ACR Meeting Abstracts

ACR Meeting Abstracts

Abstract Number: 2166

Intravenous Immunoglobulins in Idiopathic Inflammatory Myopathies: Efficacy and Predictive Factors for Clinical Response

Simone Barsotti1,2, Ilaria Cavazzana3, Rossella Neri4, Francesco Locatelli5, Mara Taraborelli3,6, Elisa Cioffi7, Ilaria Chiapparoli5, Angela Tincani8, Franco Franceschini3 and Marta Mosca1, 1Rheumatology Unit, University of Pisa, Pisa, Italy, 2Department of Medical Biotechnologies, University of Siena, Siena, Italy, 3Rheumatology and Clinical Immunology, Spedali Civili of Brescia, Brescia, Italy, 4Rheumatology Unit, University of Pisa, PISA, Italy, 5Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foudation, Pavia, Italy, 6Internal Medicine; Ospedale Mellini, Chiari (Brescia), Italy, 7Rheumatology Unit, Rheumatology Unit, University of Pisa, Pisa, Italy, 8Rheumatology and Clinical Immunology, Spedali Civili and University of Brescia, Brescia, Italy

Meeting: 2017 ACR/ARHP Annual Meeting

Date of first publication: September 18, 2017

Keywords: , ,

Session Information

Date: Tuesday, November 7, 2017

Title: Muscle Biology, Myositis and Myopathies Poster

Session Type: ACR Poster Session C

Session Time: 9:00AM-11:00AM

Background/Purpose:

Despite the absence of specific guidelines or trials on intravenous immunoglobulins (IvIg) in patients with idiopathic inflammatory myopathies (IIM), the treatment is considered effective in refractory IIMs. The aim of our study is to evaluate the efficacy of IvIg in patients with IIM followed in 3 third level centers and to identify predictive factors correlated to their efficacy.

Methods:

retrospective study of IIM pts classified according to Bohan and Peter criteria treated with IvIg (2gr/kg/month). At baseline and after treatment the following data were collected: muscle necrosis enzymes, myositis specific autoantibodies; muscle strength (manual muscle test-MMT), clinical symptoms (skin lesions, dysphagia severity according to patient’s VAS, dyspnea, loss of strength) and clinical response according to physician judgment. We analyzed indications to treatment, major organ involvement, previous therapies.

Results:

92 pts were included (30 M, 62 F), 34 affected by PM, 48 DM, 3 IBM and 7 IIM overlap (3 Sjögren syndrome, 4 SSc); 12 were affected by cancer associated MII. Mean age at the start of IVIG therapy was 57.4±15 years, mean disease duration was 39±88 months.

Main indications to the treatment were: loss of strength (74), dysphagia (42), lung involvement (6), DM rash (27), arthritis (3).

IvIg were started in all pts for refractory disease to treatment with corticosteroids (CS). CS were used alone (22 pts) or associated with immunosuppressive drugs (70 pts). In 19/22 pts treated with cs alone, immunosuppressive drugs were contraindicated for neoplasia (9), recurrent infections (9), pregnancy (1).

The treatment was suspended after an average duration of 14 months; at the treatment suspension, muscle necrosis enzyme CK, LDH, GOT and GPT decreased significantly (p<0.001) and the mean MMT value increased (102.3±24.37 vs 125.0±23.16 p<0.001); mean dysphagia VAS decreased from 40.5±36.8 to 11.7±21.7 (p<0.001). The mean weekly corticosteroid dose decreased from 152.5±126.3 mg to 71.2±63.3 p<0.001.

According to physician judgment, 71 pts were classified as responder to IVIG (77.1%); 41 pts responded after six months of treatment and 30 pts after more than 6 months of treatment (mean 26.3±29.3 months).

Responders had a shorter disease duration (p<0.001), higher CK levels at baseline (2038±2479 vs 626±736 p<0.001), higher prevalence of dysphagia (p=0.012) and lower prevalence of Raynaud phenomenon (p=0.031) compared to non responders.

Conclusion:

Despite their high cost, IvIg confirmed their efficacy in refractory IIM pts, particularly in muscular and oesophageal manifestations. A shorter disease duration, higher CK levels and specific clinical features are associated to a better response.

Disclosure: S. Barsotti, None; I. Cavazzana, None; R. Neri, None; F. Locatelli, None; M. Taraborelli, None; E. Cioffi, None; I. Chiapparoli, None; A. Tincani, None; F. Franceschini, None; M. Mosca, None.

To cite this abstract in AMA style:

Barsotti S, Cavazzana I, Neri R, Locatelli F, Taraborelli M, Cioffi E, Chiapparoli I, Tincani A, Franceschini F, Mosca M. Intravenous Immunoglobulins in Idiopathic Inflammatory Myopathies: Efficacy and Predictive Factors for Clinical Response [abstract]. Arthritis Rheumatol. 2017; 69 (suppl 10). https://acrabstracts.org/abstract/intravenous-immunoglobulins-in-idiopathic-inflammatory-myopathies-efficacy-and-predictive-factors-for-clinical-response/. Accessed .

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