Onco-Hematological Malignancies in Systemic Necrotizing Vasculitides: Pooled Analysis of Five Prospective, Randomized, Controlled Trials

Lafarge Antoine¹, Christian Pagnoux², Xavier Puéchal³, Maxime Samson⁴, Mohamed Hamidou⁵, Alexandre Karras⁶, Thomas Quémeneur⁷, Matthieu Groh⁸, Luc Mouthon⁹, Loïc Guillevin for the French Vasculitis Study Group³ and Benjamin Terrier¹⁰, ¹Medecine Interne, Hôpital Cochin, Paris, France, ²Mount Sinai Hospital, Toronto, ON, Canada, ³Service de Médecine Interne, Centre de Référence Maladies Auto-Immunes et Auto-Inflammatoires Systémiques Rares, Hôpital Cochin, Paris, France, ⁴Department of Internal Medicine and Clinical Immunology, Hôpital François Mitterrand, CHU de Dijon, Dijon, France, ⁵Medecine Interne, CHU Hôtel Dieu, Nantes, France, ⁶nephrology, HEGP, Paris, France, ⁷Service de néphrologie, médecine interne et vasculaire, Hôpital de Valenciennes, Valenciennes, France, ⁸National Referral Center for Rare Systemic Autoimmune Diseases, Hôpital Cochin, AP–HP, Université Paris Descartes, Paris, France, ⁹Université Paris Descartes Sorbonne Paris, Paris, France, ¹⁰Internal Medicine, Cochin University Hospital, Paris, France

Meeting: 2017 ACR/ARHP Annual Meeting

Date of first publication: September 18, 2017

Keywords: Cancer, immunosuppressants, Malignancy, tolerance and vasculitis

Session Information

Date: Tuesday, November 7, 2017

Title: Vasculitis Poster III: Other Vasculitis Syndromes

Session Type: ACR Poster Session C

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

Background/Purpose:

The use of long-term immunosuppressive agents in patients with systemic necrotizing vasculitides has dramatically improved the overall prognosis, but expose patients to potential severe adverse events. Also, pro-oncogenic effects of these drugs have been previously showed, but data on onco-hematological malignancies in this patient population are scarce. The aim of this study is to describe and analyse solid cancers and malignant hemopathies in patients with systemic necrotizing vasculitides.

Methods:

Data from 5 prospective, randomized, controlled trials conducted by the French Vasculitis Study Group (FVSG) (CHUSPAN 1, CHUSPAN 2, WEGENT, CORTAGE, MAINRITSAN), were pooled and analysed. These studies evaluated therapeutic strategy for the treatment of polyarteritis nodosa (PAN), granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA). Primary endpoint of this study was the occurrence of a solid cancer or a malignant hemopathy.

Results:

Seven hundred and thirty-three patients were included between 1993 and 2012, including 398 men (54.3%), with a median age of 60 years old (IQR 47-70). Vasculitis diagnoses were MPA in 231 (31.5%) patients, GPA in 226 (30.8%), GEPA in 186 (25.4%) and PAN in 85 (11.6%).

After a median follow-up of 5.2 years (IQR 3-9.7), 39 (5.3%) patients developed an onco-hematological complication, including solid cancer in 34 (4.6%) cases and malignant hemopathy in 5 (0.7%). Solid cancers included gastrointestinal cancers in 9 (26.5%), skin cancers in 8 (23.5%), pulmonary cancers in 6 (17.7%), urinary tract cancers in 5 (14.7%), gynecologic cancers in 4 (11.8%), and cerebral tumor and metastatic cancer of unknown origin in 1 (2.9%) case each. In one-third of patients, synchronous metastases were present at cancer diagnosis. Malignant hemopathies included myelodysplastic syndrome in 2 cases, and multiple myeloma, myeloproliferative syndrome and kidney lymphoma in 1 case each. Proportion of patients experiencing onco-hematological complications decreaed over time: 7.7% for patients included between 1993-1999, 6.3% between 2000-2005 and 3% between 2006-2012. Immunosuppressive agents that patients received prior the occurrence of solid cancer or malignant hemopathy were cyclophosphamide in 26 (66.7%), azathioprine in 18 (46.2%), methotrexate in 10 (25.6%), and mycophénolate mofetil and rituximab in 3 (7.7%) cases each.

Median time from treatment initiation to onco-hematological malignancies was 4.1 years (IQR 1.4-7.9). Finally, 19/39 patients died because of their onco-hematological complications, representing 16.7% of all causes of death.

Conclusion:

Onco-hematological complications represent a rare complication in systemic necrotizing vasculitides with a decreasing frequency over the last decades. These complications occur lately after vasculitis diagnosis, stressing the importance of thinking about it in patients in remission. Although rare, onco-hematological complications account for almost 20% of causes of deaths. Statistical analysis will enable us to identify patients at risk of such complications.

Disclosure: L. Antoine, None; C. Pagnoux, None; X. Puéchal, None; M. Samson, None; M. Hamidou, None; A. Karras, None; T. Quémeneur, None; M. Groh, None; L. Mouthon, None; L. Guillevin for the French Vasculitis Study Group, None; B. Terrier, None.

To cite this abstract in AMA style:

Antoine L, Pagnoux C, Puéchal X, Samson M, Hamidou M, Karras A, Quémeneur T, Groh M, Mouthon L, Guillevin for the French Vasculitis Study Group L, Terrier B. Onco-Hematological Malignancies in Systemic Necrotizing Vasculitides: Pooled Analysis of Five Prospective, Randomized, Controlled Trials [abstract]. Arthritis Rheumatol. 2017; 69 (suppl 10). https://acrabstracts.org/abstract/onco-hematological-malignancies-in-systemic-necrotizing-vasculitides-pooled-analysis-of-five-prospective-randomized-controlled-trials/. Accessed .

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