Table 1

Topics for the systematic literature search

SLR 1: General ManagementSLR 2: Treatment
  • Diagnosis: recognition, referral criteria, fast-track diagnosis, role of imaging for diagnosis, role of biopsy for diagnosis, interdisciplinary work-up, considerations for sub-types of disease such as cranial/ischaemic/large vessel, isolated aortitis, IgG4-related disease, LVV disease in other vasculitides.

  • Prognostic and therapeutic implications of disease phenotypes: cranial vs extra-cranial, isolated aortitis, other forms including IgG-4 related disease, imaging, other biomarkers, comorbidities and complications, disease damage versus activity.

  • Long-term follow-up of patients: clinical assessments and frequency, imaging, patient-reported outcomes, physical therapies and management of complications.

  • Patient education and other aspects of patients-centred care.

  • Drug therapy: dosing, length of therapy, outcome and treatment-related side effects for the following drugs: glucocorticoids, methotrexate and other non-biological immunosuppressive agents, tocilizumab and other biological agents.

  • Specific treatment of organ complications: loss of vision and stroke), relapsing, refractory, glucocorticoid-dependent disease.

  • Revascularisation procedures: indications for referral, management of aneurysms and/or vessel stenosis.

  • Adjunctive therapies and prophylaxis: aspirin, cardiovascular and cerebrovascular disease, infections, vaccination, osteoporosis.

  • LVV, large vessel vasculitis; SLR, systematic literature review.