RESUMO
The aim of this study was to determine the characteristics, treatment, and outcome according to each etiology of pachymeningitis.We conducted a retrospective multicenter French nationwide study between 2000 and 2016 to describe the characteristics, outcome, and treatment of pachymeningitis.We included 60 patients (median age 55.5 years; interquartile range [IQR] 30-80, female/male ratio 0.43). Neurologic signs were present in 59 patients (98%) and consisted of headache in 43 (72%), cranial nerve palsy in 33 (55%), confusion in 10 (17%), seizures in 7 (12%), and focal neurologic signs in 9 (15%). Fever and weight loss were present in 8 (13%) and 13 cases (22%), respectively. Cerebral venous thrombosis was present in 8 cases (13%). Analysis of cerebrospinal fluid showed moderate hyperproteinorachia (median 0.68âg/L; IQR 0.46-3.2) with or without pleiocytosis. Diagnosis included idiopathic pachymeningitis (nâ=â18; 30%); granulomatosis with polyangiitis (nâ=â13; 17%); Erdheim-Chester disease (nâ=â10; 17%); IgG4-related disease and tuberculosis (nâ=â3; 5% each); Rosai-Dofman disease, microscopic polyangiitis, and sarcoidosis (nâ=â2, 3% each); cryptococcal meningitis, Lyme disease, ear-nose-throat infection, postlumbar puncture, low spinal-fluid pressure syndrome, and lymphoma (nâ=â1 each). We found no difference in demographics and neurologic presentation among idiopathic pachymeningitis, Erdheim-Chester disease, and granulomatosis with polyangiitis. In contrast, frequencies were lower with idiopathic pachymeningitis than Erdheim-Chester disease for general signs (6% and 40%, respectively, Pâ=â.041) and complete neurologic response (0% vs 39%, Pâ=â.045).The detection of extraneurologic signs and routine screening are needed to classify the pachymeningitis origin. Prospective studies are warranted to determine the best treatment in each case.
Assuntos
Granulomatose com Poliangiite , Meningite , Proteínas do Líquido Cefalorraquidiano/análise , Diagnóstico Diferencial , Gerenciamento Clínico , Feminino , França/epidemiologia , Granulomatose com Poliangiite/diagnóstico , Granulomatose com Poliangiite/epidemiologia , Humanos , Masculino , Meningite/diagnóstico , Meningite/epidemiologia , Meningite/fisiopatologia , Meningite/terapia , Pessoa de Meia-Idade , Exame Neurológico/métodos , Estudos Retrospectivos , Avaliação de SintomasRESUMO
BACKGROUND: As intravenous immunoglobulins (IVIG) exhibit immunomodulatory and antifibrotic properties, they may be a relevant treatment for systemic sclerosis (SSc). The objectives of this work were thus to report on the efficacy and safety of IVIG in a population of SSc patients and to review the available literature. METHODS: 46 patients from 19 French centers were retrospectively recruited. They were included if they had a diagnosis of SSc and received at least 1 IVIG infusion at a dosage >1g/kg/cycle. Relevant data collected at IVIG discontinuation were compared to those collected at IVIG initiation. A comprehensive literature review was performed. RESULTS: We observed a significant improvement of muscle pain (74% vs. 20%, p<0.0001), muscle weakness (45% vs. 21%, p=0.01), joint pain (44% vs. 19%, p=0.02), CK levels (1069±1552UI vs. 288±449UI, p<0.0001) and CRP levels (13.1±17.6mg/L vs. 9.2±16.6mg/L, p=0.001). We also noted a trend for an improvement of gastro-esophageal reflux disease (68% vs. 53%, p=0.06) and bowel symptoms (42% vs. 27%, p=0.06). Skin and cardiorespiratory involvements remained stable. Finally, corticosteroid daily dose was significantly lower by the end of treatment (13.0±11.6mg/day vs. 8.9±10.4mg/day, p=0.01). Only two severe adverse events were reported (one case of deep vein thrombosis and one case of diffuse edematous syndrome). CONCLUSION: Our work suggests that IVIG are a safe therapeutic option that may be effective in improving musculoskeletal involvement, systemic inflammation, digestive tract symptoms and could be corticosteroid sparing.
Assuntos
Imunoglobulinas Intravenosas/uso terapêutico , Escleroderma Sistêmico/tratamento farmacológico , Estudos de Coortes , Feminino , França , Humanos , Imunoglobulinas Intravenosas/farmacologia , Pessoa de Meia-Idade , Estudos Retrospectivos , Escleroderma Sistêmico/patologia , Resultado do TratamentoAssuntos
Arterite de Células Gigantes/complicações , Doenças da Língua/complicações , Doenças da Língua/diagnóstico , Língua/patologia , Idoso de 80 Anos ou mais , Feminino , Arterite de Células Gigantes/diagnóstico , Arterite de Células Gigantes/patologia , Humanos , Necrose/diagnóstico , Doenças da Língua/patologiaAssuntos
Antidepressivos de Segunda Geração/efeitos adversos , Citalopram/efeitos adversos , Depressão/tratamento farmacológico , Dermatomiosite/induzido quimicamente , Hiperlipidemias/tratamento farmacológico , Pravastatina/efeitos adversos , Idoso , Antidepressivos de Segunda Geração/administração & dosagem , Doença Hepática Induzida por Substâncias e Drogas/complicações , Doença Hepática Induzida por Substâncias e Drogas/diagnóstico , Citalopram/administração & dosagem , Depressão/complicações , Dermatomiosite/diagnóstico , Feminino , Humanos , Hiperlipidemias/complicações , Hipolipemiantes/administração & dosagem , Hipolipemiantes/efeitos adversos , Pravastatina/administração & dosagemAssuntos
Lúpus Eritematoso Sistêmico/diagnóstico , Pericardite/diagnóstico , Artrite/diagnóstico , Artrite/etiologia , Diagnóstico Diferencial , Feminino , Humanos , Lúpus Eritematoso Sistêmico/complicações , Pericardite/complicações , Radiografia Torácica , Tomografia Computadorizada por Raios X , Ultrassonografia , Adulto JovemRESUMO
A 56-year-old woman with ankylosing spondylitis, treated for 3 months with infliximab, developed miliary tuberculosis with mediastinal lymphadenopathies and brain and splenic lesions. After initial improvement under anti-tuberculous therapy, she suffered an unexpectedly prolonged paradoxical worsening with several episodes of lymphadenopathy, including life-threatening ones, over a period of more than 14 months of follow-up. The outcome was favorable as a result of corticosteroid and surgical treatments. This phenomenon reflects a paradoxical reaction precipitated by infliximab withdrawal.