Initial laboratory studies revealed a white blood cell count of 10.7×10⁹/L (4.4 to 11.0×10⁹/L), hemoglobin level of 111 g/L (normocytic, normochromic) and platelet level of 599×10⁹/L. Electrolytes and liver enzyme levels were normal and her creatinine level was 98 pmol/L. The erythrocyte sedimentation rate was 125 mm/h. Urinalysis showed many red blood cells with no white blood cells or casts, and prote- inuria of 0.27 g/day. Her estimated creatinine clearance was 70 mL/min.

Esophagogastroduodenoscopy two days after admission revealed multiple discrete and punched out ulcers starting at 20 cm and extending to the esophagogastric junction (Fig­ure 1). The ulcers were atypical for ulcerative esophagitis and appeared vasculitic in nature. Because of the endoscopic findings, prednisone therapy was initiated. Within 24 h, im­provement was noted in her gastrointestinal symptoms, as well as the ulcerations in the nose and oropharynx. Esophag- eal biopsies revealed fibrinoid necrosis with ulceration and inflammatory cell infiltration surrounding small vessels, in keeping with a vasculitic process (Figure 2). Biopsy of a skin lesion showed fibrinoid degradation of small arteries with acute inflammatory cell infiltration (Figure 3). Granulomata were not noted in either biopsy.

Figure 1) Esophagoscopy showing punched out ulcerations extending from 20 cm to the esophagogastric junction

Further laboratory studies revealed negative tests for anti- nuclear antibody, cryoglobulins, cold agglutinins and hepati­tis B surface antigen. C3 and C4 levels were normal, and rheumatoid factor was positive at 32 U/mL. Measurement of cytoplasmic antinuclear antibody (c-ANCA) was positive at a titre of 1:160.

Figure 2) Photomicrograph of an esophageal biopsy showing fibrinoid necrosis, ulceration and inflammatory cell infiltration surrounding small vessels consistent with a vasculitic process

Wegener’s granulomatosis was diagnosed on the basis of ear, nose and throat involvement (otitis media, nasal and oral ulcerations), kidney involvement (hematuria, protein­uria and renal insufficiency), positive c-ANCA and biopsies consistent with a small vessel vasculitis. Treatment was initi­ated with cyclophosphamide 100 mg once daily and predni- sone 60 mg once daily. This resulted in resolution of the ischemic areas and vasculitic lesions on her arms, fingers and feet. Her renal function stabilized and fell to 20.
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Figure 3) Photomicrograph of a skin biopsy showing fibrinoid degradation of small arteries with acute inflammatory cell infiltration