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Review
. 2023 Nov;22(11):103441.
doi: 10.1016/j.autrev.2023.103441. Epub 2023 Sep 12.

Immunoglobulins in systemic sclerosis management. A large multicenter experience

J Tandaipan  1 , A Guillén-Del-Castillo  2 , C P Simeón-Aznar  2 , P E Carreira  3 , C De la Puente  4 , J Narváez  5 , J Lluch  5 , M Rubio-Rivas  6 , J J Alegre-Sancho  7 , G Bonilla  8 , C Moriano  9 , I Casafont-Sole  10 , R García-Vicuña  11 , V Ortiz-Santamaría  12 , E Riera  13 , B Atienza-Mateo  14 , R Blanco  14 , C Galisteo  15 , J J Gonzalez-Martin  16 , J M Pego-Reigosa  17 , A Pros  18 , S Heredia  19 , I Castellví  20
Affiliations
Review

Immunoglobulins in systemic sclerosis management. A large multicenter experience

J Tandaipan et al. Autoimmun Rev. 2023 Nov.

Abstract

Objective: To analyze the effectiveness and safety of intravenous immunoglobulin (IVIG) given in routine care to patients with systemic sclerosis (SSc).

Methods: A retrospective multicenter observational study was conducted in SSc patients treated with IVIG. We collected data on epidemiological parameters and clinical outcomes. Firstly, we assessed changes in organ manifestations during IVIG treatment. Secondly, we analyzed the frequency of adverse effects. The following parameters were collected from baseline to the last follow-up: the patient's weight, modified Rodnan Skin Score (mRSS), modified manual muscle strength scale (MRC), laboratory test(creatine kinase(CK), hemoglobin and protein levels), The University of California Los Angeles Scleroderma Clinical Trials Consortium gastrointestinal tract 2.0 (UCLA GIT 2.0) questionnaire, pulmonary function tests, and echocardiography.

Results: Data were collected on 78 patients (82% females; 59% with diffuse SSc). Inflammatory idiopathic myopathy was the most frequent concomitant overlap disease (41%). The time since Raynaud's phenomenon and SSc onset were 8.8 ± 18 and 6.2 ± 6.7 years respectively. The most frequent IVIG indication was myositis (38/78), followed by gastrointestinal (27/78) and cutaneous (17/78) involvement. The median number of cycles given were 5. 54, 53 and 9 patients have been treated previously with glucocorticoids, synthetic disease-modifying antirheumatic drugs and biologic therapies respectively. After IVIG use we found significant improvements in muscular involvement (MRC ≥ 3/5 92% IVIG, p = 0.001 and CK levels from 1149 ± 2026 UI to 217 ± 224 UI, p = 0.02), mRSS (15 ± 12.4 to 13 ± 12.5, p = 0.015) and improvement in total score of UCLA GIT 2.0 (p = 0.05). None Anti-RNA polymerase III patients showed an adequate response in gastrointestinal involvement (0/7) in comparison with other antibodies (0 vs. 25, p = 0,039). Cardiorespiratory involvement remained stable. A total of 12 adverse events were reported with only one withdrawn due to serious adverse effect.

Conclusions: this study suggest that IVIG may improve myositis, gastrointestinal and skin involvement in SSc patients treated in routine care and seems to have a good safety profile.

Keywords: Immunoglobulins intravenous; Myositis; Systemic sclerosis; Treatment.

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Conflict of interest statement

Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper.

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