A case of refractory polyarteritis nodosa successfully treated with rituximab

Abstract

A 59-year-old man who presented with continuous fever, livedo reticularis, and left leg ischemia with multiple tibial artery stenosis and renal artery aneurysm, as demonstrated by arteriography, was diagnosed with polyarteritis nodosa (PAN) 6 years ago. Although he frequently relapsed in spite of intensive immunosuppressive therapies, the disease activity of PAN was controlled with repeated rituximab (RTX) therapies and steroid doses were tapered safely. Peripheral CD19 B-cells disappeared soon after the 1st administration of RTX. Although CD19 B-cells remained absent, 3.1% of CD3CD20 T-cells were observed in the peripheral blood prior to the 2nd administration of RTX. Recent studies have suggested the pathogenic role of CD3CD20 T-cells in autoimmune diseases in the context of RTX therapy; therefore, their roles in the pathogenesis of PAN also need to be considered.

Sacroiliitis and Polyarteritis Nodosa in a Patient with Familial Mediterranean Fever

“…Methotrexate, azathioprine, or mycophenolate mofetil are used in maintenance treatment. While there are no randomized controlled studies on TNF alpha blockers and rituximab in refractory patients, there are publications reporting good responses to treatment [ 10 , 11 ]. In cases of hepatitis B-associated PAN, antiviral treatment and plasmapheresis are more commonly used in comparison to immunosuppressive agents [ 12 ].…”

Polyarteriitis Nodosa with Real Arteries and Vein Involvement and Uroligical Manifestations: Case Report

“…Patients with a FFS ≥ 1 should receive intermittent intravenous cyclophosphamide in addition to corticosteroids [26,27]. In the steroid-refractory and recurrent PAN rituximab and infliximab or plasma exchanges has been used occasionally [28][29][30]. As our patient had FFS 2 (proteinuria and elevated serum creatinine), after nephrectomy, which was indicated given the combination of renal artery aneurism and stenosis with peri-vasal hematoma, kidney shrinking and absence of kidney function, he was started on the combination of prednisone and cyclophosphamide.…”

“…Here we present a case of PAN with extremely severe combined kidney damage. 28 years old Caucasian man, admitted May 31 2017.…”

Orbital inflammatory disease management

“…In PAN not confined to the orbits, induction treatment has also been successfully carried out with rituximab [100,101] and infliximab [102] in separate, isolated case reports. With regard to maintenance therapy, azathioprine, methotrexate, and mycophenolate mofetil have been successfully utilized in smaller, non-blinded case series [94,103].…”