Page 378 - The Vasculitides, Volume 1: General Considerations and Systemic Vasculitis

352 Sarah Conway and David S. Younger

found that 21% of patients had episcleritis or uveitis, while none with normocomplementemic
urticarial vasculitis had ocular manifestations. Iridocyclitis, so noted in two patients by
Corwin and Baum [140] was postulated to result from immune complex deposits. Thorne and
colleagues [141] described a 67-year-old-man with eye pain, redness, rash, arthralgia and
malaise later noted to have bilateral anterior scleritis without evidence of keratitis or uveitis,
and diagnosed with HUV. Mosawi and Hermi [142] described an 8-year-old boy with
conjunctivitis who later developed episcleritis. The diagnosis of HUV is ultimately based on
clinical presentation, laboratory findings, and tissue biopsy; however since ocular
manifestations of disease are so pervasive, Wisnieski and co-workers [138] suggested that
suspected patients should be screened for ocular inflammation with a slit lamp exam.

The treatment of HUV consists of dapsone and systemic corticosteroids, and nonsteroidal
anti-inflammatory medication (NSAID) for symptomatic arthralgia and arthritis, and
plasmapheresis in severe cases [135, 139]. Wisnieski and colleagues [138] studied 8 patients
with C1q/HUV with conjunctivitis, episcleritis, and inflammation of the uveal tract,
identifying three patients with scleral inflammation and noting that none developed chronic or
irreversible ocular injury. All responded to prednisone and ophthalmic steroids. Thorne and
colleagues [141] documented the response of scleritis to treatment in a 67 year-old-man with
bilateral scleritis, who responded to 1 mg/kg/day of prednisone 1 mg/kg daily and 30
mg/kg/day of mycophenolate mofetil in six months. The response of iridocyclitis to
cycloplegic and topical corticosteroids was shown by Corwin and Baum [140].

Cryoglobulinemic Vasculitis

Cryoglobulinemic vasculitis is a small vessel vasculitis involving the skin, joints,
peripheral nervous system and kidneys. The disease is more common in Southern Europe than
in Northern Europe or Northern America, and the prevalence of essential mixed
cryoglobulinemia is reported to be approximately 1 case per 100,000 people [143]. The mean
age of onset of CV is in the fifth to sixth decade of life, with an age at onset of 27 to 78 years,
and a female to male ratio of 1.3 to 3:1 [144-146]. The histopathology of CV is characterized
by a leukocytoclastic vasculitis, B-lymphocyte expansion and tissue B-cell infiltrates [146].

The 2012 Revised CHCC defined CV as a vasculitis with cryoglobulin immune deposits
affecting small vessels associated with serum cryoglobulins. Skin, glomeruli, and peripheral
nerves are often involved [3]. Ocular signs and symptoms are neither part of the diagnostic
criteria for CV, nor are they very common.

The first ocular manifestation of CV was likely described by Wintrobe and Buell [147] in
a patient suffering from multiple myeloma that had bilateral thrombosis of the central retinal
veins and visual impairment. Other ocular manifestations included anterior uveitis, scleritis,
and peripheral ulcerative keratitis. Ryan and colleagues [148] described a 42-year-old woman
with CV who had attacks of acute arthritis and urticarial lesions with painful red eyes and
photophobia, later found to have 4+ cellular infiltrates and flare on slit-lamp examination
consistent with anterior uveitis. Anterior uveitis was documented by Nicholson and Sobrin
[149] in the description of a 40-year-old man with severe anterior uveitis associated with type
II essential cryoglobulinemia. Scleritis and peripheral ulcerative keratitis were other
manifestations so noted by Kedhar and co-workers [150] in a 49-year-old woman with

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