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<p>Primary angiitis of the central nervous system (PACNS) is a&#xa0;very rare form of organ-specific vasculitis of the central nervous system. Patients usually present with either nonspecific neurological symptoms such as headache or cognitive impairment, or with focal neurological deficits. In MRI, focal or diffuse cortical or subcortical hyperintensities, bleedings, and focal meningeal contrast enhancements are seen. If PACNS is suggested clinically or by MRI, a&#xa0;thorough investigation of cerebrospinal fluid and blood is warranted to rule out infection, autoimmune disease, or malignancy. Magnetic resonance angiography and, subsequently, digital subtraction angiography can detect typical string-of-beads signs of vasculitis of large- and medium-sized arteries. If no signs of vasculitis can be visualized on imaging, biopsy of a&#xa0;lesion suspicious in MRI is recommended. Histologically, granulomatous, lymphocytic, or necrotic lesions of different-sized arteries are found. Important differential diagnoses include reversible cerebral vasoconstriction syndrome, posterior reversible encephalopathy syndrome, and systemic vasculitis. Once PACNS has been diagnosed, intravenous methylprednisolone pulse therapy is administered as first-line treatment. If medium-sized or large vessels are involved or necrotizing or granulomatous vasculitis is present, induction therapy with cyclophosphamide is added. After clinical improvement, the glucocorticoid dose should be tapered, and cyclophosphamide therapy should be switched to maintenance therapy with either mycophenolate mofetil or azathioprine for at least 2&#xa0;years. In refractory disease, immunosuppressive therapy with either rituximab, tocilizumab, or tumor necrosis factor blockers may be considered.</p>

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Primäre Angiitis des zentralen Nervensystems

  • 期刊论文

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  • 发表日期：

    2026年7月28日

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  • Josef Hermann,
  • Thomas Seifert-Held

摘要

Primary angiitis of the central nervous system (PACNS) is a very rare form of organ-specific vasculitis of the central nervous system. Patients usually present with either nonspecific neurological symptoms such as headache or cognitive impairment, or with focal neurological deficits. In MRI, focal or diffuse cortical or subcortical hyperintensities, bleedings, and focal meningeal contrast enhancements are seen. If PACNS is suggested clinically or by MRI, a thorough investigation of cerebrospinal fluid and blood is warranted to rule out infection, autoimmune disease, or malignancy. Magnetic resonance angiography and, subsequently, digital subtraction angiography can detect typical string-of-beads signs of vasculitis of large- and medium-sized arteries. If no signs of vasculitis can be visualized on imaging, biopsy of a lesion suspicious in MRI is recommended. Histologically, granulomatous, lymphocytic, or necrotic lesions of different-sized arteries are found. Important differential diagnoses include reversible cerebral vasoconstriction syndrome, posterior reversible encephalopathy syndrome, and systemic vasculitis. Once PACNS has been diagnosed, intravenous methylprednisolone pulse therapy is administered as first-line treatment. If medium-sized or large vessels are involved or necrotizing or granulomatous vasculitis is present, induction therapy with cyclophosphamide is added. After clinical improvement, the glucocorticoid dose should be tapered, and cyclophosphamide therapy should be switched to maintenance therapy with either mycophenolate mofetil or azathioprine for at least 2 years. In refractory disease, immunosuppressive therapy with either rituximab, tocilizumab, or tumor necrosis factor blockers may be considered.