Juxta-vertebral lesions in granulomatosis with polyangiitis

https://doi.org/10.1016/j.semarthrit.2016.07.009Get rights and content

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Objectives

To describe the clinical, pathological, serological, and radiological characteristics of juxta-vertebral masses occurring in patients with granulomatosis with polyangiitis (GPA).

Methods

We analyzed the clinical records of patients with juxta-vertebral lesions from our GPA study cohort and reviewed the English literature for other cases of GPA with juxta-vertebral localization.

Results

Out of 74 patients in our GPA study cohort, six (8%) had juxta-vertebral lesions. We found 10 cases of juxta-vertebral GPA described in the English literature. Overall, juxta-vertebral lesions were detected at GPA onset in 11/16 (69%) patients, and preferentially occurred on the right side of the spine (12/15 patients, 80%). Fifteen patients (94%) with juxta-vertebral lesions had systemic GPA. Juxta-vertebral lesions were associated with back pain at GPA onset in 8/16 (50%) patients. In all of them juxta-vertebral lesions resolved or improved after treatment.

Conclusions

Preference for the right-anterior side of the spine, increased 18FDG uptake on PET scan, low or absent invasiveness of the surrounding tissues, and occurrence in the context of systemic disease were the main features of juxta-vertebral GPA. Symptomatic lesions showed a better response to immunosuppressive therapies.

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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    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.

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