Juxta-vertebral lesions in granulomatosis with polyangiitis
Introduction
Granulomatosis with polyangiitis (GPA, formerly Wegener’s Granulomatosis) is a systemic autoimmune condition, characterized by the presence of anti-neutrophil cytoplasmic antibodies (ANCA) in the majority of patients’ sera [1]. Hallmark histological features of GPA include leukocytoclastic small-vessel vasculitis and non-caseating granulomas. The former is typically observed when the lung, kidney, skin, and peripheral nervous system are involved; the latter are preferentially found in upper and lower respiratory tract, as well as in orbital and meningeal disease localizations. However, uncommon manifestations in other organs might occur [2], [3], [4], [5].
Here, we report six patients with atypical juxta-vertebral localization of GPA, and describe the characteristic clinical, pathological, serological, and radiological findings of a unique presentation that might complicate the differential diagnostic process. A review of 10 cases of juxta-vertebral GPA already described in the literature is also presented.
Section snippets
Methods
We reviewed the clinical records of 74 patients diagnosed with GPA according to the criteria of the American College of Rheumatology and to Watts’ algorithm, and actively followed up at our tertiary care center from 2005 to 2015 [6]. Patients with GPA and evidence of juxta-vertebral lesions on imaging studies were included in the analysis. We then searched the entire PubMed and Google Scholar online databases for the terms “Wegener,” “Wegener’s granulomatosis,” “granulomatosis with
Case 1
A 52-year-old woman was referred for sinusitis, cough, and progressive renal failure. A whole-body computed tomography (CT) scan revealed maxillary and sphenoid sinusitis, multiple bilateral lung lesions, and a para-vertebral mass extending from T3 to T7 on the right-anterior side of the spine. All lesions showed 18-fluorodexoxyglucose (18FDG) uptake on positron emission tomography (PET). A renal biopsy showed a necrotizing extracapillary pauci-immune glomerulonephritis consistent with GPA.
Discussion
Juxta-vertebral lesions represent an uncommon manifestation of GPA, which typically involves the ear–nose–throat district, lungs, kidneys, and, less frequently, the periorbital tissue and the meninges. In the present study, we reviewed the clinical, serological, pathological, and radiological characteristic of this atypical presentation, and found that almost 10% of patients with GPA in our cohort had a juxta-vertebral mass on imaging studies.
In 50% of cases, juxta-vertebral lesions were
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2022, Autoimmunity ReviewsCitation Excerpt :They are mostly asymptomatic and not associated with bone erosion or compression of nearby vessels, therefore their frequency may be underestimated. Patients with paravertebral masses had a mean age of 59 [51–72] years, without difference in sex distribution, positive PR3-ANCA was observed in almost 90% of cases. The detection of the paravertebral lesions occurred at the time of GPA diagnosis in 72% of the cases.
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These authors equally contributed.