PMR & Giant Cell Arteritis
PMR: bilateral shoulder/hip girdle pain and stiffness in elderly. ESR/CRP markedly elevated. Dramatic response to low-dose prednisone. GCA: temporal headache, jaw claudication, vision loss. Emergent steroids -do not wait for biopsy.
🔍 Overview
PMR vs GCA
| Feature | PMR | GCA |
|---|---|---|
| Age | > 50 (mean ~72) | > 50 (mean ~72) |
| Symptoms | Bilateral shoulder/hip girdle pain, morning stiffness > 45 min | New temporal headache, jaw claudication, scalp tenderness, visual changes |
| Feared complication | 15–20% develop GCA | Permanent vision loss (anterior ischemic optic neuropathy) |
| ESR/CRP | ↑↑ (ESR often > 40–50) | ↑↑↑ (ESR often > 50–100) |
| Diagnosis | Clinical + ↑ inflammatory markers + response to steroids | Temporal artery biopsy (gold standard). Temporal artery US. |
| Treatment | Prednisone (Deltasone) 12.5–25 mg daily | Prednisone (Deltasone) 60 mg daily (or 1g IV methylpred × 3 days if vision threatened) |
If GCA is suspected with visual symptoms → start high-dose steroids IMMEDIATELY. Do NOT wait for biopsy. Vision loss is irreversible. Biopsy can be done within 1–2 weeks of starting steroids without affecting results.
PMR vs GCA Comparison
| Feature | PMR | GCA |
|---|---|---|
| Age | > 50 (mean ~72), women > men | > 50 (mean ~72), women > men |
| Symptoms | Bilateral shoulder/hip girdle pain, morning stiffness > 45 min, difficulty rising from chair | New temporal headache, jaw claudication, scalp tenderness, vision changes, fever |
| ESR/CRP | ↑↑ (ESR often > 40–50) | ↑↑↑ (ESR often > 50–100) |
| Treatment dose | Low-dose: Prednisone 15–20 mg daily | High-dose: Prednisone 60 mg daily (or IV methylpred 1g × 3d if vision loss) |
| Biopsy needed? | No -clinical diagnosis + response to steroids | Yes -temporal artery biopsy is gold standard (but do NOT delay treatment) |
| Duration of treatment | 12–18 months taper (relapse common) | 1–2 years taper; tocilizumab as steroid-sparing agent |
GCA Red Flags -Act Immediately:
• Jaw claudication (most specific symptom for GCA)
• Sudden vision loss (anterior ischemic optic neuropathy -irreversible)
• New headache in patient > 50 years old
• Temporal artery tenderness or nodularity on palpation
• Scalp tenderness (pain when combing hair)
• Jaw claudication (most specific symptom for GCA)
• Sudden vision loss (anterior ischemic optic neuropathy -irreversible)
• New headache in patient > 50 years old
• Temporal artery tenderness or nodularity on palpation
• Scalp tenderness (pain when combing hair)
Mnemonic -TEMPORAL for GCA Features:
Temporal headache · ESR elevated (> 50) · Monocular vision loss · Polymyalgia overlap (15–20%) · Older age (> 50) · Response to steroids (dramatic) · Artery biopsy (skip lesions -may need bilateral) · Large vessel involvement (aortitis, subclavian)
Temporal headache · ESR elevated (> 50) · Monocular vision loss · Polymyalgia overlap (15–20%) · Older age (> 50) · Response to steroids (dramatic) · Artery biopsy (skip lesions -may need bilateral) · Large vessel involvement (aortitis, subclavian)
🚨 Management
PMR Treatment
- Prednisone (Deltasone) 12.5–25 mg daily -dramatic response within 24–72h (if no response, reconsider diagnosis)
- Taper over 12–18 months (very slow -relapse is common)
- Calcium + Vitamin D + DEXA (steroid-induced osteoporosis prevention)
GCA Treatment
- Prednisone (Deltasone) 60 mg daily -immediate start
- If vision threatened: Methylprednisolone (Solu-Medrol) 1g IV daily × 3 days, then oral prednisone
- Slow taper over 1–2 years
- Tocilizumab (Actemra) -IL-6 inhibitor, steroid-sparing agent (GiACTA trial)
- Low-dose aspirin -reduces ischemic complications (visual, stroke)
Steroid Taper Guide
| Condition | Starting Dose | Taper Schedule |
|---|---|---|
| PMR | Prednisone 15–20 mg daily | ↓ 2.5 mg every 2–4 weeks to 10 mg, then ↓ 1 mg/month. Total duration 12–18 months. Relapse common -increase dose if flare and re-taper slowly. |
| GCA (no vision loss) | Prednisone 60 mg daily | ↓ 10 mg every 2 weeks to 20 mg, then ↓ 2.5 mg every 2–4 weeks to 10 mg, then ↓ 1 mg/month. Total 1–2 years. |
| GCA (with vision loss) | IV Methylprednisolone 1g daily × 3 days, then prednisone 60 mg | Same taper as GCA above after completing IV pulse. Add tocilizumab early for steroid-sparing. |
🧪 Workup
- ESR, CRP -both markedly elevated
- CBC -normocytic anemia common, ↑ platelets
- Temporal artery biopsy -GCA gold standard. Get ≥ 1 cm sample. Skip lesions mean negative biopsy doesn't exclude GCA.
- Temporal artery US -"halo sign" (hypoechoic wall thickening). Increasingly used as first-line in Europe.
- CK -normal in PMR (elevated CK → consider myositis instead)
- RF, anti-CCP -negative (rule out RA, which can mimic PMR)
💊 Medications
| Drug | Dose | Indication |
|---|---|---|
| Prednisone (Deltasone) | 12.5–25 mg (PMR) / 60 mg (GCA) | First-line both conditions |
| Methylprednisolone (Solu-Medrol) | 1g IV × 3 days | GCA with threatened vision loss |
| Tocilizumab (Actemra) | 162 mg SC weekly | GCA steroid-sparing (GiACTA trial) |
| Methotrexate (Trexall) | 7.5–15 mg weekly | Steroid-sparing in relapsing PMR (limited evidence) |
| Aspirin | 81 mg daily | GCA -reduces ischemic events |
📋 On Rounds
Pimp Questions
Should you wait for biopsy results before starting steroids in suspected GCA with vision symptoms?
Absolutely not. Vision loss from GCA (anterior ischemic optic neuropathy) is irreversible. Start high-dose steroids immediately -IV methylprednisolone 1g daily if vision is threatened. Temporal artery biopsy remains diagnostic for up to 2 weeks after starting steroids, so there is no reason to delay treatment. The risk of permanent blindness far outweighs the risk of a short course of steroids if the diagnosis turns out to be wrong.
📋 Case 1, Classic PMR Presentation
Patient: 72F presents with 3 weeks of bilateral shoulder and hip girdle pain, worse in the morning. Morning stiffness lasting 2 hours. Cannot lift arms above head or rise from a chair without assistance. No headache or vision changes.
Labs: ESR 88 mm/hr (↑↑), CRP 45 mg/L (↑↑), CK normal, RF negative, anti-CCP negative.
Management:
- Start Prednisone (Deltasone) 15 mg daily
- Dramatic response within 24–48 hours -patient can raise arms and rise from chair by day 2
- This dramatic response essentially confirms the diagnosis
- Begin slow taper: ↓ 2.5 mg every 2–4 weeks to 10 mg, then ↓ 1 mg/month
- Start calcium 1000 mg + vitamin D 800 IU daily, order DEXA scan (steroid osteoporosis prevention)
- Monitor ESR/CRP q2–4 weeks during taper; counsel patient that relapse is common
- Screen for GCA symptoms at every visit: headache, jaw claudication, vision changes
Key lesson: PMR responds dramatically to low-dose prednisone. If no improvement in 24–72h, reconsider the diagnosis. Normal CK rules out myositis; negative RF/anti-CCP rules out RA.
📋 Case 2, GCA with Jaw Claudication
Patient: 68M with 2 weeks of new-onset severe right temporal headache, jaw pain when chewing (jaw claudication), and scalp tenderness. No vision changes. Temporal artery is tender and nodular on palpation.
Labs: ESR 102 mm/hr (↑↑↑), CRP 68 mg/L, platelets 450k, normocytic anemia (Hgb 11.2).
Management:
- Start Prednisone (Deltasone) 60 mg daily IMMEDIATELY -do not wait for biopsy
- Schedule temporal artery biopsy within 1–2 weeks (steroids do not affect biopsy results for up to 2 weeks)
- Start aspirin 81 mg daily (reduces ischemic complications)
- Biopsy result: Granulomatous inflammation with giant cells, fragmented internal elastic lamina → confirms GCA
- Plan: Taper prednisone (↓ 10 mg q2 weeks to 20 mg, then slow). Consider Tocilizumab (Actemra) for steroid-sparing.
- Ophthalmology follow-up -monitor for visual complications
Key lesson: Jaw claudication is the most specific symptom for GCA. Start high-dose steroids immediately. Biopsy confirms but should never delay treatment.
📋 Case 3, PMR Patient with Sudden Vision Loss
Patient: 75F with known PMR on prednisone 10 mg (tapering), presents with sudden painless monocular vision loss in the left eye lasting 20 minutes, now resolved. Reports new left temporal headache × 3 days.
Exam: Left temporal artery tender, diminished pulse. Visual acuity 20/20 right, 20/200 left with afferent pupillary defect.
Labs: ESR 75 (rising from 30 on prior visit), CRP 38.
This is an ophthalmologic emergency:
- IV Methylprednisolone 1g NOW -do not wait for any testing. Repeat daily × 3 days.
- This is amaurosis fugax (transient ischemic visual loss) → impending permanent vision loss
- Urgent ophthalmology consult within hours
- After 3 days IV pulse → transition to prednisone 60 mg daily with slow taper
- Add Tocilizumab (Actemra) 162 mg SC weekly as steroid-sparing agent
- Temporal artery biopsy within 1–2 weeks
- Key: 15–20% of PMR patients develop GCA -always screen for GCA symptoms during follow-up
Key lesson: PMR can evolve into GCA. Any new headache, jaw claudication, or vision changes in a PMR patient = emergent GCA workup. Vision loss from GCA is irreversible -IV methylprednisolone must be given immediately.
⚡ Summary
Two Related Diseases, Same Age Group
PMR: bilateral shoulder and hip girdle pain with morning stiffness beyond 45 min, in patients over 50. GCA: new temporal headache, jaw claudication, scalp tenderness and visual change. 15 to 20% of PMR patients develop GCA, and about 40 to 50% of GCA patients have PMR symptoms, so each is a prompt to ask about the other.
Treat Suspected GCA Before the Biopsy
Start high-dose steroids immediately when GCA is suspected with visual symptoms. Do not wait. Vision loss from anterior ischemic optic neuropathy is irreversible and can become bilateral within days. The biopsy stays positive for 1 to 2 weeks after steroids start, so treating first costs nothing diagnostically.
Jaw Claudication Is the Most Specific Symptom
Pain in the masseters on chewing that eases with rest, from ischemia of the muscles of mastication. It carries the highest likelihood ratio of any GCA feature, well above headache or scalp tenderness, so asking about it directly is more useful than any single blood test.
Dose by Whether Vision Is Threatened
GCA without visual symptoms: prednisone 40 to 60 mg daily. GCA with visual loss or amaurosis: methylprednisolone 1 g IV daily for 3 days, then oral. PMR: prednisone 12.5 to 25 mg daily. Giving a PMR dose to GCA is a sight-threatening error, which is why the two diagnoses must be separated explicitly rather than treated as a spectrum.
The Steroid Response Is Diagnostic in PMR
Dramatic improvement within 24 to 72 h is expected. Failure to respond that quickly means reconsider the diagnosis: seronegative rheumatoid arthritis, inflammatory myopathy, hypothyroidism, malignancy or infection. Do not simply escalate the dose in a non-responder.
Interpret the Inflammatory Markers Carefully
ESR is often above 50 to 100 in GCA and above 40 in PMR, but normal markers occur in a small minority of GCA and do not exclude it when the clinical picture is strong. Check both ESR and CRP, since CRP is the more sensitive of the two and one may be raised without the other.
Biopsy a Long Segment, and Consider Ultrasound
Temporal artery biopsy remains the gold standard, but skip lesions mean a short segment gives false negatives, so take at least 1 to 2 cm and consider a bilateral biopsy. Temporal artery ultrasound showing the halo sign is a useful non-invasive alternative where expertise exists, and it is fastest when it is done before steroids blunt the findings.
Plan the Long Taper and Protect the Patient
PMR usually needs 1 to 2 years; GCA needs 1 to 2 years or longer, tapering slowly and guided by symptoms rather than by ESR alone. Tocilizumab is a proven steroid-sparing agent in GCA and reduces cumulative exposure and relapse; methotrexate is an option in relapsing PMR. Every patient needs bone protection with calcium, vitamin D and a bisphosphonate, plus glucose, blood pressure and cataract monitoring, because the steroid course is measured in years and its complications are what most of these patients ultimately suffer from. Screen GCA patients for thoracic aortic aneurysm, a late complication that appears years afterward.
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