ACR Meeting Abstracts

ACR Meeting Abstracts

Abstract Number: 290

Cancer and Necrotizing Immune Mopathy: High Incidence in Anti-3-Hydroxy-3-Methylglutaryl-Coenzyme a Reductase Positive and Seronegative Patients but Not in Anti-Single Recognition Particle Positive Patients

Yves Allenbach1, Jeremy Kaeren1, Nicolas Champtiaux1, Yoland Schoindre1, Kuberaka Mariampillai1, Aude Rigolet1, Lucile Musset2, Jean- Luc Charuel2, Anthony Behin3, Bruno Eymard4, Pascal Laforet5, Tanya Stojkovic5, Olivier Boyer6, Baptiste Hervier7 and Olivier Benveniste1, 1Department de Internal Medicine and Clinical Immunology, Hôpital Pitié-Salpêtrière, AP-PH, UPMC, Paris, France, 2Laboratoire d'immunochimie, Pitié- Salpêtrière, Paris, France, 3Institute of Myology, Pitie-Salpetriere Hospital, Paris, France, 4Department of Neurology, Hôpital Pitié-Salpêtrière, AP-PH, UPMC, Paris, France, 5Department of Neurology, Myology Institute, Assistance Publique - Hôpitaux de Paris, Pitié-Salpêtrière University, Paris, France, 6Immunology, Inserm 905 & Institute for Biomedical Research, University of Rouen, Rouen, France, 7Department de Internal Medicine and Clinical Immunology, Hôpital Pitié-Salpêtrière, AP-PH, UPMC, Paris, Paris, France

Meeting: 2015 ACR/ARHP Annual Meeting

Date of first publication: September 29, 2015

Keywords: , ,

Session Information

Date: Sunday, November 8, 2015

Title: Muscle Biology, Myositis and Myopathies Poster

Session Type: ACR Poster Session A

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

Background/Purpose: Twenty percent of inflammatory myopathy are associated with a synchronous cancer occurring ±3 years around the diagnosis. Malignancy is a major cause of mortality in inflammatory myopathy. The association is mainly reported in dermatomyositis (DM) patients. It has been clarified that DM patients positive for either anti-TIF1γ or anti-NXP2 myositis specific anti-bodies have increased risk of cancer. Some reports showed that malignancy could also occur in patients with necrotizing myopathies (NAM).

We aim to analyse the incidence of cancer in a large cohort of NAM with or without the myositis specific anti-single recognition particle (SRP) or anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase (HMGCR) auto-antibodies.

Methods: One hundred fifteen NAM patients were analysed: 48 SRP patients, 52 HMGCR patients and 15 seronegative NAM. NAM was defined based on pathological criteria.

Results: Malignancy occurred in 28.5% of seronegative NAM patients and in 17.3% of HMGCR patients compared to 6.1% in SRP patients (p=0.04). The median time between the diagnostic of malignancy and the myopathy was 4.9±21 months in seronegative NAM patients, 25.1±58 months in HMGCR patients and 80.1±76.2 months in SRP patients. Synchronous malignancy (±3 years) was diagnosed in 21.4% of seronegative NAM patients, 11.5% of HMGCR patients and 4% of SRP patients (p=0.03). No specific types of cancer was predominant. Compare to the French cancer registry increase incidence of cancer occurs in seronegative NAM and HMGCR NAM with standard incidence ratios of 8.35 [1.7-24.4] and 2.79 [1.02-6.07] respectively. Survival analysis showed that patients suffering from a synchronous cancer had a poor out-come with a median survival of 57.2 years (p<0.0001).

Conclusion: Seronegative NAM and anti-HMGCR patients have an increased risk of malignancy. The presence of cancer is associated with a poor survival. Cancer screening is necessary in seronegative and in anti-HMGCR NAM patients.

Disclosure: Y. Allenbach, None; J. Kaeren, None; N. Champtiaux, None; Y. Schoindre, None; K. Mariampillai, None; A. Rigolet, None; L. Musset, None; J. L. Charuel, None; A. Behin, None; B. Eymard, None; P. Laforet, None; T. Stojkovic, None; O. Boyer, None; B. Hervier, None; O. Benveniste, None.

To cite this abstract in AMA style:

Allenbach Y, Kaeren J, Champtiaux N, Schoindre Y, Mariampillai K, Rigolet A, Musset L, Charuel JL, Behin A, Eymard B, Laforet P, Stojkovic T, Boyer O, Hervier B, Benveniste O. Cancer and Necrotizing Immune Mopathy: High Incidence in Anti-3-Hydroxy-3-Methylglutaryl-Coenzyme a Reductase Positive and Seronegative Patients but Not in Anti-Single Recognition Particle Positive Patients [abstract]. Arthritis Rheumatol. 2015; 67 (suppl 10). https://acrabstracts.org/abstract/cancer-and-necrotizing-immune-mopathy-high-incidence-in-anti-3-hydroxy-3-methylglutaryl-coenzyme-a-reductase-positive-and-seronegative-patients-but-not-in-anti-single-recognition-particle-positive-pa/. Accessed .

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