1. Vasculitis Syndromes
Polymyalgia Rheumatica,Giant Cell Arteritis, Wegener’s Granulomatosis, Polyarteritis Nodosa

2. What is Vasculitis?
Disease characterized by inflammation of blood vessel walls, leading to altered blood flow through obstructed walls. This causes ischemia and tissue damage.
In addition there is an intense inflammatory rxn causing further systemic signs and symptoms
Can be fatal

3. You Should Suspect Vasculitis
Unexplained signs and sxs
Multisystem disease
Unexplained elevated ESR/CRP
Skin lesions (palpable purpura)
Ischemic vascular changes (Raynaud’s, gangrene, livedo, claudication)
Glomerulonephritis
Mononeuritis multiplex
Intestinal angina
Inflammatory ocular diease
Arthalgias/arthritis, myalgias
Sudden visual loss/headache

4. Select Vasculitides Polymyalgia Rheumatica
Giant Cell or Temporal Arteritis
Wegener’s Granulamatosis
Polyarteritis Nodosa

5. Polymyalgia Rheumatica (PMR)
Most ‘benign‘ of the group
Common: 50/100,000, age > 50, average age 75. Highest prevalence in northern European ancestry, females>>males
Cause unknown

6. PMR Clinical Presentation
Usually abrupt onset
Intense morning stiffness and pain that can last all day involving the shoulders and hip girdle area
No small joint involvement
Muscle strength normal
Fatigue and anorexia common
Elevated CRP and ESR; anemia of chronic disease, elevated platelets
15% get GCA (more later)

7. PMR Treatment Low Dose Steroids (10-20 mg/day)
The only drug that works
Look to normalize the CRP and ESR; if they continue to be elevated, rethink the dx (?paraneoplastic syndrome or GCA)
Usually self-limited: 65% of patients able to taper off Prednisone by 1 year, >85% in 2 years
Disease flares not uncommon as prednisone is tapered and may require dose adjustments

8. Giant Cell Arteritis Can occur exclusively but often seen with PMR
Rare: 15/100,000
Age >50
Cause unknown
Involves the medium/large blood vessels of the head and neck including the blood vessels that supply the optic nerve

9. GCA Pathophysiology
Unknown trigger causes inflammatory response with the release of IL-1 and IL-6.
This leads to systemic symptoms and the infiltration of inflammatory cells into the adventitia of the temporal and other involved arteries
Typical histologic pattern: Giant Cells

11. GCA Clinical Presentation
Variable
Scalp tenderness
Temporal headaches
Jaw Claudication
Sudden loss of vision
+/- PMR sxs
Rare- upper extremity claudication due to subclavian involvement
Constitutional sxs: FUO, wt loss, fatigue
Bounding OR absent temporal artery pulses
Rarely subclavian bruits

13. GCA Diagnostic Studies
Temporal Artery Biopsy is the gold standard
Elevated ESR and CRP, usually levels higher than in PMR
Anemia
Elevated LFTs not uncommon

14. Treatment of GCA
High dose Steroids (60 mg/day) is the only drug that works
Slow taper over time usually 1-2 years. Some patients require low dose (<10 mg/day) chronically

15. GCA Complications Blindness Scalp Necrosis Lingual Infarction
Aortic Dissection/Aneurysm
Complications from high dose steroids: osteoporosis, cataracts, elevated blood sugars, wt. gain etc.

16. Wegener’s Granulomatosis (WG)
Potentially fatal vasculitis involving small vessels
Rare: 3-14/million, more common in whites, any age but rare in children
Pathology shows necrotizing granulomas usually in upper airways, lungs and kidneys

17. WG Pathophysiology
Complex immunopathogenic events in which the production and activity of ANCAs (usually c-ANCA) play a central role. These autoantibodies interact with primed neutrophils to cause vascular injury and necrosis.
Histologic lesions show granulomas

18. WG Clinical Presentation
Variable, multisystem involvement
Organs:
Eyes: episcleritis/scleritis, proptosis due retro-orbital mass
CNS: rare mass lesion
Upper airway: otitis media, nasal chondritis, sinusitis with purulent drainage and epistaxis, ulcerations, subglottic stenosis
Kidney: neprotic syndrome, proteinuria, renal failure
Skin: palpable purpura due to leukocytoclastic vasculitis, pyoderma gangrenosum, panniculitis
Lung: cough, hemoptysis, hemorrhage, resp failure
Cardiac: pericarditis, conduction abnormalities
Systemic: fever, night sweats, wt loss, fatigue

19. Palpable Purpura

20. Eye Involvement

21. Face Involvement

22. WG Diagnostic Studies
Presence of c-ANCA (cytoplasmic staining pattern antineutrophil cytoplasmic antibodies + clinical picture is often enough to make the diagnosis. It is % of generalized WG.
If the c-ANCA is -, tissue biopsy of lung or kidney is recommended.
“Limited” refers to disease limited to the airways; c-ANCA often is -.

23. Additional labs Elevated CRP and ESR
Anemia, leukocytosis, & thrombocytosis
Elevated Cr
Active urine sediment with red cell casts, hematuria and proteinuria

24. WG Clinical Course/Progression
Prior to immunosuppression therapies, WG was uniformly fatal. Now survival rates almost 90% with aggressive treatment.
High dose steroids and Cyclophosphamide are cornerstone of therapy. Methotrexate or Azathioprine sometimes used as steroid sparing agents.

25. Polyarteritis Nodosa (PAN)
Medium vessel vasculitis
Can be caused by Hep B
5/million cases
Peak incidence 50’s & 60’s, slightly more common in males

26. PAN Pathophysiology
In Hep B assoc cases immune complexes play significant role
In non Hep B cases, the pathophysiology is less understood

27. PAN Clinical Presentation
Systemic: fever, fatigue, wt loss
Abdominal pain due to mesenteric angina/ischemia
Mononeuritis multiplex
Myalgias/arthalgias/mild arthritis
Hypertension
Skin: livedo reticularis, palpable purpura, fingertip ulceration, subcutaneous nodules
Testicular pain or tenderness

28. Livedo reticularis

29. Subcutaneous Nodules

30. Complications of PAN Chronic renal failure Bowel perforation
Stroke/cerebral hemorrhage due to HTN
Foot/wrist drop

31. Wrist Drop

32. Labs of PAN Elevation of acute phase reactants (ESR, CRP etc)
Absence of ANCA
Elevated transaminases, decreased albumin
+/- Hep B
Urine: proteinuria and hematuria without casts

33. Imaging Studies of PAN
Mesenteric and/or renal angiography is the test of choice
Biopsies seldom done

34. Angiogram

35. PAN Treatment High dose steroids and Cyclophosphamide
Methotrexate or Azathioprine is used as steroid sparing agents later once the disease is controlled
Treatment for Hep B with antivirals. Sometimes plasma exchange is used to remove immune complexes