Meningeal Involvement in Wegener Granulomatosis: Case Report and Review of the Literature

Abstract

Wegener Granulomatosis (WG) is a multisystem autoimmune disorder characterized by necrotizing granulomatous vasculitis that most commonly involves the upper respiratory tract, lungs, and kidneys. The involvement of the central nervous system (CNS) is infrequent and can cause stroke, cranial nerve abnormalities, cerebrovascular events, seizures, and meningeal involvement. Meningeal involvement is rare and may occur due to local vasculitis, directly spread from adjacent disease in the skull base, paranasal or orbital region. We describe the case ofa 20-year-old Caucasian man who was diagnosed with sinonasal WG with frontal focal meningeal involvement. A literature review on diagnosis and treatment of meningeal involvement in course of WG was carried out. The importance of an early diagnosis and treatment of localized WG has been emphasized, in order to avoid the progression to a severe form of disease, especially in younger patients and in paucisymptomatic cases. Case reportA twenty-year-old Caucasian man with history of parietaria allergy presented with nasal congestion, occasional epistaxis and purulent rhinorrhea that had started 1 year previously. He had been seen at another hospital and had received a diagnosis of allergic rhinosinusitis. Large doses of intranasal steroids and multiple courses ofantibiotic therapy did not result in an improvement. An X-ray of the paranasal sinuses carried out six months previously was negative. His familial history was unremarkable; his parents were both living and well; the patient was the only child, he was a medical student at University in his own city, he was single and lived with his parents. He had no allergies to medications, and he denied use of alcohol, illicit drugs and smoking.On admission to our Department, headache, neck stiffness, dyspnoea, cough, fever, night sweats, fatigue, myalgia or arthralgia were absent. On examination, the patient was alert, oriented, and cooperative. There was nasal congestion and copious discharge. The lungs were clear on the auscultation, the cardiac and abdominal examinations were normal. Neurologic examination was negative for focal motor or sensory deficits as well as examination of cranial nerves II through XII. A complete blood count revealed a white-cell count of 8,430 per cubic millimeter, with 46% polymorphonuclear cells. The hematocrit was 41%, and the platelet count 296,000 per cubic millimeter. The blood urea nitrogen level was 27 mg per deciliter, and the creatinine level 0.70 mg per deciliter. Erythrocyte sedimentationrate (ESR)

Central Nervous System Involvement in Pediatric Systemic Autoimmune Diseases

“…167 Case reports have also described the use of rituximab for CNS disease in GPA. 168,169 Hashimoto et al's adult clinical trial revealed potential use of plasmapheresis in GPA and EGPA, 29 and a pediatric case report demonstrated neurologic response to cyclophosphamide and plasmapheresis. 158 However, an adult randomized controlled trial reported that combined treatment with corticosteroid, cyclophosphamide, and plasmapheresis was not superior to corticosteroid and cyclophosphamide alone.…”

Management of primary and secondary central nervous system vasculitis

“…The frequency of CNS involvement has been described in 0-10% of patients with granulomatosis with polyangiitis (GPA, formerly Wegener's granulomatosis) [25,26], Five percent of those with eosinophilic granulomatosis with polyangiitis (EGPA, also known as Churg-Strauss syndrome) [27], and 11% of those with microscopic polyangiitis (MPA) [28]. AAV-related CNS manifestations encompass meningitis, pituitary gland involvement and focal neurologic findings; CNS lesions are usually ischemic, but can also be granulomatous (in GPA) or hemorrhagic [29][30][31][32]. As a rule, CNS involvement secondary to vasculitis is considered a severe manifestation carrying a significant risk of morbidity [33] and mortality [33,34].…”

Поражение Опорно-Двигательного Аппарата При Узелковом Полиартериите

“…Поражение суставов и периартикулярных тканей является одним из основных признаков всех СВ [12]. Сейчас широко обсуждаются изменения опорно-двигательного аппарата при мелкососудистых СВ, ассоциированных с антинейтрофильными цитоплазматическими антителами, -микроскопическом полиангиите [13], гранулематозе с полиангиитом Вегенера [10,11], эозинофильном гранулематозном полиангиите Черджа -Стросса [3,9]. Такие исследования при среднесосудистом узелковом полиартериите (УПА) отсутствуют [4,6].…”