**3.2 Medium vessel vasculitis**

*Vascular Biology - Selection of Mechanisms and Clinical Applications*

forms of the disease [7].

more relapses [7].

this hypothesis.

medication was stopped [7, 8].

ies are necessary though [7].

maximum dose is 80 mg/day [7].

cases, methylprednisolone in pulses of 500 mg<sup>−</sup><sup>1</sup>

Rheumatology Society prefers oral corticosteroid [7].

the management are variable from 7.5–15 mg/week [7].

until controlled in the patient. These strategies are valid for simple and complicated

Simple form is defined by cephalic isolated symptoms with visual disturbance or changes in central nervous system. Nowadays, the gold standard treatment is oral prednisone or equivalent 40–60 mg/day, and methylprednisolone is not used anymore. The initial dose recommended is 0.7 mg/kg day of prednisone and the

The complicated form is defined when there is an ophthalmologic or a central nervous or extracephalic complication, mainly aorta and its branches. In these

It is strategic to introduce corticosteroid-sparing drugs when the disease course is lasting long. Methotrexate is the most used treatment and with more evidence levels. There are four trials with methotrexate as a sparing drug, but the doses and

Cyclophosphamide has demonstrated effectiveness in patients dependent on corticosteroids or resistant to treatment. It is necessary to give more than 20 mg/day

injection in 6 courses is standard from 5 months on average. It is highly important to

Nowadays, one option is biologics medicines. Anti-TNF-alpha does not have enough studies that showed control of the disease with this class of medication [7]. Tocilizumab, a humanized antibody that blocked membranous and soluble receptors of IL-6 (IL-6R), is a current option since IL-Il-6 is implicated in the etiopathogenesis of this affection. Three main studies evaluated the efficacy of the drug,

A randomized control trial with 30 patients had tested 20 patients receiving corticoids and tocilizumab (8 mg/kg every 4 week during a year), and 10 received corticoids and placebo. The survival without relapses during a year was highest in patients treated with tocilizumab. However, there are no data available after the

Another promising biotherapy is abatacept. This medication with corticoids could decrease the risk of relapses, although more data is necessary to corroborate

Ustekinumab, a subunit against anti-p40 IL-12/23 targeting Th1 and Th17 responses, has been showing similar side effects as corticoids. Some patients, who had refractory disease, have been treated with anakinra and they achieved success. Anakinra is a biopharmaceutical drug that blocks IL-1. All patients who had taken the drug had shown improvement in inflammation biomarkers and/or in their symptoms, with the disappearance of arterial inflammation in PET/CT. More stud-

Other pharmacological drugs can be used as adjuvant treatments such as antiplatelets and anticoagulants, but there is no official recommendation about these

Azathioprine therapy has less controlled studies. Some studies showed a modest improvement. Hydroxychloroquine, an antimalarial synthetic drug, was tested also. One French study divided patients into two groups: one took prednisone 0.7 mg/ kg/day in the beginning associated with hydroxychloroquine (400 mg/day) and the control group just received prednisone and placebo. The group that had hydroxychloroquine and corticoid had usually stopped later the prednisone and they had

for 6 months or 10 mg/day for 1 year or more. The dose from 500 mg/m<sup>2</sup>

warn patients of several side effects such as bone marrow suppression [7].

although the therapeutics was different between these studies [7, 8].

consecutive days. During maintenance, some authors believe in doses less than 5 mg/day. The American Society of Ophthalmology recommends pulses of intravenous methylprednisolone, when patients have ophthalmologic symptoms, but the

g a day shall be used for 1 to 5

or 500 mg/

**138**
