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Dorsal prevertebral lesions in Wegener granulomatosis: report on four cases
Paulo Barreto1, Christian Pagnoux, Luminata Luca
1Department of Internal Medicine, National Referral Center for Necrotizing Vasculitides and Systemic Sclerosis, Hôpital Cochin, Assistance publique-Hôpitaux de Paris, Université Paris Descartes, 27, rue du Faubourg Saint-Jacques, 75879 Paris cedex 14, France.
Abstract:
Retroperitoneal fibrosis has been reported in several patients with Wegener granulomatosis (WG), but only three isolated cases of dorsal prevertebral lesions, closely resembling fibrosing mediastinitis, have been published so far. We describe four new WG patients (two men, two women), 49-59 years old at diagnosis, with dorsal prevertebral lesions, mainly right-sided, and with adjacent pleural thickening in two. These lesions were detected on computed-tomography scans at diagnosis in two patients, and occurred later in the two others. Only one of them had mild back pain. Two patients' lesions were biopsied, revealing granulomatous inflammation. In one patient, the lesion regressed under WG treatment. Lesion size did not change in the remainings. Intralesional calcifications appeared in two. None of the patients had local bone erosion, vascular or neurological complications. These prevertebral lesions might represent a dorsal form of retroperitoneal fibrosis in WG, but usually with a more benign presentation and course. WG should be included in the differential diagnosis of fibrosing mediastinitis (with tuberculosis, neoplastic diseases, sarcoidosis, histiocytosis and inflammatory pseudotumor), which may have a similar radiological appearance.
Insights
Wegener granulomatosis (WG) can cause dorsal prevertebral lesions, a rare manifestation resembling fibrosing mediastinitis. These lesions in WG patients typically present benignly and may respond to treatment.
Area of Science:
- Rheumatology
- Radiology
- Pathology
Background:
- Retroperitoneal fibrosis is a known complication of Wegener granulomatosis (WG).
- Dorsal prevertebral lesions are rare, with only three prior cases reported resembling fibrosing mediastinitis.
Observation:
- Four new WG patients (49-59 years old) presented with dorsal prevertebral lesions, often right-sided, with pleural thickening in two.
- Lesions were detected via computed tomography (CT) scans at diagnosis or later.
- Only one patient reported mild back pain; biopsies revealed granulomatous inflammation.
Findings:
- WG-associated prevertebral lesions may represent a dorsal form of retroperitoneal fibrosis.
- These lesions generally have a more benign presentation and course than typical fibrosing mediastinitis.
- One lesion regressed with WG treatment; others remained stable. Intralesional calcifications appeared in two cases without bone erosion or complications.
Implications:
- Wegener granulomatosis should be considered in the differential diagnosis of fibrosing mediastinitis.
- Radiological findings of dorsal prevertebral lesions can mimic other conditions like tuberculosis, neoplasms, sarcoidosis, histiocytosis, and inflammatory pseudotumor.