Transcript Rheumatoid Vasculitis - UNC School of Medicine

RHEUMATOID VASCULITIS
Kamal Kolappa
UNC Internal Medicine Morning Report
7.7.10
BACKGROUND
 Rheumatoid Vasculitis (RV) is a rare
complication of longstanding, severe
Rheumatoid Arthritis (RA)
 Estimated incidence in 2-5% of RA patients1
 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
RV cutaneous ulcer
 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
factor2
 Anecdotal evidence that viral infections and
drug reactions can precipitate RV occurrence in
RA patients3
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
INVOLVEMENT OF LARGE
ARTERIES
 Classically, disease often limited
to small and medium arteries;
case reports of large artery
involvement exist
 Bowel6
 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
DIAGNOSIS OF RV
Fibrinoid Necrosis
in vessel wall
Source: Up to Date
 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 RF7, 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 nonspecific to RV
 Full Thickness Skin Biopsy: As above, would show
evidence of fibrinoid necrosis of vessels
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)
TREATMENT OF RHEUMATOID
VASCULITIS
 Differs based on extent of involvement:
Cutaneous vs. Systemic8
 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
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
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