Case reports in rheumatology · 2026
Addition of Intravenous Immunoglobulin in Refractory Cutaneous Polyarteritis Nodosa: A Case Report.
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Kohlmann Fabio, Odehnal Daniela, Buchtala Laura, Kernder Anna, Baraliakos Xenofon
Аннотация
BACKGROUND: Cutaneous polyarteritis nodosa (cPAN) is a rare necrotizing vasculitis affecting medium-sized skin vessels and may be refractory to standard immunosuppressive therapies.
CASE PRESENTATION: We report a 23-year-old male with HLA-B27-positive enthesitis-associated arthritis (ERA), which was reclassified during the disease course and is currently in clinical remission under interleukin-17 inhibition, and reading epilepsy, presenting with histologically confirmed cPAN. Despite multiple treatments, including methotrexate, corticosteroid pulses, tumor necrosis factor-α (TNF-α) inhibitors, and tocilizumab, the patient developed persistent painful livedo reticularis-like lesions with palpable subcutaneous nodules and systemic symptoms. Intravenous immunoglobulin (IVIG) was initiated at 1.5 g/kg per cycle (administered over three consecutive days; total dose ∼100 g per cycle) and repeated every eight weeks, followed by a clinically meaningful response after the first cycle and complete resolution of symptoms within one month.
CONCLUSION: The addition of IVIG may represent an effective escalation option for refractory cPAN. Further studies are needed to clarify its role in this rare vasculitis.
CASE PRESENTATION: We report a 23-year-old male with HLA-B27-positive enthesitis-associated arthritis (ERA), which was reclassified during the disease course and is currently in clinical remission under interleukin-17 inhibition, and reading epilepsy, presenting with histologically confirmed cPAN. Despite multiple treatments, including methotrexate, corticosteroid pulses, tumor necrosis factor-α (TNF-α) inhibitors, and tocilizumab, the patient developed persistent painful livedo reticularis-like lesions with palpable subcutaneous nodules and systemic symptoms. Intravenous immunoglobulin (IVIG) was initiated at 1.5 g/kg per cycle (administered over three consecutive days; total dose ∼100 g per cycle) and repeated every eight weeks, followed by a clinically meaningful response after the first cycle and complete resolution of symptoms within one month.
CONCLUSION: The addition of IVIG may represent an effective escalation option for refractory cPAN. Further studies are needed to clarify its role in this rare vasculitis.