Background/Purpose:

Aortitis can occur alone or associated with other conditions. It is often refractory to standard immunosuppressive therapy. IL-6 has been implicated in the mechanisms leading to aortitis. Tocilizumab (TCZ) is a humanized monoclonal anti-IL6 receptor (IL-6R) antibody. Our aim was to assess the efficacy and side-effects of TCZ in patients with refractory inflammatory aortitis.

Methods:

Multicenter study of 13 patients diagnosed of inflammatory aortitis due to different underlying conditions. The diagnosis of aortitis was based on imaging (CT angiography, MR angiography, PET and/or echocardiogram).

Results:

Patients (12 women/1 man) had a mean age ±SD of 50.69±21.93 years (table). The underlying conditions were: Takayasu arteritis (TKY) (6 cases), giant cell arteritis (GCA) (5 cases), relapsing polychondritis (RP) (1 case) and idiopathic aortitis (1 case).
TCZ was the first biological drug  in all patients with GCA, and in the case of idiopathic aortitis but in only 1 of 6 patients with TKY. In the remaining cases anti-TNF inhibitors had previously been prescribed. TCZ standard dose was 8 mg/k/iv/4 weeks.
After a median [interquartile 25-75 range (IQR)] follow-up of 13 [11-18] months most patients experienced clinical improvement and a reduction of ESR (from 46±37 mm/1st h to 6±4 mm/1st h in the last visit). Patients also had clinical improvement.  At TCZ onset, 15% of patients had polymyalgia rheumatica and 31% fever. After 3 months on TCZ therapy, these  manifestations had disappeared. Also, constitutional syndrome observed in 31% of patients at TCZ onset, improved following this therapy (16% at 3 months), and disappeared completely after 6 months of treatment. In addition, a reduction in the dose of corticosteroids was achieved (prednisone or equivalent dose: from 28±17 mg/day at TCZ onset to 4±3 mg/day in last visit).
TCZ was relatively safe; only in 1 patient TCZ had to be discontinued because of neutropenia.

Conclusion:

Our results indicate that TCZ is a biologic agent effective and safe in patients with inflammatory aortitis refractory to conventional drugs including corticosteroids or other biologic immunosuppressive drugs.

Case	Age/Sex	Underlying disease	Previous synthetic and/or biologic immunosuppressive drugs	Prednisone dose or equivalent (at TCZ onset)   mg/d	Prednisone dose or equivalent  (at last visit)      mg/d	CRP/ESR* (at TCZ onset)	CRP/ESR* (at last visit)	Follow-up with TCZ (months)	Serious side effects
1	7/F	TKY	MTX, CYM,MM, IFX, ETN	30	0	12/72	<0.1/5	24	None
2	57/F	TKY	CYM	45	5	3.33/99	0,25/2	18	None
3	26/F	TKY	MTX, AZA, IFX	50	7.5	2.8/33	0.03/2	12	None
4	16/F	TKY	MTX, ADA	50	7.5	0.5/14	0.1/7	12	None
5	45/F	TKY	MTX, AZA, MM, IFX	25	0	<0.1/28	<0.1/3	13	None
6	41/F	TKY	MTX, ADA, IFX	40	10	3.7/29	0.03/10	3	None
7	77/F	GCA	MTX	10	2.5	3.7/120	1.7/7	5	None
8	59/F	GCA	MTX	60	5	<0.1/2	<0.1/2	16	None
9	65/F	GCA	MTX	17.5	0	<0.1/3	<0.1/2	20	Suspended: neutropenia
10	67/F	GCA	MTX	10	7.5	1.9/44	1.45/13	4	None
11	74/F	GCA	MTX	0	0	0.8/46	<0.1/4	11	None
12	50/F	RP	MTX, CyA, leflunomide, CYM, IFX	30	5	0.9/13	<0.1/13	20	None
13	75/M	Idiopathic	None	0	0	-/98	-/-	17	None

TCZ: tocilizumab;  IFX: infliximab   ADA: adalimumab    ETN: etanercept    RTX: rituximab    MTX: methotrexate  AZA: azathioprine  CYM: cyclophosphamide  CyA: cyclosporine A   MM: Mycophenolate mofetil

* CRP: C-reactive protein (CRP) (mg/L)/ ESR: erythrocyte sedimentation rate in 1^st hour

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/tocilizumab-in-refractory-aortitis-a-multicenter-study-of-13-patients/