cerinic1
  1. 1Division of Medicine I and Rheumatology, Department of Medicine and Surgery, University of Florence, Florence, Italy
  2. 2Center for Autoimmune Disease, Department of Medicine B, Sheba Medical Center, Tel Hashomer, Israel
  3. 3Incumbent of the Laura Schwarz-Kipp Chair for Research of Autoimmune Disease, Tel Aviv University, Tel Aviv, Israel
  4. 4Division of Medicine II, Department of Medicine and Surgery, University of Florence, Florence, Italy
  1. Correspondence to:
    Professor M Matucci Cerinic
    Department of Medicine, Section of Rheumatology, Villa Monna Tessa, Viale Pieraccini 18, 50122 Firenze, Italy; cerinic{at}


Background: In systemic sclerosis (SSc), joint involvement may reduce the functional capacity of the hands. Intravenous immunoglobulins have previously been shown to benefit patients with SSc.

Aim: To verify the efficacy of intravenous immunoglobulins on joint involvement and function in SSc.

Patients and methods: 7 women with SSc, 5 with limited and 2 with diffuse SSc, with a severe and refractory joint involvement were enrolled in the study. Methotrexate and cyclophosphamide pulse therapy did not ameliorate joint symptoms. Hence, intravenous immunoglobulins therapy was prescribed at a dosage of 2 g/kg body weight during 4 days/month for six consecutive courses. The presence of joint tenderness and swelling, and articular deformities (due to primary joint involvement and not due to skin and subcutaneous changes) were evaluated. Before and after 6 months of treatment, patients were subjected to (1) Ritchie Index (RI) evaluation of joint involvement; (2) Dreiser Algo-Functional Index (IAFD) evaluation of hand joint function; (3) pain visual analogue scale (VAS) to measure joint pain; (4) Health Assessment Questionnaire (HAQ) to evaluate the limitations in everyday living and physical disability; and (5) modified Rodnan Skin Score for skin involvement.

Results: After 6 months of intravenous immunoglobulins therapy, joint pain and tenderness, measured with the VAS, decreased significantly (p<0.03), and hand function (IAFD) improved significantly (p<0.02), together with the quality of life (HAQ; p<0.03). All patients significantly improved, except for one. The skin score after 6 months of intravenous immunoglobulins therapy was significantly reduced (p<0.003).

Conclusion: This pilot study suggests that intravenous immunoglobulins may reduce joint pain and tenderness, with a significant recovery of joint function in patients with SSc with severe and refractory joint involvement. The cost of intravenous immunoglobulins might limit their use only to patients who failed disease-modifying antirheumatic drugs.

  • dSSc, diffuse systemic sclerosis
  • HAQ, Health Assessment Questionnaire
  • IAFD, Dreiser Algo-Functional Index
  • RA, rheumatoid arthritis
  • RI, Ritchie Index
  • SSc, systemic sclerosis
  • VAS, visual analogue scale

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  • Competing interests: None declared.

  • Published Online First 7 March 2007