Imaging Findings of Central Nervous System Vasculitis Associated with Goodpasture's Syndrome: a Case Report

Kim, Jee Young; Ahn, Kook Jin; Jung, Jung Im; Jung, So Lyung; Kim, Bum Soo; Hahn, Seong Tai

doi:10.3348/kjr.2007.8.6.545

Cited by 9 publications

(4 citation statements)

References 7 publications

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“…The stereotactic biopsy remains the gold standard to assure the diagnosis of a CNS vasculitis [ 5 ], but the diagnosis in daily clinical routine is often only based on clinical and imaging findings as well as the response to treatment. This might be a potential reason for overdiagnosing PACNS in clinical routine.…”

mentioning

confidence: 99%

Primary angiitis of the CNS with pure spinal cord involvement: a case report

et al. 2010

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mentioning

confidence: 99%

Primary angiitis of the CNS with pure spinal cord involvement: a case report

et al. 2010

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“…Goodpasture's syndrome may be associated with concomitant extra pulmonary–renal involvement in 10%–30% of cases 3. Only four cases of CNS vasculitis associated with Goodpasture's syndrome have been reported in the literature 3–6.…”

Section: Discussionmentioning

confidence: 99%

Status epilepticus as the initial presentation of antibody-negative Goodpasture's syndrome

Ting

Halim²,

Adnan³

et al. 2017

BMJ Case Reports

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Goodpasture's syndrome is a rare pulmonary-renal disease. It is characterised by presence of auto-antibodies directed against the glomerular basement membrane (GBM) antigen. These antibodies that bind to the GBM antigens cause rapidly progressive glomerulonephritis. The alveolar basement membrane also contains similar antigen, leading to pulmonary haemorrhage in active disease. We report a case of a young man who initially presented with status epilepticus and later was found to have rapidly progressive glomerulonephritis with pulmonary haemorrhage. Serum anti-GBM antibody was negative but the renal biopsy confirmed the diagnosis by showing typical linear IgG along the GBM on immunofluorescent study. He was treated with plasmapheresis and high-dose steroid in combination with oral cyclophosphamide. His renal function normalised after treatment.

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“…Central nervous system (CNS) vasculitis is rarely in GPS patients, especially in those with negative antineutrophil cytoplasmic antibodies (ANCA). All of them presented recurrent seizures, highly suspicious of cerebral vasculitis [8][9][10][11][12]. Sugawara reported a case of anti-GBM disease complicated SLE but without lung hemorrhage presenting central nervous system involvement [13].…”

Section: Introductionsmentioning

confidence: 99%

Goodpasture’s syndrome with neurologic and renal vascular involvement and negative ANCA

Lei

Chen

Liang

et al. 2023

Preprint

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Goodpasture’s syndrome (GPS) is a rare but severe vasculitis disease, characterized by rapidly progressive glomerulonephritis (RPGN), diffuse pulmonary hemorrhage in the presence of anti-glomerular basement membrane antibody (anti-GBM antibody). Central nervous system (CNS) was rarely involved, especially in the absence of anti-neutrophil cytoplasmic antibodies (ANCA). Typically, CNS vasculitis was one of important causes. Herein we present a case of GPS presenting RPGN, clonic-tonic seizures and bloody sputum. She underwent renal puncture and bleeding complication occurred afterwards. Due to the insufficiency of conservative therapy including transfusion, fluid infusion, selective renal arteriography embolization was then employed. Angiogram results showed beading changes of renal artery. Brain CT scan was found negative, but MRA imaging demonstrated the obvious involvement of cranial vessels. The changes of renal and cranial vascular involvement were highly suspicious of nervous vasculitis based on the primary disease. The patient was treated with corticoids and plasma exchange. Patient’s condition improved quickly and anti-GMB antibodies proved to be negative, but renal failure persisted, dependent on long-term hemodialysis. We described for the first-time association of Goodpasture’s syndrome with both renal and nervous vascular involvements.

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Imaging Findings of Central Nervous System Vasculitis Associated with Goodpasture's Syndrome: a Case Report

Cited by 9 publications

References 7 publications

Primary angiitis of the CNS with pure spinal cord involvement: a case report

Primary angiitis of the CNS with pure spinal cord involvement: a case report

Status epilepticus as the initial presentation of antibody-negative Goodpasture's syndrome

Goodpasture’s syndrome with neurologic and renal vascular involvement and negative ANCA

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