2232: Clinical Features and Long-term Outcomes of Patients with Systemic Polyarteritis Nodosa Diagnosed Since 2005: Data from 196 Patients

Julien ROHMER¹, ludovic trefond², Yann Nguyen³, Christian Agard⁴, Jean Sebastien ALLAIN⁵, Alice Berezne⁶, Pierre Charles⁷, Pascal Cohen⁸, guillaume gondran⁹, Matthieu GROH¹⁰, Tessa HUSCENOT¹¹, carole lacout¹², Estibaliz Lazaro¹³, Jonathan London¹⁴, Francois Maurier¹⁵, Arsene Mekinian¹⁶, Isabelle Nubourgh¹⁷, Rafik MESBAH¹⁸, Xavier Puéchal⁸, Laurent Perard¹⁹, Mathieu Puyade²⁰, Gregory Pugnet²¹, Viviane Queyrel²², Diane Rouzaud²³, Arthur Roux²⁴, Cecile-Audrey DUREL¹⁹, Loïc Guillevin⁸ and Benjamin Terrier⁸, ¹APHP, Suresnes, France, ²Chu clermont ferrand, Clermont-Ferrand, France, ³AP-HP.Centre Universit Paris Cit Hôpital Cochin, Montmorency, France, ⁴Internal medicine, Nantes University Hospital, Nantes, France, ⁵CHU rennes, Rennes, ⁶CH Annecy, Annecy, France, ⁷Institut Mutualiste Montsouris, Service de Médecine Interne, Paris, France, ⁸National Referral Center for Rare Systemic Autoimmune Diseases, Cochin Hospital, Paris, France, ⁹CHU limoges, Limoges, France, ¹⁰Foch Hospital, Suresnes, France, ¹¹APHP, Boulogne, France, ¹²CHU Angers, Angers, France, ¹³Bordeaux Hospital University, Bordeaux, France, ¹⁴Hôpital Croix-Saint-Simon, Paris, France, ¹⁵Hôpitaux privés de Metz, Metz, France, ¹⁶AP-HP, Hopital Saint Antoine, Paris, France, ¹⁷Ch St Pierre, Brussels, Belgium, ¹⁸Boulogne Ch, Boulogne, France, ¹⁹CHU Lyon, Lyon, France, ²⁰Centre Hospitalier Universitaire de Poitiers, Poitiers, France, ²¹CHU Toulouse Purpan Service de Medecine Interne, Toulouse, France, ²²CHU NIce, Nice, France, ²³Bichat, Paris, France, ²⁴MGEN, Paris, France

Background/Purpose: The etiological landscape of systemic polyarteritis nodosa (PAN) has substantially changed since the onset of hepatitis B virus (HBV) vaccination and the discovery of new entities such as adenosine deaminase 2 (ADA2) deficiency or PAN related to myelodysplastic syndrome (MDS) or hematological malignancies. Recent data regarding the current picture of systemic PAN, especially baseline characteristics, causes and predictors of outcomes are lacking.

Methods: We conducted a retrospective study including patients with systemic PAN referred to the French Vasculitis Study Group (FVSG) from 2005 to 2019. Clinical characteristics, associated conditions and long-term outcomes were collected in order to identify predictors of relapse and death.

Results: One hundred and ninety-seven patients with systemic PAN were included. One hundred and twenty-one (61.4%) patients were male and mean age was 53.62 (18.04) years. The main clinical manifestations at diagnosis were constitutional symptoms (84%), skin involvement (67%), musculoskeletal (58%) and neurological manifestations (54%), mainly multiple mononeuropathy. Five Factor Score 1996 was 0 in 55.8%. PAN secondary to an identified condition accounted for 28% of patients and were associated with MDS (9%), solid malignancy (7%), lymphoproliferative disorders (4%), autoinflammatory syndromes (4%) and hypereosinophilic syndrome (HES) (2.5%). No patient had active HBV infection at the time of PAN diagnosis.

Most patients (98%) received a first-line treatment based on glucocorticoids (GCs) alone in 41% or in combination with immunosuppressive agents in 57%. Assessable patients achieved vasculitis remission in 173 (90%) cases whereas 20 (10%) patients were non-responders.

After a median (IQR) follow-up of 43 (16-90) months, 76 (39%) patients experienced vasculitis relapse after a median time of 23 (8-45) months. Also, 25 (13%) patients died with 1-, 5- and 10-years overall survival rates being of 91%, 89% and 88% respectively.

In multivariable analysis, variables associated with an increased risk of relapse were the following: age >65 years (Hazard Ratio 1.85 (1.12-3.08, p=0.017)), severe gastrointestinal involvement (HR 1.95 (1.09-3.52, p=0.025)) and skin necrotic lesions (HR 1.95 (1.24-3.05, p=0.004)). Variables associated with an increased risk of death were the following: age >65 years (HR 2.80 (1.23-6.37, p=0.014)), necrotic purpura (HR 4.16 (1.62-10.70, p=0.003)), acute kidney injury (HR 4.89 (1.71-13.99, p=0.003)) and secondary PAN (HR 2.98 (1.29-6.85, p=0.010)).

Conclusion: Although widespread HBV vaccination led to a dramatic decrease of HBV-related PAN, 28% of systemic PAN in France remain associated with another condition, mainly myelodysplastic syndrome and solid malignancies. The rate of relapse remains high, especially in patients with gastrointestinal involvement and skin necrotic lesions. Same variables as well as secondary forms of PAN were associated with increased mortality.

Disclosures: J. ROHMER, None; l. trefond, None; Y. Nguyen, None; C. Agard, None; J. ALLAIN, None; A. Berezne, None; P. Charles, None; P. Cohen, Roche; g. gondran, None; M. GROH, None; T. HUSCENOT, None; c. lacout, None; E. Lazaro, None; J. London, None; F. Maurier, None; A. Mekinian, None; I. Nubourgh, None; R. MESBAH, None; X. Puéchal, Roche; L. Perard, None; M. Puyade, None; G. Pugnet, None; V. Queyrel, None; D. Rouzaud, None; A. Roux, None; C. DUREL, None; L. Guillevin, Roche; B. Terrier, AstraZeneca, GlaxoSmithKline, Bristol-Myers Squibb(BMS), Eli Lilly, LFB, Boehinger Ingelheim, Vifor Pharma, Pfizer, Roche.