Page 50 - The Vasculitides, Volume 1: General Considerations and Systemic Vasculitis

26 David S. Younger

A meta-analysis of 15 studies based on a comprehensive review of the literature in the
Medline database from 1956 to 2007, and Cochrane Controlled Trials Registry among 15
studies and over 1300 patients [46] found that early treatment conferred a protective effect on
developing persistent renal disease (odds ratio [OR] .43) and the likelihood of surgical
intervention for abdominal pain (OR .75), as well as a statistically significant positive effect
on shortening the duration of abdominal symptoms (OR 5.42).

C1q (Hypocomplementemic Urticarial) Vasculitis
McDuffe and colleagues [83] later described several patients with recurrent attacks of

erythematous, urticarial, and hemorrhagic skin lesions that lasted 24 hours at a time,
associated with recurrent attacks of fever, joint swelling, abdominal distress, and depressed
serum complement. When tested by immunodiffusion against purified preparations RF and
human C1q, several patients reacted strongly with purified C1q. Skin biopsies showed
leukocytoclasia, characteristic of necrotizing vasculitis, anaphylactoid purpura, or mild
nonspecific perivascular infiltration. Immunofluorescence of skin specimens showed fixation
of Ig in the patient with necrotizing vasculitis. Renal biopsy showed mild to moderate
glomerulonephritis indistinguishable for those seen in other forms of chronic
membranoproliferative glomerulonephritis. The differences from SLE included more
urticarial and purpuric skin lesions, with relatively mild renal or absent and other visceral
involvement in the patients with HUV. An etiopathogenesis related to chronic vascular
inflammation resulting from deposits of immune complexes in small vessel walls seemed
likely. Zeiss and colleagues [84] characterized C1q IgG precipitins from HUV sera that
precipitated C1q in agarose gel among four additional patients. Wisnieski and Naff [85] later
showed C1q binding activity in IgG from HUV sera. Buck and colleagues [86] defined the
HUV syndrome as the presence of UV with multi-organ involvement notably arthralgia or
arthritis, angioedema, pulmonary, ocular, renal, and pericardial. However, anti-C1q
antibodies are elevated and the serum low C1q levels are reduced in virtually all children so
studied. Antihistamines are the drug of choice with cutaneous lesions to control itching, but
they may be insufficient in controlling the formation of immune complexes when given late
in the inflammatory cascade. There is yet a consensus on the most effective therapeutic
regimen; however plasmapheresis and intravenous immune globulin (IVIg) are alternative
immunosuppressant modalities.

Variable Vessel Vasculitis

Behçet Disease
Behçet [87] described the clinicopathological findings of a 28-year-old Turkish patient

with relapsing oral, genital and oral eruptions over 4 years, accompanied by severe headache,
memory loss, dizziness, lethargy, fatal seizures and coma. Postmortem examination showed
perivascular inflammatory cell infiltration of the meninges, brain, and central retinal artery
and optic nerve with necrotic cerebral lesions. The first well-documented American patient
with nervous system involvement of BD was described by Wolf and coworkers [88], namely
a 22-year-old woman with a five year history of recurrent oral and genital ulceration, and a
two year course of progressive visual loss, headache, hemiparesis, ataxia, tremor, dysarthria,
cranial nerve palsy, cerebellar and corticospinal tract disease, and mental deterioration, which

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