Page 324 - The Vasculitides, Volume 1: General Considerations and Systemic Vasculitis

298 Elana J. Bernstein and Robert F. Spiera

from classical or definite RA, those with RV have a reduced survival compared to the general
population that is immediately evident and continues for up to six years [1, 2]. Vasculitis is a
particularly significant ExRA manifestation as it typically requires an escalation of
immunosuppressive therapy at a time when patients have significant disease-related tissue
damage and therapy-related complications. It is important to consider RV is highly suspected
patients because unrecognized and therefore untreated, there may be excess morbidity. This
chapter discusses the epidemiology, clinical manifestations, histopathology, and treatment of
RV.

Historical Aspects

The earliest reported patients with RA were children described in 1951 by Pirani and
Bennett [3] with particularly severe RA progressing from childhood. The first patient was ill
for four decades with numerous exacerbations and rare remissions with progressive limitation
of the joints and a diagnosis of Still disease that emerged into chronic disseminated RA
before he died. Postmortem examination was notable for severe deforming RA and ankylosis,
with left cerebellar hemorrhage, atrophy of bone, skin and muscle; healed glomerulonephritis
and marked inflammatory spinal arachnoiditis with splenic follicular necrosis. The second
patient presented at age 22 years after many years of disability unable to walk bedridden
when he died. Postmortem examination was notable for severe deforming RA and ankylosis
with widespread polyarteritis nodosa (PAN). A third patient developed progressive joint
deformities from age 17 months before fatal pneumonia. Postmortem examination was
notable for RA involving all joints and chronic aortitis. In the same year, Sokoloff and
colleagues [4] reported arteritis in small muscular arteries of muscle biopsy tissue in 5 of 57
(10%) adults with RA, the histopathology of which included, mononuclear and
polymorphonuclear cell adventitial infiltration, slight necrosis, endothelial cell injury, and
secondary elastic involvement without vascular thrombosis or aneurysm formation, and
frequent cardiac complication manifesting as pericarditis. The incidence of arteritis was
estimated to occur overall in about 8.8% of patients with RA. These observations were
followed by 10 additional patient descriptions in 1957 by Sokoloff and Bunim [5] of arteritis
in patients with RA. The age range was 30 to 65 years (mean 47 years), at presentation, with a
duration of arthritis of 6 months to 30 years (mean 9 years). The diagnosis of RV was
established at postmortem examination in five patients and by biopsy of muscle,
subcutaneous nodules, and synovial tissue antemortem in five patients. Postmortem
examination showed widespread arteritis in three patients, with isolated involvement of the
testis in one patient, and the sciatic nerve and coronary artery in another patient. Among the
five patients who were diagnosed antemortem, arteritis was detected in muscle biopsy tissue
in two patients, in early subcutaneous nodules in two others, and in synovial tissue of another
patient. In contrast to the earlier study conducted by the same author of vasculitic muscle
biopsy lesions in RA [4], the pathological features of the subsequent 10 patients (5)
resembled PAN. Two additional patients with RV were mentioned after publication of the
latter study by the same authors [5], both with necrotizing arteritis, in which one was
associated with arteritis of the pancreas, and another with isolated peripheral nerve vasculitis
(PNV).

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