Review Question - QID 101687

This woman's symptoms of pain with chewing food, along with weight loss, fever, and stiffness, are suggestive of temporal arteritis, which affects large-medium sized arteries and may lead to blindness if untreated.

Temporal arteritis (also called giant cell arteritis) is caused by inflammation damaging large to medium sized arteritis including the temporal artery. Symptoms include pain with mastication or brushing hair (due to scalp tenderness). Patients may lose vision on the side of the affected eye, which can be permanent. The patient's symptoms of shoulder and hip pain are characteristic of polymyalgia rheumatica (PMR), which is a co-morbid autoimmune condition seen in about 50% of patients with temporal arteritis. PMR is typically seen in females over the age of 50 and presents with muscle soreness without weakness. Of those with PMR, about 15-30% have temporal arteritis.

Unwin et al. review PMR and temporal arteritis. They note that untreated PMR may result in significant disability, and that blindness and stroke are both potential complications of untreated temporal arteritis. The treatment for both conditions is steroids, with higher dose required if temporal arteritis is diagnosed. The duration of treatment may be up to 5 years before remission is reached.

Muratore et al. note that while glucocorticoids are highly effective at inducing remission of co-occurring PMR and temporal arteritis, they result in considerable side effects including weight gain, loss of bone density, and mood changes, as well as metabolic derangements. They conclude that the literature supports gradual weaning of corticosteroids when patients have been stabilized, with relapses occurring more often in patients with temporal arteritis. Immunosuppressive agents may also be used as adjuncts in frequently recurring disease.

Illustration A shows the size of arteries affected by the different vasculitides.
Illustration B shows granulomatous inflammation in temporal artery wall (short arrows) and near-complete luminal obstruction due to intimal hyperplasia (long arrow).
Illustration C presents the most recent EULAR/ACR diagnostic criteria for PMR with and without ultrasound information.

Incorrect Answers:
Answer 1: This is a symptom of dermatomyositis, a cause of proximal muscle pain AND weakness.
Answer 3: Patients with Systemic Lupus Erythematosus often have a butterfly-shaped rash across their face and cheeks, which can be mistaken for being "easily sunburned" by patients.
Answer 4: Granulmoatosis with polyangiitis (Wegener's Granulomatosis) is a c-ANCA positive vasculitis that leads to lung, sinonasal, and renal complications, and may present with hemoptysis.
Answer 5: Thickened, tight skin on the fingers or toes is called sclerodactyly and is a sign of scleroderma.