PMR & Giant Cell Arteritis

Rheumatology

Overview

Polymyalgia rheumatica (PMR) and giant cell arteritis (GCA) are closely related inflammatory conditions on a clinical spectrum, almost exclusively affecting adults over 50. PMR causes bilateral shoulder and pelvic girdle pain and stiffness with raised inflammatory markers and a dramatic response to low-dose corticosteroids. GCA is a large-vessel vasculitis and a medical emergency because of the risk of irreversible visual loss. The two are intimately linked: around 50% of GCA patients have PMR features, and around 15% of PMR patients develop GCA. UK practice is anchored by NICE CKS for PMR, BSR 2020 for GCA, and NICE TA518 for tocilizumab. SEM relevance: masters athletes and older event populations may present pitchside or in event medicine - any new headache, jaw claudication, or visual symptoms in an over-50 athlete is an emergency.

Anatomy & Pathophysiology

PMR and GCA share immunopathological mechanisms: a T-cell driven granulomatous inflammatory response in genetically susceptible individuals (HLA-DR4, northern European ancestry). The inflammatory target differs.

PMR inflammation targets periarticular structures: synovitis of the shoulders (glenohumeral, subacromial/subdeltoid bursitis) and hips (trochanteric, iliopsoas bursitis), plus biceps tenosynovitis. The pain arises from extra-articular bursitis and synovitis, not from the muscles themselves despite the name. Ultrasound consistently demonstrates bilateral subacromial/subdeltoid bursitis and biceps tenosynovitis.

GCA inflammation targets the arterial wall: granulomatous vasculitis of large and medium arteries. The process begins in the adventitia (vasa vasorum). Dendritic cells activate T cells; T cells and macrophages infiltrate the wall; multinucleated giant cells form in the media (the histological hallmark); the internal elastic lamina is disrupted; intimal hyperplasia narrows the lumen and produces ischaemia.

IL-6 is the dominant cytokine driving the systemic inflammatory response (fever, fatigue, raised CRP/ESR, anaemia) and is the therapeutic target of tocilizumab. Visual loss in GCA arises from posterior ciliary artery occlusion causing anterior ischaemic optic neuropathy (AION) - sudden, painless, irreversible monocular blindness with the contralateral eye at imminent risk. Visual loss occurs in 15-20% of untreated GCA and is almost always preventable with prompt steroids. Large-vessel GCA can affect the aorta and major branches and may present without cranial symptoms; aortic aneurysm is a long-term complication.

Clinical Pearl

PMR-GCA spectrum:

  • PMR = periarticular bursitis and synovitis (shoulders, hips, biceps tenosynovitis).
  • GCA = granulomatous arterial wall inflammation; multinucleated giant cells are the histological hallmark.
  • IL-6 is the dominant cytokine and the therapeutic target of tocilizumab.
  • Visual loss = posterior ciliary artery occlusion producing AION - PREVENTABLE with prompt steroids.
  • ~50% of GCA have PMR; ~15% of PMR develop GCA.
  • Screen every PMR patient for GCA features at every review.

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