MKSAP Quiz: 3 weeks of fatigue, low-grade fevers
A 74-year-old woman, recently diagnosed with polymyalgia rheumatica, is evaluated for three weeks of fatigue, low-grade fevers, and right-sided headache and scalp tenderness. During this time, she has also had recurrence of shoulder achiness and hand stiffness. Following a physical exam and lab studies, what is the most appropriate diagnostic test to perform?
A 74-year-old woman is evaluated for 3 weeks of fatigue, low-grade fevers, and right-sided headache and scalp tenderness. During this time, she has also had recurrence of shoulder achiness and hand stiffness that lasts about 30 minutes and is worse in the morning. Polymyalgia rheumatica diagnosed 4 months ago had dramatically improved after initiation of prednisone, 15 mg/d, followed by a taper, with no symptoms until 3 weeks ago. Apart from low-dose prednisone, she takes no other medications.
On physical examination, vital signs are normal. Tenderness over the right scalp is noted. Eye examination is unremarkable. Pain occurs with abduction of both arms and in the hip girdle area and persists with and without movement. There are bony prominences over the proximal interphalangeal joints. No rash, synovitis, or bruits are observed.
Laboratory studies at follow-up:
| Erythrocyte sedimentation rate | 88 mm/h | High |
| C-reactive protein | 4.0 mg/dL (40 mg/L) | High |
The prednisone dose is increased.
Which of the following is the most appropriate diagnostic test to perform next?
A. CT angiography of the chest, abdomen, and pelvis
B. MRI of the brain
C. Rheumatoid factor and anti–cyclic citrullinated peptide antibodies
D. Temporal artery biopsy
MKSAP Answer and Critique
This content is available to ACP MKSAP subscribers in the Rheumatology section. More information about ACP MKSAP is available online.
The most appropriate diagnostic test to perform next is temporal artery biopsy (Option D). Giant cell arteritis (GCA) can develop in up to 20% of patients with polymyalgia rheumatica (PMR). Symptoms of GCA may include headache; jaw claudication; visual changes; hip and shoulder girdle pain (which are also symptoms of PMR); and constitutional symptoms, such as fever and fatigue. Findings may include tenderness over the temporal artery, reduced peripheral pulses, bruits, and significantly elevated inflammatory markers. A complication of GCA is ischemic optic neuropathy, which can cause irreversible blindness; early recognition and treatment of GCA are critical. PMR classically presents with pain and stiffness of the shoulder and hip girdles, but in approximately 20% of cases, patients may also have peripheral inflammatory arthritis affecting the small joints of the hand. Because of the relationship between PMR and GCA, all patients with PMR should be regularly asked about GCA symptoms. GCA may develop soon after the onset of PMR, but it may also occur while glucocorticoids for PMR are being tapered. If symptoms of GCA develop, high-dose glucocorticoid therapy should be started to avoid complications, such as vision loss, and should not be delayed for biopsy. A temporal artery biopsy should be performed on the affected side. Ideally, this would be performed urgently (within 2 weeks) to increase the likelihood of a positive pathologic result. This patient with a recurrence of PMR symptoms and findings suggestive of GCA should begin receiving high-dose glucocorticoids and should undergo temporal artery biopsy for diagnostic confirmation.
All patients with GCA should be screened for large-vessel involvement using noninvasive imaging, such as CT angiography (Option A), to assess for extracranial disease. This patient does not yet have a diagnosis of GCA and does not have audible bruits or signs of limb claudication. Although CT angiography should be considered in the future, it would not be the next diagnostic test in this patient suspected of having GCA.
Although MRI of the brain (Option B) may be useful in patients with unexplained persistent headaches (especially if concerning neurologic deficits or systemic symptoms are present), this patient's right-sided headache with associated scalp tenderness on a background of PMR suggests GCA. MRI of the brain is not recommended to diagnose GCA.
Rheumatoid factor and anti–cyclic citrullinated peptide antibodies (Option C) are useful in evaluating rheumatoid arthritis. Although this patient has hand stiffness, she does not have features of rheumatoid arthritis on examination (e.g., synovitis, prolonged morning stiffness). Additionally, symptoms have been present for only 3 weeks. GCA better explains her symptoms and significantly elevated inflammatory markers.
Key Points
- Symptoms of giant cell arteritis may include headache, jaw claudication, visual changes, and constitutional symptoms.
- Giant cell arteritis is suspected on the basis of clinical presentation, and temporal artery biopsy can confirm the diagnosis.