Page 327 - The Vasculitides, Volume 1: General Considerations and Systemic Vasculitis

Rheumatoid Arthritis Vasculitis 301

Clinic and Sweden, the HLA-C3 allele was positively associated with RV (allele frequency
0.411 in RV patients versus 0.199 in RA controls without ExRA (p-value < 0.001) [24].

Clinical Presentation

Rheumatoid vasculitis can manifest in a variety of ways including, deep cutaneous ulcers
[26], digital gangrene [27], nail fold infarcts [28], MNM [29], and scleritis [30, 31]. Systemic
vasculitis commonly extends beyond the peripheral nervous system (PNS) and skin, to
internal organs including, the stomach, heart, intestine, pancreas, kidney, and gallbladder [17,
32- 34]. A postmortem series of 81 Japanese patients with RA showed histologically evident
necrotizing arteritis in 25 (30.8%) patients; however there was no mention of the frequency of
clinically significant antemortem vasculitic disease. Therefore, the true prevalence of
clinically significant arteritis in this cohort might have been significantly less than 30.8%.

Scott and colleagues [19] reported the clinical features of 50 patients with RV based
clinically on the development of deep cutaneous ulceration, acute peripheral neuropathy,
mononeuritis or MNM; peripheral gangrene or severe systemic disease in the presence of
typical digital or nail-fold infarcts. So defined, the clinical manifestations of RV in the 50
patients included cutaneous features of digit infarcts, ulcerations, purpura, and gangrene noted
in 44 (88%) of patients; rheumatoid nodules in 43 (86%); systemic features of weight loss,
hepatomegaly and splenomegaly in 41 (82%); sensory or motor neuropathy in 21 (42%);
cardiac involvement including pericarditis, arrhythmia, aortic incompetence, and myocardial
infarction in 17 (34%); pulmonary involvement including fibrosing alveolitis, pleurisy,
effusions and lung nodules in 17 (34%); renal involvement due to amyloid, chronic renal
failure, proteinuria and hematuria in 12 (24%); ophthalmic manifestations of scleritis in 7
(14%) of patients; and gastrointestinal features in 5 (10%) of patients. In the subsequent study
by Scott and Bacon [9] three years later, nail infarcts were noted in 78% and 70% of patients
respectively receiving cyclophosphamide and methylprednisolone versus other treatments; leg
ulcers in 435 and 33%, peripheral gangrene in 14% and 8%, neuropathy in 52% and 25%,
cardiac involvement in 34% and 18%; ophthalmic manifestations in 14% and 12%; and
rheumatoid nodules in 90% and 71% of patients. So defined, the diagnosis of vasculitis was
noted in 57% of patients treated with cyclophosphamide and methylprednisolone versus 46%
of those receiving other treatments.

Puéchal and colleagues [35] studied RV among 32 patients defined by the RA and
histologically-proven necrotizing vasculitis in cutaneous nerve or muscle biopsy tissue
specimens so noted respectively in 4 (14%) and 10 (36%) of patients, and together in 14
(50%). The clinical presentation of their patients included 5 (14%) patients with
mononeuritis, 18 (51%) with MNM, and 12 (34%) patients with distal symmetrical sensory or
sensorimotor neuropathy. Cutaneous lesions were seen in 12 (38%) patients including,
purpura in nine, ulcers in six, nail-edge infarcts in four, livedo in four, gangrene in two, and
one patient each with maculopapular rash or bullous rash. Cardiac involvement was
evidenced in 4 (12.5%) patients including, pericarditis, fatal rupture of a small mitral valve,
congestive heart failure, and myocardial infarction. Eye involvement was noted in 2 (6%)
patients including scleritis and scleromalacia. Gastrointestinal involvement was noted in 2
(6%) patients that was the cause of death in both due to small bowel perforation and necrosis

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