Am Fam Physician. 2004 Apr 1;69(7):1727-1728.
A 78-year-old woman presented to the emergency department with complaints of malaise, decreased urine output, and lower extremity swelling for one month. She said that she looked “different” (Figure 1). She was treated for sinusitis two months earlier with ciprofloxacin and an antihistamine but had no improvement. Physical examination revealed a patient in no distress. She had bilateral pitting pedal edema. There was no rash, and the rest of the examination was unremarkable. Urinalysis showed protein 4+, white blood cell (WBC) count of 20 to 50 per high power field, and red blood cell count of 20 to 50 per high power field. Laboratory testing revealed a hemoglobin level of 8 g per dL (80 g per L); WBC count, 10,200 per mm3 (10.2 × 109 per L); platelet count, 220,000 per mm3 (220.0 × 109 per L); blood urea nitrogen level, 121 mg per dL (43.0 mmol per L); and creatinine level, 9.7 mg per dL (857.5 μmol per L). Serologic testing demonstrated an antineutrophil cytoplasmic antibody (ANCA) titer that was elevated at 1:64 (normal <1:16). Antiglomerular basement membrane antibody (anti-GBM) titer was negative. After stabilizing the patient with dialysis, a kidney biopsy was performed (Figure 2).
Based on the patient's history and physical examination, which one of the following is the most likely diagnosis?
A. Goodpasture's syndrome.
B. Polyarteritis nodosa.
C. Wegener's granulomatosis.
D. Cryoglobulinemic vasculitis.
E. Poststreptococcal glomerulonephritis.
The answer is C: Wegener's granulomatosis. The photograph of the patient shows a prominent depression of the nose (Figure 1) caused by the collapse of the nasal bridge. The accompanying kidney biopsy shows a crescent formation in the glomerulus (white arrows, Figure 2). These findings, in combination with a positive serology for ANCA, are highly suggestive of Wegener's granulomatosis, a vasculitic disease that predominantly affects small blood vessels.1 The classic form of the disease involves the upper airway, lungs, and kidneys. Some patients present with a limited form affecting only one of these sites, but may progress later to wider involvement.
Patients often present with nonspecific findings of fever, malaise, weight loss, arthralgia, and myalgia. Chronic rhinitis and sinusitis are common findings. Destructive changes in the upper airway may cause bloody or purulent nasal discharge and sometimes lead to the so-called saddle nose deformity seen in this patient, when damage to nasal cartilage is severe. Pulmonary symptoms are present in most patients and may include cough, dyspnea, hemoptysis, or chest pain. Pulmonary nodules with cavitations are sometimes seen on chest radiography, and pulmonary hemorrhage may occur. Renal involvement is variable, depending on the stage of disease. Patients with renal involvement have an active urine sediment, with proteinuria, white blood cells, red blood cells, and casts. Kidney biopsy shows segmental necrosis of the glomerulus with fibrin deposition (bright red areas seen within the glomerulus in Figure 2) and crescentic glomerulonephritis. Glomerulonephritis usually progresses rapidly and may lead to irreversible renal failure without treatment.
Serum antibodies that react with cytoplasmic components of neutrophils (ANCA) are present in most patients, especially those with active multisystem disease. The cANCA (cytoplasmic) pattern, where immunofluorescent staining of neutrophils is seen diffusely throughout the cytoplasm, is more specific for Wegener's granulomatosis. ANCA antibody alone, however, is not sufficiently accurate to make or exclude the diagnosis. Tissue biopsy of an affected site typically is indicated for confirmation, because treatment of the disease involves potentially toxic chemotherapeutic agents.
Goodpasture's syndrome is less likely in this patient because the anti-GBM serology was negative. This syndrome involves a more localized type of vasculitis that attacks pulmonary alveoli and the glomerulus. Pulmonary hemorrhage and acute nephritis typically occur, and the syndrome is difficult to distinguish from Wegener's granulomatosis until serologic testing is done.
Polyarteritis nodosa is a vasculitis of medium-sized blood vessels. It typically causes renal infarction, not crescentic nephritis. Skin findings such as purpura, petechiae, or ulcerated lesions are common. An asymmetric polyneuropathy also is seen frequently.
Poststreptococcal glomerulonephritis shows diffuse proliferation of endothelial cells on glomerular biopsy and does not have crescents. The onset of poststreptococcal glomerulonephritis often is abrupt, typically occurring one to four weeks after streptococcal pharyngitis or pyoderma. It is rare in patients older than 40 years.
Cryoglobulinemic vasculitis often is associated with hepatitis C infection. Almost all of the patients with this type of vasculitis have cutaneous findings, typically erythematous macules or purpuric lesions. Raynaud's phenomenon may be seen with exposure to cold, and some patients with severe disease even progress to gangrenous changes in the digits.
1. Mansi IA, Opran A, Rosner F. ANCA-associated small-vessel vasculitis. Am Fam Physician. 2002;65:1615–20.
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