Antiphospholipid Vasculopathy or Polyarteritis Nodosa – Case of „Both“ or „Either“

Abstract

Background: Polyarteritis nodosa is a systemic necrotizing vasculitis affecting medium-sized arteries and, less commonly, small-caliber vessels. In the differential diagnostic context, one of the conditions that may present with a similar clinical picture is vasculopathy associated with antiphospholipid syndrome. Case presentation: We present the clinical case of a 62-year-old female patient whose symptoms began approximately three months prior to her first hospitalization in the Rheumatology Clinic. The initial manifestations included gradual appearance of a rash on the lower extremities in the form of livedo reticularis/purpura retiforme, progressing to ulcerations, accompanied by pain in the lower limbs. Following outpatient assessment the patient was admitted to the Rheumatology Clinic. After completion of a stepwise diagnostic workup, a diagnosis of polyarteritis nodosa was established based on the ACR criteria. Immunological testing demonstrated a tenfold elevation of anticardiolipin antibodies. According to the 2023 EULAR classification criteria, the patient fulfilled the required criteria for antiphospholipid syndrome/antiphospholipid vasculopathy. Pulse therapy treatment was initiated consisting of methylprednisolone and cyclophosphamide, in combination with vasodilator and anticoagulant therapy. After four months, a favorable therapeutic response was observed. However, due to secondary infection of the ulcerations, surgical intervention became necessary, along with reduction of the immunosuppressive therapy. During the subsequent follow-up period corticosteroid therapy was successfully discontinued completely, and sustained remission without new relapses was achieved. Conclusion: An open question remains regarding the extent of overlap between the two nosological entities and whether the positive antiphospholipid antibodies may represent merely an immunological phenomenon occurring in the setting of active vasculitis.