Wegener's Granulomatosis With Peripheral Eosinophilia: Atypical Variant of a Classic Disease

doi:10.1378/chest.104.4.1290

Chest

Volume 104, Issue 4, October 1993, Pages 1290-1292

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A patient with Wegener's granulomatosis (WC) diagnosed by ultrasound-guided transthoracic biopsy of a pulmonary nodule is reported. The case is atypical because of marked eosinophilia in the peripheral blood and the pleural effusion. The granulomatous infiltrate of the lung showed the classic picture of WG without eosinophils. The patient responded dramatically to treatment with steroids and cyclophosphamide. This variant form of WG poses problems in its distinction from Churg-Strauss syndrome, and the differential diagnosis between these two entities is discussed.

Section snippets

Case Report

A 43-year-old man was admitted to the hospital because of ankle arthritis. He had been well until four weeks earlier, when pain and swelling developed in the ankle joints. The patient smoked one pack of cigarettes daily for 20 years and lost 16 kg in weight during the year before hospitalization.

On admission, his temperature was 36.6°C. Examination of the lung revealed a right pleural effusion. The left ankle was swollen, warm, and tender. Moderate effusion and a few petechial lesions were

Discussion

The diagnosis of WG in our case was based on the finding of upper and lower respiratory tract involvement, renal disease, variable degrees of disseminated vasculitis involving joints, skin, and nervous system, and a classic pathologic picture by lung biopsy. The patient had no history of allergy, asthma or bronchospastic disorder. The pulmonary findings in the case presented include subpleural cavitary mass and marked eosinophilic pleural effusion. Pleural involvement in the form of a pleural

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Citation Excerpt :
Churg-Strauss syndrome (CSS), among the vasculitides, is the disorder that is associated with high-grade, persistent eosinophilia (see Wechsler and colleagues for fuller treatise). Although mild eosinophilia is common, marked eosinophilia is uncommon in many of the other vasculitides but has been seen in patients with cutaneous necrotizing vasculitis [117–119], thromboangiitis obliterans with eosinophilia of the temporal arteritis [120], and unusual cases of Wegener's granulomatosis [121,122]. The principal cardiac sequela of eosinophilic diseases is damage to the endomyocardium (see the article by Ogbogu and colleagues elsewhere in this issue).
Hyperosinophilic syndromes (HES) are a group of heterogeneous disorders many of which remain ill-defined. By definition, the HES must be distinguishedfrom other disorders with persistently elevated eosinophilia with a defined cause. Although marked eosinophilia worldwide is most commonly caused by helminth (worm) infections, the diagnostic approach must include noninfectious (nonparasitic) causes of marked eosinophilia as well.
Eosinophilia in Wegener's granulomatosis
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Significant eosinophilia is a prominent feature in Churg-Strauss syndrome but has only rarely been described in Wegener's granulomatosis (WG). We describe two Wegener's granulomatosis patients with > 30% eosinophilia on their initial presentations. Other etiologies that could account for their eosinophilia were excluded. Both patients had pulmonary alveolar hemorrhage, sinusitis, arthritis, high-titer cytoplasmic antineutrophil cytoplasmic antibodies (cANCA), and proteinase-3 antibodies, but no evidence of renal disease. Herein we discuss eosinophilia, the differential diagnosis of pulmonary infiltrates and eosinophilia, the role of cANCA in vasculitis and autoimmune disease, compare Wegener's granulomatosis and Churg-Strauss syndrome, and review possible pathogenic mechanisms.
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Chest

Selected ReportsWegener's Granulomatosis With Peripheral Eosinophilia: Atypical Variant of a Classic Disease

Section snippets

Case Report

Discussion

Chest

Am J Kidney Dis

Hum Pathol

The spectrum of vasculitis: clinical, pathologic immunologic and therapeutic considerations

Ann Intern Med

Selected Reports
Wegener's Granulomatosis With Peripheral Eosinophilia: Atypical Variant of a Classic Disease