Session Type: ACR Poster Session C
Session Time: 9:00AM-11:00AM
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.
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.
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.
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.
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 January 22, 2022.
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