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Vol. 29. Issue. 4.August 2009
Pages 285-378
Vol. 29. Issue. 4.August 2009
Pages 285-378
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Multiple masses as a presentation of Wegener's disease
Masas múltiples como presentación de enfermedad de Wegener
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Pablo Younga, Bárbara C Finna, Maria E Ceballosa, Martin Iturraspea, Mariano Forrestera
a Hospital Británico de Bueno Aires Bueno Aires Argentina, Argentina,
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Dear Editor:

Wegener¿s granulomatosis (WG) is a systemic vasculitis affecting vessels of small and medium diameter, the upper and lower respiratory tract, as well as the kidneys.1 Although rare, systemic vasculitis can present itself as lesions resembling tumours, which can initially delay diagnosis and treatment.2,3 We present the first such case of WG described in the literature, which presented with multiple masses in different locations.

37 year old woman who presented with weight loss, febricula, fatigue and night sweats. Symptoms had been present for six months. A physical examination revealed a palpable right supraclavicular mass and a mass on the abdominal wall at hypogastric level. The rest of the examination was normal. The laboratory showed a haematocrit of 23%, erythrocyte sedimentation rate (ESR) of 120mm in one hour (normal <20), with no microhaematuria or renal insufficiency. The chest x-ray showed a left apical mass which in the CT measured 6cm by 5cm with central necrosis, without cavitation, or hilar or mediastinal adenopathy. The antineutrophil cytoplasmic antibodies (ANCAs) were positive in high titres (810 AU, normal <10) and the enzyme immunoassay (ELISA) revealed specificity for the proteinase 3 antigen. A surgical biopsy of the right supraclavicular mass showed a necrotising granulomatous inflammation. Afibrobronchoscopy with bronchoalveolar lavage was performed, which showed up negative for neoplastic cells and infection; the transbronchial biopsy showed necrotising vasculitis. PPD and cultures were negative. Based on the above, the patient was diagnosed with WG. Treatment with cyclophosphamide 150mg/day and prednisone 60mg/day improved all manifestations. Disappearance of the pulmonary and hypogastric masses was observed. The patient remained asymptomatic after one year.

In the literature, 89 cases of vasculitis presenting as a mass have been described. 2,3 In all of these cases the mass was present in only one location or organ, unlike our patient. The average age in the cases described was 50.5 + 15.8 years, and 51% were female. In 82% of cases, the ¿tumour¿ was associated with constitutional symptoms and an elevated ESR. In approximately half of the patients, surgery was performed prior to diagnosis. The most frequent location for the masses was the breast (22%), followed by lesions in the central nervous system (16%). Another common location is the ovary (10%), caused by giant cell arteritis (GCA), and in the male, in the genitourinary system, caused by polyarteritis nodosa (PAN).

WG is the most common cause of vasculitic masses (32 cases), followed by GCA (18 cases) and PAN (17 cases), among others. WG is distinguished by the diversity of the locations in which the lesions (masses) are found, comprising twelve different positions (the most common being the breast and kidney). In patients with GCA, the masses are located only on the breast and ovary.

The purpose of this article is to draw attention to this unusual presentation of vasculitis. The improvement on old imaging methods, such as highresolution tomography or nuclear magnetic resonance, among others, and the arrival of ANCAs, can assist with preoperative diagnosis. The inclusion of vasculitis in the differential diagnosis of lesions or masses leads to an earlier correct diagnosis and, consequently, the establishment of an appropriate treatment, avoiding unnecessary surgery.

Bibliography
[1]
Shafiei K, Luther E, Archie M, Gulick J, Fowler MR. Wegener granulomatosis: case report and brief literature review. J Am Board Fam Pract 2003;16:555-9. [Pubmed]
[2]
Revital K, Yechezkel S, Hanan G. Systemic vasculitis presenting as a tumorlike lesion. Four case reports and an analysis of 79 reported cases. Medicine (Baltimore) 2000;79:349-59.
[3]
Roussou M, Dimopoulos SK, Dimopoulos MA, Anastasiou-Nana MI. Wegener¿s granulomatosis presenting as a renal mass. Urology 2008;71:547.e1-2.
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