Page 377 - The Vasculitides, Volume 1: General Considerations and Systemic Vasculitis

Ophthalmologic and Neuro-Ophthalmologic Aspects of Vasculitides 351

should be done on patients with MPA to aid in the evaluation of the patient and determine
appropriate treatment.

MPA is typically treated with corticosteroids and cyclophosphamide, and in severe cases
plasmapheresis and IVIG have shown to be beneficial. If renal function is preserved,
methotrexate may be added to induce remission. Maintenance therapy is recommended with
azathioprine [132]. A favorable response to treatment occurs in up to 79% of patients with 5-
year survival rates ranging from 65% to 80% [128, 133].

Since ocular features in MPA are rare, the effects of treatment on ocular disease have
been documented through single patient reports or small series. Mihara and co-workers [134]
described two patients, one of whom had hypopyon iridocyclitis in the right eye, and
ophthalmoscopy with retinal cotton-wool spots in the left eye, both of which responded to
oral prednisolone, topical instillation of 1% atropine sulfate and subconjunctival injections of
betamethasone.

The patient with bilateral scleritis and corneal infiltration responded to the betamethasone
treatment. Altaei and co-workers [129] described a patient with initial VA of hand movement
only in the right eye that improved to 6/9 in the right eye after 4 months of pulsed 100 mg/day
of methylprednisolone followed by 60 mg/day of oral prednisolone and 100 mg/day of
azathioprine.

Immune Complex Mediated

Hypocomplementemia Urticarial Vasculitis

HUV is a rare severe systemic form of urticarial vasculitis characterized by chronic non-
pruritic urticarial lesions, angioedema, ocular inflammation, arthritis or arthralgia, obstructive
lung disease, and glomerulonephritis. Its exact incidence is unknown, but it is twice more
common in women than in men, and its peak incidence is seen in the fifth decade of life
[135]. The histopathology of HUV is characterized by an interstitial neutrophilic infiltrate of
the dermis, and a necrotizing vasculitis with immunoglobulin or C3 deposits in the blood
vessels on immunofluorescence [136].

The diagnostic criteria for HUV were first described by Schwartz and colleagues [137].
The major criteria are urticaria for more than 6 months duration and hypocomplementemia.
The minor criteria, two of which are required for diagnosis, were dermal venulitis on biopsy,
arthralgia or arthritis, uveitis or episcleritis, mild glomerulonephritis, recurrent abdominal
pain, and a positive C1q precipitin test by immunodiffusion, with reduced circulating C1q
levels. Exclusion criteria included significant cryoglobulinemia, elevated anti-DNA antibody
titer, high titer of antinuclear antibody (ANA), hepatitis B virus (HBV) antigenemia,
decreased C-esterase inhibitor levels, and inherited complement deficiency. HUV and SLE
share many clinical features, thus it is important to note that COPD and uveitis are typically
found in HUV, but not SLE [138].

Ocular manifestations of HUV are found in up to 60% of patients. A study by Wisnieski
and colleagues [138] in 18 patients with HUV found that 11 (61%) patients had ocular
manifestations, 8 (44%) had conjunctivitis, episcleritis, and/or inflammation of the uveal
tract, while 3 (16.6%) had scleral inflammation and photophobia. Davis and colleagues [139]

Complimentary Contributor Copy