Page 69 - The Vasculitides, Volume 1: General Considerations and Systemic Vasculitis

Overview of Primary and Secondary Vasculitides 45

another; and two patients, one of whom had fatal sepsis and another metastatic bladder cancer
as a consequence of cyclophosphamide toxicity. Recommendations for the treatment of
NSPNV [210] include prednisone monotherapy unless there is rapidly progressive neuropathy
at the dose of 1 mg per kilogram per day, with tapering over one year to a low dose.
Combination therapy employing cytotoxic drugs include Cyclophosphamide de,
methotrexate, and azathioprine. Other agents such as IVIg may be effective as adjunctive
therapy. Careful monitoring should be performed to observe desired therapeutic responses
and to avoid potentially serious drug side effects.

Laboratory Evaluation

Most experts concur with the following three principles to guide the evaluation and care
of patients. First, vasculitis it is a potentially serious disorder with a propensity for permanent
disability owing to tissue ischemia and infarction. Second, undiagnosed and untreated, the
outcome of vasculitis is potentially fatal. Third, a favorable response to an empiric course of
immunosuppressive and immunomodulating therapy should not be considered a substitute for
the histolopathologic confirmation of vasculitis in involved organ. Table 2 lists useful
laboratory studies that should be considered in patients with suspected primary systemic and
nervous system vasculitides.

Table 2. Laboratory Evaluation of Systemic and Nervous System Vasculitides

Studies in Blood, Urine and Body Fluids
CBC, chemistry panel, ANA, screening ANCA by IF and more specific ANCA ELISA serology specific
for PR3 and MPO; ESR, CK, T- and B-cell subset panel, circulating IC, acute and convalescent viral,
retroviral, bacterial, fungal, TB, syphilis and Lyme serology; quantitative immunoglobulins, IFE, C1q,
complement proteins, RF, cryoglobulins, anticardiolipin, aPL, and dsDNA antibodies; and appropriate
HLA haplotypes.
Urine spot and 24 hour collection for chemical and cellular microscopic analysis.
Cerebrospinal fluid analysis for protein, glucose, cell count, IgG level, oligoclonal bands, cytology,
VDRL, bacterial gram stain and culture; India ink, Cryptococci antigen and fungal culture; acid-fast and
TB culture; viral encephalitis panel for real-time analysis of DNA and RNA viruses by real-time PCR;
Lyme and HIV1 serology.
Radiological Studies
Screening color Doppler ultrasonography of the temporal arteries and great vessels.
3-T MRI and high field MRA or CTA, and DSA of vascular beds and major vessels.
18FDG body PET-CT.
Nuclear medicine cerebral perfusion (brain) SPECT.
Histopathological Studies
Punch skin biopsy of the distal leg and thigh for ENF density and histology employing PLP 9.5, with IF of
vessel walls and microscopic analysis for leukocytoclasia.
Bronchoscopy or needle tissue biopsy of lung lesion.
Endoscopic kidney biopsy.
USG-guided temporal artery biopsy.
Nerve and muscle biopsy for epineurial and epimysial vasculitic foci.
Leptomeningeal and brain biopsy for vasculitic foci in arteries and veins.
Abbreviations: ANA, antinuclear antibody; ANCA, antineutrophil cytoplasmic antibody; aPL,

antiphospholipid; CBC, complete blood count; CK, creatine kinase; CT and CTA, computed

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