A Case of Polyarteritis Nodosa Associated with Vertebral Artery Vasculitis Treated Successfully with Tocilizumab and Cyclophosphamide

Abstract

Pediatric polyarteritis nodosa is rare systemic necrotizing arteritis involving small- and medium-sized muscular arteries characterized by aneurysmal dilatations involving the vessel wall. Aneurysms associated with polyarteritis nodosa are common in visceral arteries; however intracranial aneurysms have also been reported and can be associated with central nervous system symptoms, significant morbidity, and mortality. To our knowledge extracranial involvement of the vertebral arteries has not been reported but has the potential to be deleterious due to fact that they supply the central nervous system vasculature. We present a case of a 3-year-old Haitian boy with polyarteritis nodosa that presented with extracranial vessel involvement of his vertebral arteries. After thorough diagnostic imaging, including a bone scan, ultrasound, Magnetic Resonance Imaging/Angiography, and Computed Tomography Angiography, he was noted to have vertebral artery vasculitis, periostitis, subacute epididymoorchitis, arthritis, and myositis. He met diagnostic criteria for polyarteritis nodosa and was treated with cyclophosphamide, methylprednisolone, and tocilizumab, which resulted in improvement of his inflammatory markers, radiographic findings, and physical symptoms after treatment. To the authors' knowledge, this is the first report of vertebral artery vasculitis in polyarteritis nodosa as well as successful treatment of the condition using the combination cyclophosphamide and tocilizumab for this condition.

The Two-Faced Cytokine IL-6 in Host Defense and Diseases

“…Case reports have shown TCZ to be effective for a variety of other diseases, including autoimmune hemolytic anemia [ 171 , 172 ], reactive arthritis [ 173 ], autoimmune encephalitis [ 174 , 175 ], myasthenia gravis [ 176 ], Blau syndrome [ 177 ], anti-Caspre2 syndrome [ 178 ], refractory organizing pneumonia associated with Sjögren’s syndrome [ 179 ], cancer-related cachexia [ 180 ] and steroid refractory immune-mediated adverse events secondary to immune check point inhibitors [ 181 ]. Besides large vessel vasculitis, several case reports have indicated that TCZ has effects on rheumatoid vasculitis [ 182 ], polyarteritis nodosa [ 183 , 184 ], microscopic polyangiitis [ 185 , 186 ] and cryoglobulinemia vasculitis [ 187 ]. A report showed that epidural administration of TCZ reduced pain due to sciatica with lumbar spinal stenosis [ 188 ].…”

Childhood polyarteritis nodosa: A rare presentation

“…4 Delayed or no treatment in PAN, may lead to significant morbidity and mortality. 6 In our case, he is continuously on treatment and follow-up since childhood. Corticosteroids are mainstay of treatment on PAN.…”

Use of Anti TNF-α Therapy in Systemic Vasculitis

“…Few cases were treated with etanercept [65][66][67], one with infliximab [68], tocilizumab [69], adalimumab [70] and lastly, a case treated with rituximab [71]. All cases showed a good response to treatment with achieved remission upon clinical and biochemical basis without serious side effects.…”