Differential diagnosis
Differential diagnosis of a pulmonary abscess like lesion in an imaging test should mainly include infectious diseases (mixed bacterial etiology, tuberculosis, Pneumonocystis carinii pneumonia, nocardia, actinomycosis, fungi, and others) or diseases of non-infectious causes like neoplasms, vasculitis or lung artery embolism.

The presence of signs and symptoms from the upper respiratory tract such as relapsing otitis, rhinitis, and sinusitis accompanied by bloody discharge led to the conclusion that a systemic disease was the initial cause of the patient's symptoms and signs.

Diagnosis
The diagnosis was suggested by a significantly increased titer of the cytoplasmic-type antineutrophil cytoplasmic antibody (c ANCA = 65.3 units, normal value up to 30 units) and was confirmed by histopathological examination of excised nasal mucosa specimens.

The diagnosis was Wegener's granulomatosis.

Therapy
The initial treatment includes high doses of corticosteroids and cytotoxic agents (e.g. cyclophosphamide) for periods of 3 to 6 months. Once the disease has been controlled, the focus of therapy shifts to maintaining disease remission with lower doses of corticosteroids and less toxic alternatives to cyclophosphamide (e.g. methotrexate or azathioprine) for periods lasting from 12 to 18 months or longer.

Useful remarks

• The biopsy specimen was taken from the region with prominent symptoms and the easiest access.
• Early diagnosis of Wegener's granulomatosis can prevent renal involvement and finally death from renal failure.
• Clinical evidence shows that chronic nasal carriage of the bacterium Staphylococcus aureus is approximately three times higher in patients with Wegener's granulomatosis and that Staphylococcus aureus is a risk factor for disease relapse. Prophylactic treatment with co-trimoxazole reduces the incidence of disease relapses.

References

1. Stone JH. Wegener's Granulomatosis Etanercept Trial Research Group. Limited versus severe Wegener's granulomatosis: baseline data on patients in the Wegener's granulomatosis etanercept trial. Arthritis Rheum. 2003;48:2299-309.
2. Kariv R, et al. Systemic vasculitis presenting as a tumorlike lesion. Four case reports and an analysis of 79 reported cases. Medicine. 2000;79:349-59.
3. Allewelt M, Lode H. Diagnosis and therapy of abscess forming pneumonia. Ther Umsch. 2001;58:599-603.
4. Mayberry JP, et al. Thoracic manifestations of systemic autoimmune diseases: radiographic and high-resolution CT findings. Radiographics. 2000;20:1623-35.
5. Paulsen JI, Rudert H. Manifestations of primary vasculitis in the ENT region. Z Rheumatol. 2001;60:219-25.
6. Rasmussen N. Management of the ear, nose, and throat manifestations of Wegener
   granulomatosis: an otorhinolaryngologist's perspective. Curr Opin Rheumatol. 2001;13:3-11.
7. Ahmad I, et al. Localised Wegener's granulomatosis in otolaryngology: a review of six cases. ORL J Otorhinolaryngol Relat Spec. 2000;62:149-55.
8. Vacchi Suzzi M, Frasca G. Clinical significance of "ANCA" in the diagnosis of Wegener's granulomatosis: 8 years of experience. Acta Otorhinolaryngol Ital. 1998;18:239-48.
9. Ropa E, et al. Staphylococcus aureus and Wegener's granulomatosis. Arthritis Res. 2002; 4:77-9.
10. Regan MJ, et al. Treatment of Wegener's granulomatosis. Rheum Dis Clin North Am. 2001;27:863-86.

Acknowledgements

1. This case was prepared for our website by E. S. Rosmarakis, MD and P. I. Vergidis, MD.
2. A modified version of this case report was accepted for publication in the journal American Family Physician [Rosmarakis ES, Kapaskelis AM, Rafailidis PI, Falagas ME. Wegener's syndrome presenting as unresponsive pulmonary infection. American Family Physician 2004 (in print)]
3. We thank G. Dokianakis, MD, and D. Vasilopoulos, MD for their contribution in the management of this case.