#61: Vasculitis and Giant-Cell Arteritis: ‘Rheum’ for improvement

October 9, 2017 | By Matthew Watto, MD

Keep your cool in the face of inflammation, and make the path to vasculitis diagnosis less tortuous with Dr. Rebecca Sharim, Rheumatologist and Assistant Professor of Medicine at Temple University. In this episode, we go with the flow from large vessel to small vessel vasculitides, and then learn how to make the diagnosis and management of Giant Cell Arteritis (GCA) and polymyalgia rheumatica (PMR) less of a headache. Correspondent, Dr. Bryan Brown cohosts!

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Case from Kashlak Memorial:  A 75 year-old woman with a PMHx of hypertension presents to the ED with three days of worsening left sided headache, now with left sided vision loss during a Norwegian folk festival. On review of systems, she also endorses a week of soreness of her shoulders and hips. This has never happened to her before.

Clinical Pearls:

1. Organizing types of Vasculitis:  “Chapel Hill Criteria” categorized by size of blood vessel involved e.g. large, medium or small. There is often “variable” vessel size involvement with considerable overlap; Large vessel: Takayasu, GCA; Medium vessel: Polyarteritis nodosa (PAN), Kawasaki; Small vessel: split into three broad categories 1) ANCA associated, 2) Immune complex mediated 3) Other
2. Types of small vessel vasculitis: ANCA associated: microscopic polyangiitis, eosinophilic granulomatosis with polyangiitis aka Churg-Strauss, granulomatosis with polyangiitis aka Wegener’s granulomatosis; immune-complex mediated: cryoglobulinemic vasculitis, Henoch–Schönlein purpura; and others: Lupus, rheumatoid arthritis, drugs, medications, cancer.
3. To guess the signs and symptoms of a given vasculitis, ask: What organs and what body parts are affected by those vessels? Then imagine what could happen from loss of blood supply (See Figure 2)
4. Systemic Symptoms (Sx) in vasculitis: Fevers, fatigue, anorexia, weight loss, night sweats. Sx will not be subtle!
5. Findings by Vessel size:
   1. Large vessel: Blood pressure difference in extremities, upper extremity claudication, aortitis, aortic aneurysm
   2. Medium vessels: supply gut → abdominal pain, testes → pain, infarct
   3. Small vessels: Skin → causing palpable purpura. Nerves → Mononeuritis multiplex/neuropathy. Kidneys → Glomerulonephritis. Eye → retinopath
6. Basic lab workup: CBC, BMP, LFTs, UA (for protein, hematuria), ESR +/- CRP, TSH, SPEP
7. Workup, how broadly to check labs: “There are no labs specific to a large vessel vasculitis.” Large vessel symptoms don’t usually warrant a big workup for small vessel vasculitis e.g. ANCAs. Check for syphilis if aorta involved. Small vessel symptoms justify a much broader workup for underlying autoimmune diseases, infections, etc. Order basic labs (above) and refer to rheumatology.
8. Giant cell arteritis (GCA) definition: Most common systemic inflammatory vasculitis in older adults with systemic, neurologic, and ophthalmic complications frequently seen. Involves large and medium sized vessels, particularly those coming off aorta: subclavian, axillary, vertebral, temporal, ophthalmic. Manifestations depend on vessels involved/damaged. Diagnosis = clinical = systemic sx + signs/sx of inflammation large vessels. Remember: Systemic sx will not be subtle e.g. extreme fatigue!
9. GCA versus Temporal Arteritis: Often used interchangeably, but GCA is a broad category and does not always involve the temporal artery!
10. Erythrocyte Sedimentation rate (ESR or sed rate): Value often >100. Over 100 is very remarkable w/differential diagnosis = vasculitis vs infection vs malignancy. ESR usually at least over 50 in GCA, but normal values increase w/age. To correct ESR for patient’s age = (age/2) +10 for women and age/2 for men. E.g. 80 yo ESR cutoff = 80/2 + 10 = 50 or above if female OR 80/2 = 40 or above if male.
11. GCA can present variably: On one hand, classic temporal arteritis: jaw claudication, headaches, vision changes, etc. Others have just systemic complaints- anemia, thrombocytosis, transaminitis, feeling terrible.
12. Jaw claudication: pain that worsens as patient chews, not just pain when patient chews. Consider the “Chewing gum test”: Reported in literature (NEJM), but not routinely used. Jaw claudication elicited after 2-3 minutes of gum chewing.
13. Role of temporal artery biopsy: Dr. Sharim recommends. Sufficient segment (at least 1-2 cm) is required due to skip lesions. Biopsy identifies 85 to 95% of cases (Cornelia NEJM 2014). Nice to be confident of diagnosis, given the morbidity of the steroid treatment once you commit. Biopsies remain positive at least one to two weeks, up to a month after initiating steroids, so don’t worry about initiating steroids prior to biopsy.
14. Treatment of GCA: Prednisone 60 mg per day or 1 mg/kg/day, followed by long slow taper over 1-3 years. Start steroids if concern. Can always stop them, but urgent rheumatology referral (w/in 24 hours) required +/- prompt ophthalmology referral depending on presence/absence of visual symptoms.  
15. Treatment of Polymyalgia Rheumatica: Use 15-20 mg daily if no concurrent GCA. Slow taper over 1-3 years.
16. Considerations with long steroid therapy: 1) Remember to screen for bone density. A bisphosphonate or other agent might be indicated. This is often missed. Check the ACR guidelines on steroid-induced osteoporosis 2) PCP prophylaxis technically indicated at prednisone doses >20mg/day, though few rheumatologists routinely prophylaxing in GCA patients 3) Watch out for hyperglycemia, or new or worsened diabetes 4) Glaucoma patients should be monitored for intraocular pressure changes
17. Don’t be afraid to call your specialist. Pick up the phone and call to expedite and discuss
18. A good review of systems is key in rheumatology

Goal: Listeners will learn a general approach to types of vasculitis, when and how to incorporate them into a differential, and how to work up and initiate management for giant-cell arteritis and polymyalgia rheumatica.

Learning objectives:
After listening to this episode listeners will…

1. Have an organized framework for types of vasculitis
2. Predict typical vasculitis symptoms by vessel involvement.
3. Recall the clinical presentation of Giant Cell Arteritis and polymyalgia rheumatica, including how it fits into a differential for headache or vision loss.
4. Recognize the uses and limitations of lab evaluation for GCA and other vasculitis, including sedimentation rate interpretation.  
5. Recognize the basic tenets of GCA treatment, including steroid and non-steroid agents.
6. Recall the complications of long-term steroid treatment

Disclosures: Dr. Sharim reports no relevant financial disclosures.

Time Stamps
00:00 Intro
03:07 Picks of the week
09:13 Getting to know our guest
15:00 Clinical case of vasculitis
15:59 Defining and classifying vasculitis
20:55 Workup for suspected vasculitis
23:17 How to explain GCA to a patient
25:08 Typical symptoms of vasculitis
28:00 Chewing gum test
29:34 Interpreting ESR
32:54 Basic exam and lab workup for vasculitis
35:23 Headache and suspicion for GCA/temporal arteritis
38:10 Is a temporal artery biopsy still mandatory?
39:20 Polymyalgia rheumatica
40:59 Steps to take when GCA/temporal arteritis suspected in clinic
43:55 PCP prophylaxis with high dose steroids
46:30 DMARDs and steroid sparing agents
48:12 Imaging studies to aid in diagnosis of GCA
50:50 Complications of long term steroid therapy
52:31 Take home points
53:26 Outro

Links from the show:

1. Bryan’s music youtube channel: youtube.com/bbrizzy89
2. T2 Trainspotting sequel, 2017 film: http://www.imdb.com/title/tt2763304/?ref_=nv_sr_2
3. Okja, 2017 film: http://www.imdb.com/title/tt3967856/
4. Ted talk by Ray Dalio: https://www.ted.com/talks/ray_dalio_how_to_build_a_company_where_the_best_ideas_win
5. Stuart’s anti-pick of the week: The Good Doctor http://www.imdb.com/title/tt6470478/
6. Jennette, J.C. “2012 Revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides” Arthritis & Rheumatism 2013; 65:1 pp 1–11. http://medecine.ulb.ac.be/ftpupload/Chapel-Hill%202012.pdf
7. Kuo, Chih Hung, et al. “Chewing Gum Test for Jaw Claudication in Giant-Cell Arteritis.” N Engl J Med 2016; 374:1794-1795. http://www.nejm.org/doi/full/10.1056/NEJMc1511420
8. Buckley, Lenore et al. “2017 American College of Rheumatology Guideline for the Prevention and Treatment of Glucocorticoid-Induced Osteoporosis.” Vol. 69, No. 8, August 2017, pp 1521–1537: https://www.rheumatology.org/Practice-Quality/Clinical-Support/Clinical-Practice-Guidelines/Glucocorticoid-Induced-Osteoporosis
9. Cornelia, Weyand and Jörg J Goronzy. “Giant-Cell Arteritis and Polymyalgia Rheumatica.” N Engl J Med 2014; 371: 50-57. http://www.nejm.org/doi/full/10.1056/NEJMcp1214825
10. Stone, et al. “Trial of Tocilizumab in Giant-Cell Arteritis.” N Engl J Med 377.4 (2017): 317-328. http://www.nejm.org/doi/full/10.1056/NEJMoa1613849