Rituximab in the Treatment of Jo-1 Antibody-Associated Antisynthetase Syndrome: Anti-Ro52 Positivity As a Marker for Severity and Treatment Response

Jutta Bauhammer¹, Norbert Blank², Hanns-Martin Lorenz³, Regina Max⁴, Dietmar Krause⁵ and Christoph Fiehn¹, ¹ACURA Centre for Rheumatic Diseases, Baden-Baden, Germany, ²Dept. of Internal Medicine 5, Division of Rheumatology, University of Heidelberg, Heidelberg, Germany, ³Department of Internal Medicine 5, Division of Rheumatology, University of Heidelberg, Heidelberg, Germany, ⁴Dept. of Internal Medicine 5, Division of Rheumatology, University of Heidelberg, Heidelberg, Germany, ⁵Dept. of Medical Informatics, Biometry and Epidemiology, Ruhr University Bochum, Bochum, Germany

Meeting: 2015 ACR/ARHP Annual Meeting

Date of first publication: September 29, 2015

Keywords: Antibodies, interstitial lung disease, myositis and rituximab

Session Information

Date: Tuesday, November 10, 2015

Title: Muscle Biology, Myositis and Myopathies Poster II: Autoantibodies and Treatments in Inflammatory Myopathies

Session Type: ACR Poster Session C

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

Background/Purpose: Rituximab (RIX) has successfully been used for the treatment of severe Jo-1 antibody-associated antisynthetase syndrome (Jo-1 ASS). The aim of this retrospective study was to evaluate the effect of RIX in severe Jo-1 ASS and to determine predictive factors for response.

Methods: 61 patients with Jo-1 ASS were identified in two centres between 2006 and 2014; 18 of these received RIX. 17 patients in a dosage of 2x1g i.v. (day 0 and 14) and 1 patient 4 weekly infusions of 375 mg/m² body surface. Treatment cycles were repeated every six months. One patient was lost to follow-up, but the remaining 17 RIX-patients, and 30 out of 43 patients who were treated with conventional immunosuppressive drugs, were followed for a mean of 35 and 84 months, respectively. Anti-Ro52 antibodies in serum were measured with a semiquantitative immunoblotting assay (EUROLINE, Euroimmun, Germany) and the test was validated with standard immunoprecipitation technique.

Results: Polymyositis (in 95%) and interstitial lung disease (ILD, in 67%) were the dominant clinical manifestations. Detection of anti-Ro52 antibodies (in 43%) in serum was significantly associated with acute onset ILD (p=0.016) with O₂ dependency, and patients with high concentrations of anti-Ro52 (in 20%) had the highest risk (p=0.0005). 16 out of 18 patients (89%) showed a fast and almost complete response to RIX. CK serum concentrations (mean CK was 663 U/l before treatment) dropped to normal values in all patients. Results in pulmonary functional tests improved significantly (p<0.05). Prednisolone-equivalent doses per day decreased from a mean of 30.4 mg/d to 6.0 mg/d. Patients received 4.6 cycles of RIX on average (range: 1–13). There were five relapses in four patients. Three of them occurred when intervals between RIX treatment cycles were extended longer than 6 months. In all these patients treatment with RIX was repeated and again had a good effect. Among those patients who were highly positive for anti-Ro52, good response to RIX was seen in 7 out of 7 cases (100%), but no response to either cyclophosphamide (n=4), cyclosporine A (n=3), azathioprine (n=9), methotrexate (n=5) or leflunomide (n=2) was observed in the patients highly positive for anti-Ro52-antibodies who did not receive RIX. One patient treated with RIX and long term medium dose prednisone died of pneumonia after the second treatment cycle. No other severe adverse events occurred during RIX therapy.

Conclusion: RIX seems to be highly effective in the treatment of severe forms of Jo-1 ASS. The presence of high anti-Ro52 antibody concentrations in these patients predicts severe acute onset ILD, non-response to immunosuppressive drugs, but good and lasting response to RIX.

Disclosure: J. Bauhammer, None; N. Blank, Roche Pharmaceuticals, 5; H. M. Lorenz, Roche Pharmaceuticals, 5; R. Max, None; D. Krause, None; C. Fiehn, Roche Pharmaceuticals, 5.

To cite this abstract in AMA style:

Bauhammer J, Blank N, Lorenz HM, Max R, Krause D, Fiehn C. Rituximab in the Treatment of Jo-1 Antibody-Associated Antisynthetase Syndrome: Anti-Ro52 Positivity As a Marker for Severity and Treatment Response [abstract]. Arthritis Rheumatol. 2015; 67 (suppl 10). https://acrabstracts.org/abstract/rituximab-in-the-treatment-of-jo-1-antibody-associated-antisynthetase-syndrome-anti-ro52-positivity-as-a-marker-for-severity-and-treatment-response/. Accessed .

« Back to 2015 ACR/ARHP Annual Meeting

ACR Meeting Abstracts - https://acrabstracts.org/abstract/rituximab-in-the-treatment-of-jo-1-antibody-associated-antisynthetase-syndrome-anti-ro52-positivity-as-a-marker-for-severity-and-treatment-response/