Terapêutica intravenosa com metilprednisolona e ciclofosfamida na vasculite do sistema nervoso periférico: avaliação de oito pacientes

Abstract

RESUMO -O comprometimento do sistema nervoso periférico é frequente nas vasculites sistêmicas e contribui decisivamente para o diagnóstico. Oito pacientes foram estudados: sete do sexo feminino e um do sexo masculino; cinco brancos, dois negros, um amarelo; média de idade de 55,9 anos; quatro com poliarterite nodosa, um com lúpus eritematoso sistêmico, um com artrite reumatóide, um com vasculite isolada do sistema nervoso periférico e um com vasculite livedóide. Todos foram submetidos a terapêutica intravenosa com pulsos mensais de metilprednisolona (1 g/dia/3dias) e ciclofosfamida (1 g/dia). Cinco pacientes melhoraram, dois permaneceram inalterados e um faleceu. A melhora neurológica objetiva ocorreu após o terceiro ou quarto pulso e nos pacientes com menor duração da doença.PALAVRAS-CHAVE: vasculite do sistema nervoso periférico, vasculite sistêmica, terapêutica intravenosa imunossupressora, pulsoterapia. Intravenous theraphy with methylprednisolone and cyclophosphamide in vasculitis of peripheral nervous system: evaluation of eight patientsABSTRACT -The peripheral nervous system is frequently involved in systemic vasculitis and it may be helpful in the disease diagnosis. We report on eight patients: seven women and one man; five white, two black and one yellow; age mean 55.9 years; four had polyarteritis nodosa, one had systemic lupus erythematosus, one had isolated peripheral nerve vasculitis and one had livedoid vasculitis. All of them received endovenous therapy with "pulse" of methylprednisolone (1 g/day/3days) and cyclophosphamide (1 g/1day). Five patients improved, two remained unchanged and one died. The neurological improvement occurred after the third or fourth pulse and in the patients who have had a shorter time of disease.KEY WORDS: vaculitis of peripheral nervous system, systemic vasculitis, intravenous immunosuppressor therapy, pulsetherapy.

Primary perineuritis, a rare but treatable neuropathy: Review of perineurial anatomy, clinicopathological features, and differential diagnosis

“…While perineuritis is not documented as a histopathological feature in most series describing vasculitic neuropathies, in one of the largest and most influential studies, perineurial inflammation was “common” near arterial lesions in patients with SVN and NSVN 7 . There are case reports of perineuritis in patients with NSVN, hepatitis B‐related SVN, and cryoglobulinemic vasculitic neuropathy 6,102–105 . In one study comparing sural nerve pathology in 21 patients with neuroborreliosis‐associated neuropathy, nine with SVN or NSVN, and 14 with non‐inflammatory neuropathies, perineurial T cells were significantly increased in both the neuroborreliosis and vasculitis groups, and perineurial macrophages were most prevalent in those with vasculitis 95 .…”