Clinical features of central nervous system involvement in patients with eosinophilic granulomatosis with polyangiitis: a retrospective cohort study in China

Liu, Suying; Guo, Ling; Fan, Xiaoyuan; Zhang, Zhaocui; Zhou, Jiaxin; Tian, Xinping; Li, Mengtao; Zeng, Xiaofeng; Wang, Li; Zhang, Fengchun

doi:10.1186/s13023-021-01780-x

Orphanet Journal of Rare Diseases

Table 2 Lesions characteristics, treatment and outcomes of EGPA patients with CNS involvement

From: Clinical features of central nervous system involvement in patients with eosinophilic granulomatosis with polyangiitis: a retrospective cohort study in China

ID	Sex/age	Lesions features	Lesions location	Induction therapy	Intrathecal injections^a	FU, months	Outcomes
1	F/39	Ischemic infarctions	Multiple	MP 80 mg qd	N	1	death
2	F/46	Ischemic infarctions	Right semiovale centers	Pred 80 mg qd + CYC	N	84	remission^b
3	M/49	Ischemic infarctions; PRES	Multiple, subacute cerebral infarcts in left basal ganglia	MP pulse + IVIG + CYC	DXM *3	72	remission
4	M/49	Ischemic infarctions	Basal ganglia	MP pulse	N	48	remission^b
5	F/71	Spinal cord involvement	Thoracic spinal cord	MP 40 mg qd	N	24	remission
6	M/64	Ischemic infarctions; PRES	Right semiovale centers	MP 80 mg qd + IVIG + CYC	N	46	remission
7	M/52	Ischemic infarctions; PRES; ataxia;	Bilateral basal ganglia; cerebellar ataxia	MP 24 mg qd + CYC + AZA	DXM 1 + MTX 4	36	remission^b
8	F/72	Ischemic infarctions	Multiple lesions in cerebrum	MP 160 mg qd	N	20	remission^b
9	F/49	HCP	Cerebral falx	MP pulse	N	15	remission^b
10	F/45	Ischemic infarctions; PRES	Under bilateral frontal and parieto-occipital cortex	Pred 40 mg qd + CYC	N	42	remission^b
11	F/47	HCP	Bilateral brain	MP pulse	DXM *5	132	remission
12	M/57	Medulla oblongata and spinal cord lesions	Medulla oblongata and cervical spinal cord	MP pulse	N	17	remission
13	F/63	Ischemic infarctions	Right basal ganglia and white matter	MP pulse	N	12	remission
14	F/55	Intracranial hemorrhages; PRES	Right frontal–temporal lobe	MP pulse + Plasma change + CYC	N	1	death
15	M/46	Ischemic infarctions	Multiple lesions in cerebrum	MP 40 mg qd + CYC	N	4	remission
16	M/25	Medulla oblongata and spinal cord involvement	Medulla oblongata and thoracic spinal cord	Pred 17.5 mg qd + CYC	N	76	remission
17	M/34	Intracranial hemorrhages	Subarachnoid space	Pred 50 mg qd + CYC	N	9	remission^b
18	M/41	Ischemic infarctions; PRES; medulla oblongata lesions; ataxia	Subcortex of bilateral frontal parietal lobe; right side of medulla oblongata; cerebellum	MP pulse + IVIG	N	20	remission
19	M/64	Ischemic infarctions; intracranial hemorrhages; PRES	Infarcts in cortex and white matter; cerebral hemorrhages in left basal ganglia, cortex, subcortex and the sulci	MP pulse	DXM *1	6	remission

Sex, F-female, M-male; FU, follow-up duration; MP, methylprednisolone; Pred, prednisone; IVIG, intravenous immunoglobulin; CYC, cyclophosphamide; PRES, posterior reversible encephalopathy syndrome; HCP, hypertrophic cranial pachymeningitis
^a“N” indicates no intrathecal injections were used during the follow-up, and the number after the asterisk refers to the number of intrathecal injections in this column; DXM, Dexamethasone; MTX, methotrexate; AZA, azathioprine
^bRelapse during glucocorticoid reduction;

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ISSN: 1750-1172

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