Table 2

Clinical features and response to treatment with biological therapies in 81 published cases

Biological agent	Papers	Type	Disorder	Previous treatment	Reason for change	Outcome	Follow-up
TNFa antagonist	Licata277	CR	Brain	S, cyclo	Relapse	Resolved	<1 y
Infliximab	Ribi278	CR	Brain	S, CyA, thalid, cyclo	Relapse	Improved	2y
	Sarwar279	CR	Brain	S	first line	Resolved	1y
	Fujikawa280	CR	Brainstem	S, cyclo, MTX	Relapse	Improved	ns
	Borhani Haghighi281	CR	Brainstem, cord	S, cyclo	Relapse	Deteriorated (progression)	2y
	Kikuchi282	5 patients	Brain, brainstem	S, MTX	Progression	Stabilised	1y
			All progressive			CSF IL-6 reduced
	Pipitone283	8 patients	Brainstem	S, cyclo	Relapse	Resolved	1y
			Brainstem	S	first line	Resolved	1y
			Brain	S, CyA, MTX	Relapse	Resolved	3y
			Brain, brainstem	S, Aza, cyclo	Relapse	Improved	<1 y
			Brainstem, ON	S, CyA	Relapse	Improved	<1 y
			Brain	S, CyA	Relapse	Improved	3y
			Brainstem	S, CyA	first line	Resolved	1y
			Brainstem	S, CyA	first line	Resolved	<1 y
	Abalos-Medina284	CR	Brain	S, MTX	Relapse	Resolved	1y
	Matsui285	2 patients	brainstem	S, MTX	Progression	Improved	2y
			Brain, brainstem	S, MTX	Progression	Partial response	2y
	BorhaniHaghighi286	3 patients	brainstem	S, cyclo	Relapse	Deteriorated (progression)	ns
			Brain, brainstem	S, cyclo	No response	Improved	2y
	Kadowaki*, 287	CR	Brainstem	S, cyclo	first line	Improved	1y
	Fasano288	CR	Brainstem	S, MTX	Relapse	Improved	ns
	Giardini289	5 patients	Brain	S, Aza, MTX, CyA	first line	Improved	ns
			“cerebral vasculitis”	S, CyA	Relapse	Resolved	1y
			“cerebral vasculitis”	S, CyA	Relapse	Resolved	1y
			“cerebral vasculitis”	S, Aza Chl	Relapse	Resolved	1y
			“cerebral vasculitis”	S, Aza, CyA	Relapse	Resolved	1y
	Coulter96	CR	“cerebral vasculitis”	S, cyclo	Relapse	Resolved	1y
	Kuroda290	CR	Cord	S	first line	Resolved	<1 y
	Kanemaru291	CR	Cord	S, MTX	Progression	Improved	<1 y
	Li292	CR	Brain, brainstem	S, CyA	first line	Resolved	2y
	di Filippo293	CR	Brainstem	S, Aza	first line	Resolved	4y
	Satuka* 294	CR	Brain	S	first line	Resolved	3y
	Uygunoglu98	CR	Cord	S, cyclo	Progression	Improved	ns
	Hibi234	3 patients	Cord	S, cyclo	first line	Improved	3y
			Brainstem	S	Relapse	Resolved	1y
			Brain	S	Relapse	Resolved	1y
	Desbois295	13 patients	Brainstem progressive	S	Relapse	Stabilised	1y
			Brain	S, cyclo, Aza, MMF	Progression	Improved	4y
			Brain	S, Aza, cyclo		Improved	1y
			Brain	S, Aza CyA		Resolved	1y
			Cord, brain, brainstem	S, Aza, Cyclo, MMF		Resolved	<1 y
			Brainstem	S, Aza		Improved	2y
			Brain	S, Aza		Improved	1y
			Brain	S, Aza, cyclo, Chl		Improved	>10 y
			Brainstem	S, cyclo		Improved	2y
			Brain	S, cyclo, Chl		Resolved	6y
			Cord, brainstem	S		No response	1y
			Brainstem	S, Aza		Resolved	<1 y
			Brainstem	S, cyclo		Improved	1y
	Zeydan253	15 patients	Brain, brainstem	S, Aza, cyclo		Improved	<1 y
	Hamada* 235	CR	Relapsing disease	Ns		four improved	1y
	Baresic296	CR	Progressive brainstem	S, MTX	Progression	Partial response	ns
			Brainstem, ON	S, Aza, CyA	Relapse	Improved	2y
Adalimumab	Belzenegui297	CR
	Lecesse259	CR	Cord, brain	S, Aza, cyclo, IFX	s/e IFX	Improved	3y
	Robinson298	CR	Brainstem	S, CyA, IFX	Relapse	Improved	<11 y
	Korkmaz260	CR	Brainstem	S, MMF	Relapse	Resolved	1y
	Desbois295	4 patients	Brainstem	S, cyclo, Aza	Relapse	Improved	<1 y
			Brainstem	S, cyclo, Aza, INF		Resolved	2y
			Brain	S, Aza		Improved	<1 y
			Cord	S, Aza		Improved	2y
			Cord	S, Aza INF		Improved	4y
Etanercept	Alty299
		CR	Brainstem	S, Aza, CyA, thalid, IFX	Relapse	Improved	3y
Golimumab	Vitale300
		?7 patients	no data			no data
IL-6 antagonist
Tocilizumab	Shapiro301	CR
	Urbeniak302	CR	Brainstem	S, Aza, MTX, MMF, IFX	s/e IFX	Resolved	<1 y
	Addimanda303	3 patients	Cord	S, Aza, IFX	Relapse	Improved	1y
			ON, brain, cord	S, IFX, cyclo	Unresponsive	Improved	1y
			ON, brain, cord	S, Aza, cyclo, INF	Relapse	Stabilised	1y
			Brain, brainstem, cord	S, CyA, IFX	Relapse	Stabilised	1y
IL-1 antagonist
Anakinra	Vitale304		ON
Canakinumab	Emmi305	CR		S, Aza	first line	Improved	ns
Gevokizumab		one case ? same as Vitale
IL-12 / −17 / −23 antagonist	no reports
Ustekinumab
Secukinumab
CD20 antagonist
Rituximab	Messina94
	Kidd306	CR		S, cyclo	Relapse	Improved	ns
	Jade307	CR	Cord	S, Aza, MMF, IFX	Relapse	Resolved	3y
		CR	Brainstem	S, col, cyclo, IFX	Relapse	Resolved	3y
			Tumefactive brain
CD52 antagonist
Alemtuzumab	Lockwood308	12 patients
			ns	S, Aza, CyA, cyclo, MMF	ns	Partial response	ns
				S		No response
				S		Resolved
				S, CyA		Partial response
				S, cyclo		Resolved
				S, Aza		No response
				S, Aza col, thalid		No response
				S		Resolved
				S, CyA		No data
				S, Aza MTX		No response
				S, Aza CyA		Good response
				S, CyA, thalid		No response
Janus kinase inhibitors	no reports

*Data derived from abstract alone.
Aza, azathioprine; Chl, chlorambucil; col, colchicine; CR, case report; CyA, cyclosporine A; cyclo, cyclophosphamide; IFX, infliximab; INF, interferon α2a; MMF, mycophenolate; MTX, methotrexate; ns, not stated; S, corticosteroids; thalid, thalidomide.