Severe Mononeuritis Multiplex due to Rheumatoid Vasculitis in Rheumatoid Arthritis in Sustained Clinical Remission for Decades

Abstract

Rheumatoid vasculitis (RV) usually occurs in patients with refractory rheumatoid arthritis (RA). An 80year-old woman was transferred to our hospital because of muscle weakness and paresthesia in all 4 limbs. She had been diagnosed with RA 30 years ago and achieved sustained clinical remission. At presentation, polyarthritis and drop foot were observed, and rheumatoid factor was prominently elevated. A peripheral nerve conduction test revealed mononeuritis multiplex in her limbs. We suspected that RV had developed rapidly despite RA having been stable for many years and started immunosuppression therapy with steroids combined with azathioprine. The treatment prevented worsening of muscle weakness and paresthesia.

Mononeuropatía múltiple en fase avanzada como manifestación de rhupus:

“…Así mismo, se ha descrito compromiso neurológico en LES y AR como entidades independientes, entre ellas hallazgos de mononeuritis múltiple, como en este caso (3,4,9). Fisiopatológicamente, se ha propuesto que la presencia de neuropéptidos (sustancia P, péptido relacionado con el gen de la calcitonina) y citocinas proinflamatorias (IL-1β, IL-6, TNF-α), así como la acción directa de autoanticuerpos, dañan estructuras del sistema nervioso periférico (3).…”

“…Fisiopatológicamente, se ha propuesto que la presencia de neuropéptidos (sustancia P, péptido relacionado con el gen de la calcitonina) y citocinas proinflamatorias (IL-1β, IL-6, TNF-α), así como la acción directa de autoanticuerpos, dañan estructuras del sistema nervioso periférico (3). De forma interesante, se ha descrito neuropatía asociada con procesos vasculíticos tanto en LES como en AR, tras varios años de evolución (3,4,9).…”

“…Desde 1971 se describió el rhupus como la presencia concomitante de artritis reumatoide (AR) y lupus eritematoso sistémico (LES), al reconocerse las implicaciones clínicas y pronósticas diferentes a las de estas patologías de forma independiente (1,2). Si bien en esta población el compromiso neurológico severo es raro y de difícil abordaje diagnóstico, conlleva serias manifestaciones clínicas y en la calidad de vida (2)(3)(4). En la literatura sobre el tema hay únicamente tres casos de mononeuritis múltiple en pacientes con rhupus (1,2).…”

Acute Neurologic Manifestations of Systemic Immune-Mediated Diseases

“…Central nervous system (CNS) ischemia and hemorrhage may occur from vasculitis, chronic systemic inflammation, or conventional risk factors (such as dyslipidemia from steroid use). 17 CNS rheumatoid vasculitis can happen in isolation, but it usually occurs in conjunction with systemic vasculitis. 18 Like with other forms of CNS vasculitis, patients often have headache and encephalopathy in addition to focal signs.…”

Hypoglossal Nerve Palsy Following COVID-19 Vaccination in a Young Adult Complicated by Various Medicines

“…Among autoimmune causes, small-to-medium-sized vasculitis, such as an antineutrophil cytoplasmic antibody (ANCA)-related vasculitis and Sjogren’s syndrome, are common [ 1 , 2 ]. The progression of mononeuritis multiplex symptoms varies depending on the human body's etiology and immunological reactions [ 3 , 4 ]. Severe cases may involve multi-extremity paralysis, which should be treated with intravenous immunoglobulin therapy, steroids, and plasma exchange, according to the etiology [ 2 , 5 ].…”