Page 328 - The Vasculitides, Volume 1: General Considerations and Systemic Vasculitis

302 Elana J. Bernstein and Robert F. Spiera

by endarteritis in despite high-dose corticosteroids and cyclophosphamide therapy, and an
acute abdomen with sepsis and gangrenous toes and cutaneous ulcers. Altogether, among the
32 patients, all had evidence of PNV accompanied by cutaneous vasculitic involvement in 12
patients, 4 of whom had involvement of the heart, and two with concomitant vasculitic
involvements of nerve, skin, and eye. Four patients had PNV and kidney involvement, and
two others had lung involvement.

Vollerstein and colleagues [1] studied 52 patients with RV at the Mayo Clinic from 1974
to 1981 who developed clinical vasculitis evidenced by classic ischemic skin lesions, MNM,
or a positive tissue biopsy in comparison to population controls. The initial manifestation of
vasculitis was seen in skin in 26 (50%) patients; in nerve tissue in 20 (38%) patients, and
both in 3 (6%) patients. Serositis, pulmonary or renal manifestations were present each in 6
patients (12%); while 3 (6%) patients had eye findings. Mononeuritis presented in 2 (4%)
patients, while MNM respectively involving two, three, or four nerves were noted in 9 (12%),
5 (10%), or 4 (13%) patients. More than 90% of tissue biopsy specimens revealed vascular
necrosis and inflammation.

Serological Studies

Among 50 patients with RV summarized by Scott and colleagues [19], the IgM
rheumatoid factor (RF) was positive in 94% of patients, the C1q assay was abnormal in 65%
of patients, and ANA titers were raised in 59% of patients; and the immune complex assay
was positive in 50% overall, with abnormally reduced C3 and C4 complement levels
respectively in 0.73% and 0.2% of patients. Serological findings were not mentioned by Scott
and Bacon [9] in their report of 45 patients with RV treated with cyclophosphamide and
methylprednisolone or other regimens.

In the series of 32 patients with RV reported by Puéchal and colleagues [35] in whom
vasculitis was obtained in cutaneous nerve or muscle biopsy tissue specimens, articular
erosions were noted in all patients, RF seropositivity was demonstrated in 97% of patients,
antinuclear antibody (ANA) antibody elevation was noted in 27%, decreased C4 complement
titers in 62%, circulating immune complexes in 66%, and cryoglobulins in 13% of patients so
studied. At onset of the disease, the erythrocyte sedimentation rate (ESR) was a mean of 80
mm/hour. Hepatitis B surface antigen was not detected in any of the 25 sera so studied,
however one patient tested positive for hepatitis B surface antibody; there was no data
available for hepatitis C seropositivity.

In the series of 52 patients with RV summarized by Vollerstein and colleagues [1], the
ESR was elevated in 44 of 51 (86%) patients; the ANA was positive for low titers in 15 of 43
(35%) so stated; cyroglobulins were present in 1 of 28 (4%); immune complexes were present
in 3 of 8 (38%); and C3 and C4 complement was abnormally reduced in 1 of 31 (3%) and 7 of
29 (24%) patients so studied. There is no data on the serological evaluation of the subjects
reported by Scott and Bacon [9].

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