Poor-Prognosis Factors of Systemic Vasculitides with Gastrointestinal Involvement: Data from a Large Retrospective Study

Segolene Gendreau¹, Benjamin Thoreau ², François Maurier ³, Tiphaine Goulenok ¹, Laure Frumholtz ¹, Arsène Mékinian ⁴, Saskia Oro ⁵, Charlotte Bernigaud ⁵, Alexandra Audemard ¹, Maxime Samson ⁶, Antoine Gaillet ¹, Romain Sonneville ¹, Jean-Benoit Arlet ¹, Adrien Mirouse ¹, Jean-Emmanuel Kahn ⁷, Aurélie Hummel ¹, Thomas Pires ⁸, Wendy Jourde ⁸, Eric Hachulla ⁹, Pierre-Louis Carron ¹⁰, Cécile-Audrey Durel ¹¹, Francoise Sarrot-Reynauld ¹², Nathalie Tieulie ¹³, Elisabeth Diot ¹⁴, Christian Agard ¹⁵, Guillaume Armengol ¹⁶, Alice Berezne ¹⁷, Christophe Deligny ¹⁸, Fleur Cohen ¹, Stanislas Faguer ¹⁹, Guillaume Le Guenno ²⁰, Mohamed Hamidou ¹⁵, Loïc Guillevin ²¹ and Benjamin Terrier ²¹, ¹APHP, Paris, France, ²CHU Tours, Tours, France, ³Service de Médecine Interne, Hôpital Belle Isle, Metz, Metz, France, ⁴Hôpital Saint-Antoine, APHP, Paris, Paris, France, ⁵APHP, Creteil, France, ⁶Service de Médecine Interne et Immunologie Clinique, CHU Dijon Bourgogne, Hôpital François Mitterrand, Dijon ; Université Bourgogne-Franche Comté, INSERM, EFS BFC, UMR1098, F-21000 Dijon, Dijon, France, ⁷APHP, Boulogne Billancourt, France, ⁸CHU Bordeaux, Bordeaux, France, ⁹CHU Lille, Lille, France, ¹⁰Grenoble, Grenoble, France, ¹¹Department of Internal Medicine, Hôpital Edouard Herriot, Lyon, France, Lyon, France, ¹²CHU Grenoble, Grenoble, France, ¹³University Hospital of Nice, Nice, France, ¹⁴APHP, Tours, France, ¹⁵CHU Nantes, Nantes, France, ¹⁶CHU Rouen, Rouen, France, ¹⁷CH Annecy Genevois, Annecy, France, ¹⁸CHU Martinique, Fort de France, France, ¹⁹CHU Toulouse, Toulouse, France, ²⁰CHU Clermont Ferrand, Clermont Ferrand, France, ²¹National Referral Center for Rare Systemic Autoimmune Diseases Paris Cochin, Paris, France

Meeting: 2019 ACR/ARP Annual Meeting

Keywords: gastrointestinal and prognostic factors, systemic necrotizing vasculitis

Session Information

Date: Monday, November 11, 2019

Title: Vasculitis – Non-ANCA-Associated & Related Disorders Poster III: Behçet’s Disease & Other Vasculitides

Session Type: Poster Session (Monday)

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

Background/Purpose: Gastrointestinal (GI) involvement was described as a poor-prognosis factor of systemic necrotizing vasculitides. However, the prognostic impact of GI involvement may vary according to clinical manifestation and vasculitis subtype. We aimed to describe initial symptoms and outcomes of GI involvement of vasculitides and identify factors predictive of poor outcomes among systemic vasculitides.

Methods: Patients with systemic vasculitides as defined by the 2012 Chapel Hill Consensus Conference and with initial GI involvement were retrospectively included. Baseline characteristics, treatments received and outcomeswere recorded. The primary endpoint was a composite of intensive care unit (ICU) admission, emergency surgical procedure, GI-related sepsis or death.

Results: Among the 191 patients diagnosed between 2006 and 2019 included (67% men, mean±SD age 50±19 years), vasculitides were distributed as follows: 75 IgA vasculitis, 50 ANCA-associated vasculitis, 35 polyarteritis nodosa (PAN), 9 large-vessel vasculitis and 22 other vasculitides (including Behçet’s disease). Seventy-four (39%) patients reached the composite primary endpoint after a median [IQR] of 10 [0–32] days.Sixty (31%) patients were admitted to the ICU, 34 (18%) required emergency surgery, 30 (16%) had GI-related sepsis. Twelve (6%) patients died of a GI cause after a median of 41 [24–68] days. Vasculitis subtype was associated with the primary endpoint: PAN diagnosis was associated with a poor outcome (hazards ratio [HR] 2.21 (95% CI 1.36–3.62); P< 0.001), while IgA vasculitis had a better outcome (HR 0.32 (95% CI 0.18–0.58); P< 0.001).
Baseline characteristics associated with the primary endpoint [HR (95% CI)] included: age [1.02 (1.0–1.03); P=0.007], fever [2.52 (1.56–4.08); P< 0.001], abdominal pain requiring morphine [3.05 (1.74–5.34); P< 0.001], abdominal guarding [2.76 (1.53–4.98); P=0.001], ileus [3.1 (1.79–5.36); P=0.001], melena [2.30 (1.33–3.98); P=0.003]and intestinal ischemia [3.95 (2.14–7.28); P< 0.001]. High serum C-reactive protein level, WBC count, lactate dehydrogenase levels, performance status, in-hospital admission SOFA score, low oxygen saturation, and hemoglobin and bicarbonate levels were associated with poor outcomes. Endoscopy-identified mucosal abnormalities were not associated with the primary outcome.
To identify patients requiring ICU admission, emergency surgery, GI-related sepsis or death, a score predictive of poor outcome is being developed.

Conclusion: Vasculitides with initial GI involvement, mainly PAN, had poor outcomes for >30% of the patients. We identified a set of clinical and biological factors present at diagnosis associated with this poor outcome. We are devising a predictive score based on these variables to identify patients requiring early supportive care.

Disclosure: S. Gendreau, None; B. Thoreau, None; F. Maurier, None; T. Goulenok, None; L. Frumholtz, None; A. Mékinian, None; S. Oro, None; C. Bernigaud, None; A. Audemard, None; M. Samson, None; A. Gaillet, None; R. Sonneville, None; J. Arlet, None; A. Mirouse, None; J. Kahn, None; A. Hummel, None; T. Pires, None; W. Jourde, None; E. Hachulla, None; P. Carron, None; C. Durel, None; F. Sarrot-Reynauld, None; N. Tieulie, None; E. Diot, None; C. Agard, None; G. Armengol, None; A. Berezne, None; C. Deligny, None; F. Cohen, None; S. Faguer, None; G. Le Guenno, None; M. Hamidou, Roche, 8; L. Guillevin, None; B. Terrier, Grifols, 8, GSK, 8, LFB, 8, Roche, 8.

To cite this abstract in AMA style:

Gendreau S, Thoreau B, Maurier F, Goulenok T, Frumholtz L, Mékinian A, Oro S, Bernigaud C, Audemard A, Samson M, Gaillet A, Sonneville R, Arlet J, Mirouse A, Kahn J, Hummel A, Pires T, Jourde W, Hachulla E, Carron P, Durel C, Sarrot-Reynauld F, Tieulie N, Diot E, Agard C, Armengol G, Berezne A, Deligny C, Cohen F, Faguer S, Le Guenno G, Hamidou M, Guillevin L, Terrier B. Poor-Prognosis Factors of Systemic Vasculitides with Gastrointestinal Involvement: Data from a Large Retrospective Study [abstract]. Arthritis Rheumatol. 2019; 71 (suppl 10). https://acrabstracts.org/abstract/poor-prognosis-factors-of-systemic-vasculitides-with-gastrointestinal-involvement-data-from-a-large-retrospective-study/. Accessed .

« Back to 2019 ACR/ARP Annual Meeting

ACR Meeting Abstracts - https://acrabstracts.org/abstract/poor-prognosis-factors-of-systemic-vasculitides-with-gastrointestinal-involvement-data-from-a-large-retrospective-study/