081. SPONTANEOUS SPINAL SUBDURAL HEMATHOMA AS A MANIFESTATION OF ANCA-ASSOCIATED VASCULITIS

Abstract

Background: Spontaneous spinal subdural hematomas (SSHs) are rare, only 120 cases were found in a review published in 2017 (de BeerMH, 2017). Five out these 120 cases had vasculitis: 1 case had primary CNS vasculitis, 2 patients (pts) were affected by GPA and 2 pts by EGPA. Method: We describe 3 cases of spontaneous SSH in pts with ANCA Associated Vasculitis (AAV) observed in our unit. Case 1 a 40 year old man with a history of vasomotor rhinitis and three months history of asthma, was admitted because of fever, diffuse arthromialgyas and since few hours' cervical-dorsal pain. Blood test revealed eosinophilia, high CRP, CPK and anti-MPO ANCA MRI showed a right spinal subdural hematoma between C7 and D3. Symptoms improved rapidly with steroid at the dose of 1 mg/kg. Complete neurological recovery and SSH resolution occurred in a few weeks. Case 2 a 55 year old woman was hospitalized for nausea, vomiting and painful left cervico-brachial syndrome. Laboratory test revealed uremia with features of rapidly progressive glomerulonephritis and anti-MPO ANCA. Extracorporeal dialysis with heparin was promptly started. The day after she developed acute flaccid tetraplegia. Spine MRI showed cervico-thoracic subdural hematoma with secondary myelopathy, for which she underwent surgical decompression. Although the patient was treated with plasmapheresis, Rituximab and intravenous steroid, she remained dialysis dependent. Neurological pattern improved slowly: improvement of upper arms weakness but persistent paraparesis with gait difficulty. Intestinal subocclusion episodes and neurological bladder further complicated the course. Case 3 a 67 year old man, with a four months history of fever, arthralgias, weight loss, left pleural effusion and high titre anti-PR3 ANCA, was admitted to start treatment. Deep venous thrombosis of the left lower limb was diagnosed and anticoagulation with LMWH started. After 36 hours he started to complain of dorsal pain and 24 hours later he developed a flaccid paraplegia with neurological bladder with sensory level D1. MRI showed SSH from C6-T1 with mielopathy, for which he underwent surgical decompression. Vasculitic manifestations went into complete remission with steroid and rituximab. After physiotherapy patient improved (mild paresis upper limbs) but he maintained paraplegia at lower limbs. Conclusion: Although rarely, active AAV patients may develop SSHs. Anticoagulation plays an important role in severe evolution. Attention should be paid to cervical and dorsal pain. Investigations must be performed prior anticoagulation.