Session Type: Abstract Session
Session Time: 3:00PM-4:30PM
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.
To cite this abstract in AMA style:ROHMER J, trefond l, Nguyen Y, Agard C, ALLAIN J, Berezne A, Charles P, Cohen P, gondran g, GROH M, HUSCENOT T, lacout c, Lazaro E, London J, Maurier F, Mekinian A, Nubourgh I, MESBAH R, Puéchal X, Perard L, Puyade M, Pugnet G, Queyrel V, Rouzaud D, Roux A, DUREL C, Guillevin L, Terrier B. Clinical Features and Long-term Outcomes of Patients with Systemic Polyarteritis Nodosa Diagnosed Since 2005: Data from 196 Patients [abstract]. Arthritis Rheumatol. 2022; 74 (suppl 9). https://acrabstracts.org/abstract/clinical-features-and-long-term-outcomes-of-patients-with-systemic-polyarteritis-nodosa-diagnosed-since-2005-data-from-196-patients/. Accessed December 1, 2022.
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