INTRODUCTION Granulomatous inflammation is a distinctive ...

Treatment
The therapeutic goals in WG have expanded dramatically over the past 30 years. Prolongation of
patient survival was the primary objective prior to the 1970s as 82% of patients with active WG
died within 1 year. Long-term survival became possible with the introduction of prednisone and
cyclophosphamide, although morbidity and mortality continued to occur as a result of treatmentinduced
toxicity and disease relapse (De Groot et al., 2001).
Glucocorticoids combined with cyclophosphamide or methotrexate are the only two regimens that
have thus far been shown to induce remission of active WG affecting a major organ. Patients with
alveolar hemorrhage, rapidly progressive glomerulonephritis, CNS disease, or other
manifestations that are immediately life threatening should initially be treated with
cyclophosphamide and glucocorticoids. Optimal treatment consists of daily intravenous or oral
cyclophosphamide (2 mg/kg/day). Monthly pulse intravenous cyclophosphamide is preferred
because of its better side effect profile, yet is insufficient to reliably induce remission, however it
may be employed to sustain remission. Cyclophosphamide treatment is generally maintained
through 1 year of stable remission. Daily prednisone, 1 mg/kg/day is routinely employed during
the first 6- 12 months. With this approach, marked improvement or partial remission was
achieved in 91% of patients and complete remission in 75%. The high morbidity rate associated
with this treatment (infections, hemorrhagic cystitis, secondary neoplasia) has led to an avid
search for alternative approaches. Methotrexate is successfully employed to induce remission in
patients who present with relatively more indolent disease and may be effective in sustaining
remission. (Hoffman et al., 1992) Azathioprine can maintain remission after induction with CYC
(Jayne, 2001).
The experience with other cytotoxic and immunosuppressive agents in the treatment of WG comes
solely from case reports and small series, and there remain insufficient data to assess efficacy.
These agents include cyclosporine, deoxyspergualin, intravenous immunoglobulin,
trimethoprim/sulfamethoxazole. Also new selective immunomodulatory agents are under trial
now, including alemtuzumab, etanercept, infliximab and rituximab (Carol, 2003).
Granulomatous angiitis
Figure 24. Wegener. Granulomatosis. T2-weighted axial MRI
shows hyperintense signal throughout much of the pons,
(Metwally, 2006-6)
Many forms of vasculitis may involve the CNS, including Behcet’s disease, polyarteritis nodosa,
vasculitis associated with connective tissue diseases such as lupus (systemic lupus erythematosis;
SLE), and others. Most of these diseases can produce vasculitis or other problems outside of the
brain, however in the description of intracranial granulomatous angiitis, it is a more specific type
of vasculitis in which the disease process is confined to the CNS and no known infection can be