1. RHEUMATOID VASCULITIS Kamal Kolappa UNC Internal Medicine Morning Report 7.7.10

2. BACKGROUND  Rheumatoid Vasculitis (RV) is a rare complication of longstanding, severe Rheumatoid Arthritis (RA)  Estimated incidence in 2-5% of RA patients 1  Associated with chronic RA: Mean lag time 13.6 years between diagnosis of RA and onset of RV  Males are 2-4x more likely to develop RV than females  Characterized by Extra-Articular involvement of disease  Specifically the small and medium vessel arteries similar to polyarteritis nodosa  Correlated to high RF levels and low complement at onset of RV development; indicating uncontrolled RA disease as a risk factor 2  Anecdotal evidence that viral infections and drug reactions can precipitate RV occurrence in RA patients 3 RV cutaneous ulcer

3. DISEASE MANIFESTATIONS  Cutaneous Manifestations secondary to vascular compromise (90% of RV patients evidence this) 4  Digital ischemia to fingers and toes  Cutaneous ulcers resulting from obstruction of superficial and medium vessels  Nail fold infarcts  Nerve Infarction (involves vasa vasorum) causing mononeuritis multiplex  foot and wrist drop  Associated w/ neuropathy characterized by numbness, burning, pain that precedes muscle weakness, paralysis, and wasting  Ocular Scleritis  Non specific signs: Fever, Weight Loss Source: Up to Date

4. INVOLVEMENT OF LARGE ARTERIES  Classically, disease often limited to small and medium arteries; case reports of large artery involvement exist  Bowel 6  Renal  Brain (CVA’s)  Coronary Vasculitis (rare) 5  Focus back to Ms. R:  Extensive CVA w/o other leading cause (MCA distribution)  Hematuric evidence of possible Renal involvement  GG pulmonary opacities can be seen w/ pulmonary vasculitis  Large cecal perforation w/ bx proven vasculitic involvement CTA-Head +CTA Chest Of Ms. R

5. DIAGNOSIS OF RV  Evidence gathered from:  H&P: Suspect RV in any RA patient w/ fevers, weight loss, skin ulcerations, necrotic digits, or sx of sensory or motor nerve dysfxn  Labwork: specifically elevated RF 7, low complement, elevated ESR, elevated Anti-CCP (citrullinated peptides)  high odds ratio for possible RV in a person w/ h/o RA  Keep in Mind: No definitive Lab dx of RV  Imaging: Angiogram rarely useful as majority of vessels involved are medium (below image resolution); findings(segmental narrowing) are non- specific to RV  Full Thickness Skin Biopsy: As above, would show evidence of fibrinoid necrosis of vessels Fibrinoid Necrosis in vessel wall Source: Up to Date

6. DIFFERENTIAL DIAGNOSTIC CONSIDERATIONS  Cryoglobulinemia (Rx w/ Plex as opposed to immunosuppression Rx of RV) 7  Presents w/ palpable purpura, cutaneous ulcers, myalgias  Usually RF positive  Small vessel vasculitis of skin(purpura, pustules) usually not seen in RV as in Cryoglobulinemia  Polyartertis Nodosa (nearly indistinguishable from RV); key is clinical features, i.e. pt w/ strong hx of RA more likely has RV rather than PN  ANCA Vasculitides: Also RF positive  Wegener’s, Churg Strauss, Microscopic Polyangiitis  Vasculitis-like Syndromes  Thrombo-embolic phenomenon (cholesterol emboli)  Infectious Endocarditis (fever, skin lesions, active urine sediment)

7. TREATMENT OF RHEUMATOID VASCULITIS  Differs based on extent of involvement: Cutaneous vs. Systemic 8  Cutaneous Involvement  Isolated Nailfold Infarctions: secondary to low grade small vessel vasculitis  symptomatic Rx, low risk of progression to systemic vasculitis  Leg ulcerations: Rx ~venous stasis, i.e. wet to moist saline dressings, compression bandages, hydrogel occlusive dressings; Higher assocation w/ systemic RV  Systemic RV  High Dose Glucocorticoids (1-3 days of Solumedrol 1gram/day)  transition to PO Prednisone  Cytotoxic agent (e.g. Cyclophosphamide); Achieves disease remission; Alt: MTX, Azathoprione, TNF inhibitors

8. REFERENCES 1. Voskuyl AE et al. Factors associated with the development of vasculitis in rheumatoid arthritis: results of a case-control study. Ann Rheum Dis. 1996; 55:190 2. Scott DG et al. Systemic Rheumatoid Arthritis: a clinical and laboratory study of 50 cases. Medicine(Baltimore) 1981; 60:288-290 3. Iyngkaran P et al. Rheumatoid vasculitis following influenza vaccination. Rheum. 2003; 42: 907-909 4. Sayah A et al. Rheumatoid Arthritis: A review of cutaneous manifestations. J Am Acad Dermatol. 2005; 53: 191-193 5. vanl Albada-Kuipers et al. Coronary arteritis complicating rheumatoid arthritis. Ann Rheum Dis. 1986; 45:963-968 6. Pagnoux C et al. Presentation and outcome of gastrointestinal involvement in systemic necrotizing vasculitides: analysis of 62 patients with polyarteritis nodosa, microscopic polyangiitis, wegener granulomatosis, churg-strauss syndrome, or rheumatoid-associated vasculitis. Medicine (Baltimore) 2005; 84:115-116 7. Geirsson AJ et al. Clinical and serological features of severe vasculitis in rheumatoid arthritis: A clinicopathologic and prognostic study of thirty-two patients. Arhtritis Rheum. 1995; 55:190-193 8. Abel T et al. Rheumatoid Vasculitis: effect of cyclophosphamide on the clinical course and levels of circulating immune complexes. Ann Internal Medicine. 1980; 93:407-408

9. APPRECIATE YOUR ATTENTION! Special Thanks to my Med U team: Eric Edwards, Andy Mcwilliams, Chris Sayed, Ross, Tim and Damon, Crystal, Eric Allman, and Paul Dombrower aka Master P