Common and uncommon manifestations of wegener's granulomatosis.
Wegener’s granulomatosis (WC) is a multisystem disorder characterized by aseptic necrotizing granulomatous inflammation and vasculitis, which affects the upper and lower respiratory tracts and the kidneys. WG most commonly affects the head and neck region, followed by the lung, kidney, and eye (1,2). Other systemic manifestations include arthralgias, fever, skin lesions, weight loss, and peripheral neuropathy (1). The serum antineutrophil antibody (ANCA) test has been shown to be a useful marker for WG. ANCA produces two major immunofluorescence patterns with ethanol-fixed neutrophils: cytoplasmic or classical (C-ANCA) and perinuclear (P-ANCA) (3,4). C-ANCA, with proteinase 3 specificity, can be found in 84 % to 99 % of patients with active generalized WG, in 50% to 71% of patients in partial remission, and in 30 % to 40 % of patients in complete remission (5,6). Changes in C-ANCA titer may correlate with a change in disease status in only 64% of cases; therefore a rise in C-ANCA titer alone should not be considered adequate evidence of an impending clinical exacerbation (7). Most P-AN&Is are directed against myeloperoxidase, and they are found most commonly in patients with idiopathic necrotizing and crescentic glomerulonephritis, regardless of the presence or absence of associated systemic vasculitis (8,9). Whereas C-ANCA is not entirely specific for WG, it is a valuable diagnostic tool, especially in cases where the traditional clinical and or pathologic criteria are not met. The combination of cyclophosphamide and prednisone is effective treatment for WG, achieving a high rate of remission (10). However, recognition of the complications of cytoxan and prednisone has led to investigation of other forms of therapy including bactrim, pulse cyclophosphamide, and methotrexate (1,ll).