Dorsal prevertebral lesions in Wegener granulomatosis: Report on four cases

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<ul><li><p>Joint Bone Spine 78 (2011) 8891</p><p>Case report</p><p>Dorsal om</p><p>Paulo Ba siePascal Coa Department o c SclerParis Descartesb Department oc Department od Department o</p><p>a r t i c l</p><p>Article history:Accepted 26 JuAvailable onlin</p><p>Keywords:Wegeners graFibrosing medRetroperitonePrevertebral lesions</p><p>ortedverter news, mauted-m ha</p><p>one pdid not change in the remainings. Intralesional calcications appeared in two. None of the patients hadlocal bone erosion, vascular or neurological complications. These prevertebral lesions might represent adorsal form of retroperitoneal brosis in WG, but usually with a more benign presentation and course.WG should be included in the differential diagnosis of brosing mediastinitis (with tuberculosis, neo-plastic diseases, sarcoidosis, histiocytosis and inammatory pseudotumor), which may have a similar</p><p>1. Introdu</p><p>Wegeneing small-scytoplasm3 (PR3) [1and throat,ifestations horbital pseuMost of theinltrationvaried wide</p><p>Herein,bral tissularwork-up fo</p><p> CorresponE-mail add</p><p>1297-319X/$ doi:10.1016/j.radiological appearance. 2010 Socit francaise de rhumatologie. Published by Elsevier Masson SAS. All rights reserved.</p><p>ction</p><p>r granulomatosis (WG) is a rare systemic and necrotiz-izedvessel vasculitis, associated with antineutrophilantibodies (ANCA), mainly directed against proteinase3]. In addition to its typical clinical triade of ear, nosepulmonary and renal involvements, several other man-ave been reported, some of which are suggestive, likedotumor or pachymeningitis, but rather unusual [46].se latter were caused by local granulomatous tissueand their responses to conventional treatment for WGly.we describe four WG patients with dorsal preverte-lesions and discuss on the presentation and diagnostic</p><p>r these exceptional lesions.</p><p>ding author. Tel.: +33 1 58 41 13 21; fax: +33 1 58 41 14 50.ress: christian.pagnoux@cch.aphp.fr (C. Pagnoux).</p><p>2. Case reports</p><p>2.1. Case 1</p><p>A 50-year-old woman consulted in 1996 for acute onsetlaryngeal stridor after a 2-year history of peripheral joint painand chronic sinusitis. Blood analyses revealed an inammatorysyndrome but no renal abnormalities. ANCA with a cytoplas-mic immunouorescence pattern (C-ANCA) were detected, withantiPR3 specicity. Computed-tomography (CT) scans of thesinuses and chest showed left maxillary destructive sinusitisand subglottic laryngeal stenosis, without bronchial or lung-parenchyma involvement. A prevertebral thoracic lesion wasalso detected, extending from dorsal vertebrae 3 to 7 (D3-D7).On magnetic resonance imaging (MRI), this lesion was regular,hypointense in T1- and T2-weighted sequences,with enhancementafter gadolinium contrast-medium injection (Fig. 1A). Vertebralbodies and intervertebral discs were normal. Tuberculin puriedprotein derivative (PPD) skin test was negative. Granulomatouslesions were found in laryngeal biopsies, supporting the clinicallysuspected diagnosis of WG.</p><p>She received glucocorticoids (GC) in combination with monthlycyclophosphamide (CYC) pulses for 6months, with rapid laryn-geal lesion regression, but persistent rhinitis and sinusitis required</p><p>see front matter 2010 Socit francaise de rhumatologie. Published by Elsevier Masson SAS. All rights reserved.jbspin.2010.07.017prevertebral lesions in Wegener granul</p><p>rretoa,b, Christian Pagnouxa,, Luminata Lucac, Jeshena, Graldine Mullerd, Loc Guillevina</p><p>f Internal Medicine, National Referral Center for Necrotizing Vasculitides and Systemi, 27, rue du Faubourg Saint-Jacques, 75879 Paris cedex 14, Francef Internal Medicine, Centro Hospitalar Lisboa Central, Lisbon, Portugalf Internal Medicine and Infectious Diseases, CHU de Poitiers, 86000 Poitiers, Francef Internal Medicine, Hpital Gnral, 21300 Dijon, France</p><p>e i n f o</p><p>ly 2010e 18 September 2010</p><p>nulomatosisiastinitisal brosis</p><p>a b s t r a c t</p><p>Retroperitoneal brosis has been reponly three isolated cases of dorsal prebeenpublished so far.Wedescribe founosis, with dorsal prevertebral lesionThese lesions were detected on complater in the two others. Only one of theing granulomatous inammation. Inatosis: Report on four cases</p><p>Aouizeratea, Isabel Ortigueirab,</p><p>osis, Hpital Cochin, Assistance publiqueHpitaux de Paris, Universit</p><p>in several patients with Wegener granulomatosis (WG), butbral lesions, closely resembling brosing mediastinitis, haveWGpatients (twomen, twowomen), 4959years old at diag-</p><p>inly right-sided, and with adjacent pleural thickening in two.tomography scans at diagnosis in two patients, and occurredd mild back pain. Two patients lesions were biopsied, reveal-atient, the lesion regressed under WG treatment. Lesion size</p></li><li><p>P. Barreto et al. / Joint Bone Spine 78 (2011) 8891 89</p><p>Fig. 1. Patient ular lavailable). B. C unchachest obtained</p><p>switching ttive dose, 9totally reso</p><p>She wasabnormalitimicroscopiclobe of thecapillary glreceived rithigh cumulnodule disa</p><p>AlthoughasymptomaCT scan (Fig</p><p>2.2. Case 2</p><p>A 49-ye2002 for coan inammwith antiPRgeal stenosiWG was diobtained lanodules and</p><p>In 2004,ance, worseright-sidedthe pleura bCT-guidedinammatioCYC pulse freduction oAZA.</p><p>In 2007,ual prevertetracheal dilandGC achiwas continu</p><p>atient 2. A. Initial CT scan of the chest revealing a right-sided dorsal pre-l lesion (arrow). B. Five years later, after WG treatment, showing some sizen.</p><p>se 3</p><p>9-year-old woman consulted in 2004 with asthenia, sinusitisiscleritis of the right eye. Blood analyses revealed inamma-ndrome but no renal abnormalities. C-ANCA with antiPR3city were detected. Chest CT scan showed no lung involve-ut a right-sided prevertebral D2D7 lesion, spreading to the(Fig. 3A), without invasion of vertebrae or intervertebral1. A. Parasagittal T2-weighted MRI of the chest and spine showing hypointense tissT scan of the chest obtained 8years later. The lesion (arrow) reportedly appeared2years later, showing no further change.</p><p>o oral CYC, continued for a total of 42months (cumula-6 g), followed by azathioprine (AZA). Her rhinitis neverlved and ANCA remained positive.referred to our department in 2007 because of renales (serumcreatinine, 189mol/L; proteinuria, 2 g/24h;hematuria) anda7-mmdiameternodule in the inferiorleft lung. Kidney biopsy showed necrotizing extra-</p><p>omerulonephritis. The GC dose was increased and sheuximab (RTX), rather than CYC because of her alreadyative CYC dose. She entered remission and the lungppeared after 9months.the prevertebralmass size did not change, it remained</p><p>tic and intralesion calcications appeared on the chest. 1B and C).</p><p>ar-old man, with a history of asthma, consulted inugh, dysphonia and hemoptysis. Blood tests revealed</p><p>Fig. 2. Pvertebrareductio</p><p>2.3. Ca</p><p>A 5and eptory syspeciment bpleuraatory syndrome, normal eosinophil count and C-ANCA3 specicity. Chest CT scan showed a subglottic laryn-s and lung nodules. No renal involvementwas detected.agnosed and treatment combining oral CYC and GCryngeal lesion attenuation and disappearance of lungANCA. CYC was stopped after 8months.</p><p>WG relapsed with lung-nodule and C-ANCA reappear-ning of the laryngeal stenosis, and appearance of aprevertebral D3D7 lesion, with lateral spreading tout without bone erosion or disk involvement (Fig. 2A).needle-biopsy of the lesion revealed granulomatousn, and no mycobacteria. Treatment with a monthlyor 6months and GC achieved remission and markedf the prevertebral mass (Fig. 2B). CYC was switched to</p><p>the laryngeal stenosis worsened again but the resid-bral lesion remained stable on the CT scan. Endoscopication and a regimen combining oral CYC, initially, RTXeved remission. CYCwas stopped andmaintenance RTXed, with no further relapse to date.</p><p>disks. Thewithout my</p><p>She receresolutionowas switchafter 4mon</p><p>Fig. 3. Patienttebral lesion (size reductionesion (arrow) anterior to D3D7 vertebrae (initial CT scan images notnged, with the appearance of internal calcications. C. CT scan of thesurgical biopsy showed granulomatous inammation,cobacteria, supporting the diagnosis of WG.ived GC and a monthly CYC pulse for 6months, withf sinusitis andepiscleritis. ANCAremainedpositive. CYCed to AZA for maintenance, changed to methotrexateths, because of hepatic toxicity.</p><p>3. A. Initial CT scan of the chest revealing a right-sided dorsal prever-arrow). B. CT scan obtained 4years later, showing no (or only minor).</p></li><li><p>90 P. Barreto et al. / Joint Bone Spine 78 (2011) 8891</p><p>In 2006,WG relapsedwith right episcleritis, cough anddyspnea.Chest CT scan showed lung nodules and persistence of the prever-tebral mass. She received a second, but ineffective, cycle of CYCpulses, then RTX as rescue therapy, with a semestrial RTX infusionformainten(Fig. 3B), an</p><p>2.4. Case 4</p><p>A 57-yeaobstructivesulted in 20and mononmatory synanalyses shturia. C-ANscan showesegmentalbined withRenal abnociency (serubut paresthsyndrome (positive.</p><p>In late 2sal pain. Tbut detectewithout boInterferon-gregimen wa</p><p>In mid-trating orbspecic ineosinophilsunchangedmonthly CYpulses, themass had tsome calciled to a swi</p><p>3. Discussi</p><p>PreverteTo our kno[79]. All ththeir locatio</p><p>Cardenapatient witprevertebralonephritis.GC. KerkenANCA-positsinusitis, glmass that ral. [7] reporitis, pleurabiopsy) andtebrae. Theextension fnantly righ[9].</p><p>Inltratilocations, limucosa, bre Ta</p><p>ble</p><p>1M</p><p>ain</p><p>char</p><p>acte</p><p>rist</p><p>icsof</p><p>the</p><p>seve</p><p>nW</p><p>egen</p><p>ers</p><p>gran</p><p>ulo</p><p>mat</p><p>osis</p><p>pat</p><p>ients</p><p>with</p><p>pre</p><p>vert</p><p>ebra</p><p>lles</p><p>ions.</p><p>Patien</p><p>tsAge</p><p>/Sex</p><p>ANCA</p><p>Oth</p><p>eror</p><p>gan</p><p>invo</p><p>lvem</p><p>ent</p><p>Prev</p><p>erte</p><p>bral</p><p>lesion</p><p>char</p><p>acte</p><p>rist</p><p>ics</p><p>Onse</p><p>tLo</p><p>caliza</p><p>tion</p><p>Exte</p><p>nsion</p><p>Pleu</p><p>ral</p><p>exte</p><p>nsion</p><p>Bio</p><p>psy</p><p>Outc</p><p>ome</p><p>Loca</p><p>lero</p><p>sion</p><p>orco</p><p>mpre</p><p>ssio</p><p>n</p><p>Cas</p><p>e1</p><p>50/F</p><p>PR3</p><p>SIN,L</p><p>uN,G</p><p>N,j</p><p>oint,</p><p>lary</p><p>nx</p><p>Atdia</p><p>gnos</p><p>isRig</p><p>ht</p><p>D3</p><p>D7</p><p>No</p><p>No</p><p>Sam</p><p>esize</p><p>,ca</p><p>lci</p><p>cation</p><p>sNon</p><p>e</p><p>Cas</p><p>e2</p><p>49/M</p><p>PR3</p><p>LuN,l</p><p>aryn</p><p>xLa</p><p>ter</p><p>Rig</p><p>ht</p><p>D3</p><p>D7</p><p>Yes</p><p>Yes</p><p>(gra</p><p>nulo</p><p>ma)</p><p>Size</p><p>reduce</p><p>dNon</p><p>e</p><p>Cas</p><p>e3</p><p>59/F</p><p>PR3</p><p>SIN,L</p><p>uN,</p><p>episcl</p><p>eritis</p><p>Atdia</p><p>gnos</p><p>isRig</p><p>ht</p><p>D2</p><p>D7</p><p>Yes</p><p>Yes</p><p>(gra</p><p>nulo</p><p>ma)</p><p>Sam</p><p>esize</p><p>(min</p><p>orsize</p><p>reduct</p><p>ion)</p><p>Non</p><p>e</p><p>Cas</p><p>e4</p><p>57/M</p><p>PR3</p><p>GN,M</p><p>N,O</p><p>RB,s</p><p>kin,</p><p>join</p><p>tsLa</p><p>ter</p><p>Rig</p><p>ht</p><p>D5</p><p>D10</p><p>No</p><p>No</p><p>Sam</p><p>esize</p><p>(min</p><p>orsize</p><p>reduct</p><p>ion),</p><p>calc</p><p>ica</p><p>tion</p><p>s</p><p>Non</p><p>e</p><p>Tojim</p><p>aet</p><p>al.[</p><p>7]63</p><p>/MNeg</p><p>ativ</p><p>eLu</p><p>N,p</p><p>leure</p><p>sy,</p><p>episcl</p><p>eritis</p><p>Atdia</p><p>gnos</p><p>is(N</p><p>R)</p><p>(NR)</p><p>(NR)</p><p>No</p><p>(NR)</p><p>Non</p><p>e</p><p>Ker</p><p>kenie</p><p>tal</p><p>.[8]</p><p>55/M</p><p>MPO</p><p>SIN,G</p><p>NAtdia</p><p>gnos</p><p>isCen</p><p>ter</p><p>D4</p><p>D10</p><p>Yes</p><p>(rig</p><p>ht)</p><p>No</p><p>Res</p><p>olve</p><p>dNon</p><p>e</p><p>Car</p><p>den</p><p>al-U</p><p>rdam</p><p>pille</p><p>taet</p><p>al.[</p><p>9]40</p><p>/MPR</p><p>3Lu</p><p>N,G</p><p>NFi</p><p>rstan</p><p>dso</p><p>lein</p><p>itia</p><p>lm</p><p>anifes</p><p>tation</p><p>Rig</p><p>ht</p><p>D8</p><p>D10</p><p>Yes</p><p>No</p><p>Res</p><p>olve</p><p>dNon</p><p>e</p><p>ANCA:a</p><p>ntineu</p><p>trop</p><p>hil</p><p>cyto</p><p>pla</p><p>sman</p><p>tibo</p><p>die</p><p>s;D:d</p><p>orsa</p><p>lver</p><p>tebr</p><p>a;F:</p><p>fem</p><p>ale;</p><p>GN:g</p><p>lom</p><p>erulo</p><p>nep</p><p>hritis;</p><p>LuN:l</p><p>ung</p><p>nod</p><p>ule</p><p>;M:m</p><p>ale;</p><p>MN:m</p><p>oneu</p><p>ritism</p><p>ultip</p><p>lex;</p><p>MPO</p><p>:mye</p><p>loper</p><p>oxid</p><p>ase;</p><p>NR:n</p><p>otre</p><p>por</p><p>ted</p><p>inth</p><p>eEn</p><p>glish</p><p>abst</p><p>ract</p><p>;ORB:</p><p>orbi</p><p>talm</p><p>ass;</p><p>PR3:</p><p>pro</p><p>tein</p><p>ase</p><p>3;SI</p><p>N:sinusitis.ance. Theprevertebral lesion onlymodestly diminishedd residual lung nodules persisted on the CT scan.</p><p>r-old man, with a history of hypertension and chroniclung disease (tobacco intoxication, 40pack-years), con-05with asthenia, fever, diffuse arthralgias, skin purpuraeuritis multiplex. Blood tests revealed an inam-drome. Serum creatinine was 143mol/L and urineowed proteinuria (&gt; 1g/24h) and microscopic hema-CA were detected with antiPR3 specicity. Chest CTd no lung involvement. Kidney biopsy revealed focalglomelurosclerosis. WG was diagnosed and AZA, com-GC, was started. Joint pain and skin lesions resolved.rmalities regressed, with persistent mild renal insuf-m creatinine, 125mol/L). Motor decit disappearedesias persisted, as did the uctuating inammatoryC-reactive protein, 1030mg/L), and ANCA remained</p><p>008, the patient reported mild and uctuating dor-he chest CT scan showed no signs of lung disease,d a right-sided prevertebral, fusiform D5D10 lesion,ne erosion or intervertebral disk invasion (Fig. S1A).amma tuberculosis blood test was negative. The WGs not modied.2009, left exophthalmia occurred due to an inl-ital lesion, whose surgical removal revealed a non-ammatory inltration of lymphocytes, plasmocytes and. Renal function was stable. The prevertebral mass wasand lungs remained unaffected. AZA was switched toC pulses and the GC dose was increased. After six CYCchest CT scan showed that the thoracic prevertebralhe same size (or slightly diminished), and containedcations (Fig. S1B); the reappearance of the orbital lesiontch to oral CYC.</p><p>on</p><p>bral lesions like those described above are rare in WG.wledge, only three similar cases have been publishedese cases are very similar radiologically, especially inn and appearance (Table 1).l-Urdampilleta et al. [9] reported on a 40-year-old WGh antiPR3 ANCA, who initially had a painful D8D10l mass, and later developed lung nodules and glomeru-Themassdisappearedafter therapy combiningCYCandi et al. [8] reported a 55-year-old antimyeloperoxidaseive patient, diagnosed with WG because of associatedomerulonephritis and a painful D4D10 prevertebralegressed under treatment for WG. Finally, Tojima etrted an ANCA-negative 63-year-old man with episcle-l effusion, lung nodules (showing vasculitis in a surgicala mass in the posterior mediastinum, adjacent to ver-lesions in our patients were also dorsal, with pleural</p><p>or two of them. Intriguingly, the lesions were predomi-t-sided in all our patients, and one previously reported</p><p>ng lesions inWGpatientshavebeendescribed in severalke the central nervous system, orbital cavity, gingivalasts, anterior and middle mediastinum and retroperi-</p></li><li><p>P. Barreto et al. / Joint Bone Spine 78 (2011) 8891 91</p><p>toneum [5,6,10,11]. Granulomatous inammation is often seen inthe biopsies of such patients, when feasible and done, and, morerarely, vasculitis. Whether dorsal prevertebral lesions, as describedin our patients, should be considered brosing mediastinitis or likeretroperitoneal brosis remains to be determined, with the latterbeing reported more frequently in WG. Their locations in the pre-vertebral areas could suggest a parallel, but none of our patients,nor those previously reported, had true associated retroperitonealbrosis. In addition, dorsal prevertebral lesions did not lead tocompression of nearby structures, as opposed to retroperitonealbrosis (with possible ureteral and/or vessel compression and inl-tration). The differential diagnoses to rule out also overlap withthose of brosing mediastinitis or retroperitoneal brosis, andmainly include neoplastic diseases, infections, especially tubercu-losis, other granulomatous diseases, like sarcoidosis, inammatorypseudotumor or histiocytosis [1215]. Imaging studies, especiallyMRI, can be helpful to exclude spondylodiscitis [15,16]. Commonvertebral osteoarthritis can also sometimes mimic these appear-ances, when internal calcications are present.</p><p>Biopsy of such lesions is not easy and carries a non-negligiblerisk of complication(s), because of the difculty in accessing thethoracic prevertebral area. None of the three previously reportedcases and only two of ours had biopsies, one surgical and theother CT-guided because the adjacent pleural spreading allowedsuch a procedure. These two patients biopsies showed granulo-matous inammation. In addition to histology, when positive, thelink between these lesions and WG is further supported by lesionregression ulished caseand in onetion on biopunchangedtions, as a h</p><p>In conclu...</p></li></ul>