Giant Cell Arteritis & PMR Management (EULAR 2023)

Algorithm Steps

1. ▶

   Start

   Suspected GCA or PMR

   Patient >50 years with suggestive symptoms

   1. ●

      Action

      Clinical Assessment

      Evaluate for GCA vs PMR vs overlap

      • GCA: New headache, scalp tenderness, jaw claudication, visual symptoms
      • PMR: Bilateral shoulder/hip girdle pain and stiffness
      • Elevated ESR and/or CRP in both
      • ~40-60% of GCA patients have PMR features
      • ~15-20% of PMR patients develop GCA
      1. ⚠

         Warning

         ⚠️ URGENT: Visual Symptoms?

         Vision loss, diplopia, amaurosis fugax

         • IMMEDIATE high-dose GC to prevent permanent vision loss
         • Do NOT delay treatment for diagnostics
         • IV methylprednisolone 1g/day x3 days often recommended
         • Urgent ophthalmology consultation
         • Irreversible blindness can occur within hours
         1. ●

            Action

            GCA Initial Treatment

            High-dose glucocorticoids

            • Prednisone 40-60 mg/day (or 1 mg/kg, max 60mg)
            • Visual symptoms: Consider IV methylpred 500-1000mg x3 days first
            • Start BEFORE biopsy/imaging (do not delay)
            • Add tocilizumab early to facilitate GC taper
            • Aspirin 75-100mg/day for CV protection (controversial)
            1. ●

               Action

               Tocilizumab for GCA

               GC-sparing biologic (Strong recommendation)

               • Tocilizumab 162mg SC weekly OR every 2 weeks (FDA approved)
               • Add early (at diagnosis or first relapse)
               • Allows faster GC taper and lower cumulative GC exposure
               • Continue for ≥12-18 months minimum
               • Monitor: infections, lipids, LFTs, GI perforation risk
               1. ●

                  Action

                  GCA Glucocorticoid Taper

                  With tocilizumab allows faster taper

                  • Without tocilizumab: Taper over 12-18+ months
                  • With tocilizumab: May taper to 0 within 6-12 months
                  • Example: Reduce by 10mg every 2 weeks until 20mg, then slower
                  • Below 10mg: reduce by 1-2.5mg every 2-4 weeks
                  • Monitor symptoms and inflammatory markers (CRP less reliable on TCZ)
                  1. ◆

                     Decision

                     Treatment Target: Remission?

                     Absence of symptoms + normalized inflammatory markers

                     • Clinical remission: no symptoms of active vasculitis/PMR
                     • Laboratory remission: normal ESR/CRP
                     • Imaging remission (GCA): resolution of arterial inflammation
                     • Monitor for relapse during and after GC taper
                     1. ✓

                        Outcome

                        GCA/PMR Controlled

                        Remission achieved, GC tapered, monitoring continues

                     2. ●

                        Action

                        Relapse Management

                        Common during GC taper

                        • Minor relapse: return to pre-relapse dose, slower taper
                        • Major relapse (GCA cranial symptoms): treat as new diagnosis
                        • PMR relapse: increase to last effective dose
                        • If frequent relapses: add tocilizumab (GCA) or MTX (PMR)
                        • Reconsider diagnosis if multiple relapses on treatment
                        1. ⚠

                           Warning

                           Specialist Referral

                           Refractory disease, large vessel complications, atypical features

      2. ◆

         Decision

         GCA or PMR?

         Distinguish based on clinical features and imaging

         1. ●

            Action

            GCA Diagnosis

            Confirm with imaging (preferred) or biopsy

            • Ultrasound FIRST-LINE imaging (halo sign)
            • Cranial + axillary arteries standard
            • Alternative: MRI, FDG-PET, CTA
            • Temporal artery biopsy if imaging negative/unavailable
            • Do NOT delay treatment for biopsy
         2. ●

            Action

            PMR Diagnosis

            Clinical diagnosis, consider imaging if atypical

            • Bilateral shoulder pain with morning stiffness >45 min
            • Elevated inflammatory markers (ESR/CRP)
            • Consider ultrasound to assess for bursitis/synovitis
            • Exclude other causes: RA, inflammatory myopathy, malignancy
            1. ●

               Action

               PMR Initial Treatment

               Moderate-dose glucocorticoids

               • Prednisone 12.5-25 mg/day initially
               • Dramatic response within days (diagnostic clue)
               • Lower end if mild, higher if severe or GCA overlap suspected
               • If no response: reconsider diagnosis
               • Assess for GCA features at each visit
               1. ●

                  Action

                  PMR Glucocorticoid Taper

                  Gradual reduction over 12-18 months

                  • Once controlled: reduce by 2.5mg every 2-4 weeks to 10mg
                  • Below 10mg: reduce by 1-2.5mg every 4-8 weeks
                  • Minimum treatment duration: 12-18 months
                  • Many patients need low-dose GC for 2-3 years
                  • Monitor for relapse (symptoms + inflammatory markers)