Case Report Uncommon Presentation of Granulomatosis with Polyangiitis Mimicking Metastatic Cancer

Edyta Maria Urbanska 1,* , Johanna Elversang 2, Bonnie Colville-Ebeling 2, Johan Olof Löfgren 3, Karl Emil Nelveg-Kristensen 4 and Wladimir M. Szpirt 4,*

1 Department of Oncology, Rigshospitalet, University of Copenhagen, 2100 Copenhagen, Denmark 2 Department of Pathology, Rigshospitalet, University of Copenhagen, 2100 Copenhagen, Denmark; [email protected] (J.E.); [email protected] (B.C.-E.) 3 Department of Clinical Physiology, Nuclear Medicine & PET, Rigshospitalet, University of Copenhagen, 2100 Copenhagen, Denmark; [email protected] 4 Department of Nephrology, Rigshospitalet, University of Copenhagen, 2100 Copenhagen, Denmark; [email protected] * Correspondence: [email protected] (E.M.U.); [email protected] (W.M.S.); Tel.: +45-3545-0604 (E.M.U.); +45-3545-1767 (W.M.S.)

Abstract: Diagnosis of anomalous intrathoracic lesions may be challenging and require a multidis- ciplinary approach. We present a case of granulomatosis with polyangiitis (GPA) clinically and radiologically mimicking metastatic with a bilateral pulmonary mass, mediastinal and cervical involvement, and . Surgical of the thoracic lesion revealed necrotic granulomatous inflammation, and the final diagnosis was subsequently confirmed by kidney biopsy and biochemical parameters. This case illustrates how comprehensive diagnosis secures  timely and relevant treatment. Systemic may be one of the key differential diagnoses in  patients with multiorgan involvement, especially with pattern-mimicking lung cancer.

Citation: Urbanska, E.M.; Elversang, J.; Colville-Ebeling, B.; Löfgren, J.O.; Keywords: intrathoracic lesions; granulomatosis with polyangiitis; GPA; vasculitis; lung cancer Nelveg-Kristensen, K.E.; Szpirt, W.M. Uncommon Presentation of Granulomatosis with Polyangiitis Mimicking Metastatic Lung Cancer. Clin. Pract. 2021, 11, 293–302. 1. Introduction https://doi.org/10.3390/ Differential diagnosis of abnormal thoracic lesions constitutes an important part of clinpract11020042 routine clinical work-up and can include a wide spectrum of diseases. As soon as standard diagnostic procedures fail to recognize a patient’s diagnosis, a collaborative approach is Received: 15 April 2021 required to secure timely and relevant treatment. Accepted: 11 May 2021 Published: 14 May 2021 Granulomatous diseases can easily mimic a malignant phenotype as in the current case of granulomatosis with polyangiitis (GPA, Wegener’s granulomatosis). GPA, micro-

Publisher’s Note: MDPI stays neutral scopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA) are with regard to jurisdictional claims in defined as small-vessel vasculitides associated with antineutrophil cytoplasmic antibodies published maps and institutional affil- (ANCAs) [1]. ANCA-associated vasculitis (AAV) is characterized by inflammation of small iations. vessel walls with preferential involvement of the upper and lower airways as well as of the kidneys. However, it can essentially affect any part of the body [2–4]. AAV carries a significant risk of mortality and morbidity notwithstanding adequate treatment, and the time from initial symptoms to actual diagnosis is positively correlated with the disease outcome [5,6]. GPA and MPA are rare diseases with a collective incidence of approximately Copyright: © 2021 by the authors. Licensee MDPI, Basel, Switzerland. 20 cases per million per year [7]; they are characterized by similar renal histopatholog- This article is an open access article ical lesions [8] and share a putative ANCA-associated pathogenesis [9]; however, their distributed under the terms and associated ANCA serology (anti-proteinase 3 and anti-myeloperoxidase) differs in genetic conditions of the Creative Commons predisposition [10], treatment response, risk of relapse [11], and [12,13]. Due Attribution (CC BY) license (https:// to the granulomatous phenotype including pseudotumors and lung as well creativecommons.org/licenses/by/ as the frequently reported longstanding prodromal constitutional symptoms, the diagno- 4.0/). sis of GPA often represents a diagnostic challenge which may delay adequate treatment

Clin. Pract. 2021, 11, 293–302. https://doi.org/10.3390/clinpract11020042 https://www.mdpi.com/journal/clinpract Clin. Pract. 2021, 12, FOR PEER REVIEW 2

the frequently reported longstanding prodromal constitutional symptoms, the diagnosis of GPA often represents a diagnostic challenge which may delay adequate treatment with a subsequent risk of more severe chronic damage, morbidity, and mortality. Ac- cordingly, this case report highlights these diagnostic challenges from the oncologist’s perspective and emphasizes possible pitfalls associated with contemporary standard ra-

Clin. Pract. 2021, 11 diological cancer screening. 294

2. Materials and Methods with a subsequent risk of more severe chronic tissue damage, morbidity, and mortality. Accordingly,A 56-year-old this case report Caucasian highlights these male, diagnostic heavy challenges smoker, from without the oncologist’s known comorbidities, was re- perspectiveferred to and the emphasizes Department possible pitfallsof Pulmonology associated with contemporary with suspected standard metastatic radio- lung tumor. During logical cancer screening. the preceding six months, he had experienced increasing , productive 2., Materials and and Methodsoccasional . He also complained of peripheral edema, muscle and jointA56-year-old pain, , Caucasian and male,unintentional heavy smoker, weight without knownloss. An comorbidities, initial chest was X-ray showed a left hilar referred to the Department of Pulmonology with suspected metastatic lung tumor. During themass preceding (Figure six months, 1), and he hadsubsequent experienced fluorodeoxyglucose increasing shortness of breath, (18 productiveF) positron emission tomography– cough,computed and occasional tomography hemoptysis. (FDG-PET/CT) He also complained revealed of peripheral an edema, 8-cm muscle FDG-avid and tumor com- joint pain, fever, and unintentional . An initial chest X-ray showed a left hilar massplex (Figure suggestive1), and subsequent of malignancy fluorodeoxyglucose in the ( 18 leftF) positron upper emission lobe tomography–with mediastinal involvement. The computedFDG-PET/CT tomography scan (FDG-PET/CT) also revealed revealed FDG-avid an 8-cm FDG-avid enlarged tumor lymph atelectasis nodes on the left side of the complex suggestive of malignancy in the left upper lobe with mediastinal involvement. Theneck, FDG-PET/CT in the scan also revealed FDG-avidat positions enlarged 4 L lymph and nodes 7 and on theat leftboth side hila, of as well as bilateral pul- themonary neck, in thenodules mediastinum suggestive at positions of 4 Lmetastases, and 7 and at both pleural hila, as wellmetastasis as bilateral in the middle right lung’s pulmonarylobe, left-sided nodules suggestive pleural of metastases,effusion, pleural a small metastasis FDG- inavid the middle lesion right in lung’s both parotid glands and a 3- lobe, left-sided pleural effusion, a small FDG-avid lesion in both parotid glands and a 3-cm FDG-avidcm FDG-avid lesion in the lesion in (Figure the prostate2A–C, maximum (Figure intensity 2A–C, projection maximum (MIP)). intensity projection (MIP)).

FigureFigure 1. Chest 1. Chest X-ray with X-ray a large with left hilar a large mass. left hilar mass.

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(B)

(A) (C)

FigureFigure 2. (A 2.) Maximum(A) Maximum Intensity Intensity Projection Projection (MIP) (MIP) FDG-PE FDG-PETT showingshowing multiple multiple FDG-avid FDG-avid lesions, lesions, (B) transaxial(B) transaxial CT image CT image showingshowing a tumor a tumor atelectasis atelectasis complex complex in the in the left left upper upper lu lung’sng’s lobe lobe and and enlarged enlarged ly lymphmph nodesnodes in in both both hila, hila, (C ()C same) same as as (B) fused(B) withfused FDG-PET. with FDG-PET. Laboratory results showed an active urine sediment with (5.48 g/day) andLaboratory togetherresults showed with elevated an active serum urin creatininee sediment (173 µwithmol/L). prot Prostate-specificeinuria (5.48 g/day) andantigen hematuria (PSA), together calcitonin, with anti-neutrophil elevated serum cytoplasmic antibodies (173 μmol/L). (ANCA), Prostate-specific myeloperoxi- an- tigendase (PSA), (MPO)-ANCA, calcitonin, anti-glomerular anti-neutrophil basal cyto membraneplasmic (GBM),antibodies and (ANCA), immunoglobulins myeloperoxidase were (MPO)-ANCA,all normal, but anti-glomerular C-reactive protein basal (CRP) membrane (70 mg/L), (GBM), proteinase and 3 (PR3)-ANCA immunoglobulins (44 kU/L), were all normal,urine albuminbut C-reactive creatinine protein ratio (UACR) (CRP) (70 (2730 mg/L), U), blood proteinase pressure 3 (172/98 (PR3)-ANCA mmHg) (44 were kU/L), urineelevated. albumin Serum creatinine albumin ratio was (UACR) low, 21 g/L. (2730 Standard U), blood additional pressure diagnostic (172/98 mmHg) procedures were el- including with endobronchial ultrasound (EBUS), evated. Serum albumin was low, 21 g/L. Standard additional diagnostic procedures in- (BAL) and cytological examination of pleural effusion did not show any signs of malig- cludingnancy. bronchoscopy Therefore, to finally with confirm endobronchial or refute cancer, ultrasound video-assisted (EBUS), thoracoscopic bronchoalveolar surgery lavage (BAL)(VATS) and with cytological biopsy from examination the pulmonal of pleural tumor ineffusion the right did middle not show lung’s any lobe signs was of per- malig- nancy.formed Therefore, (Figure3 to), andfinally four confirm days later, or refu becausete cancer, of the video-assisted coexisting and thoracoscopic abovementioned surgery (VATS)abnormal with renal biopsy parameters, from the renal pulmonal biopsy was tumor performed. in the right middle lung’s lobe was per- formed (Figure 3), and four days later, because of the coexisting and abovementioned ab- normal renal parameters, renal biopsy was performed.

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FigureFigure 3. 3.FDG-PET/CT FDG-PET/CT showing pleural metastasis inin thethe rightright lung’slung’s middlemiddle lobe.lobe.

3.3. ResultsResults HistologicalHistological examination examination of of the the formalin-fixed formalin-fixed and and paraffin-embedded paraffin-embedded tissue tissue from from the rightthe right lung’s lung’s middle middle lobe lobe revealed revealed a nodular a nodular lesion lesion consisting consisting of of a a consolidated consolidated alveolar alveolar parenchymaparenchyma with with necrosis, necrosis, granulomatous granulomatous inflammation, , multinucleated multinucleated giant giant cells, cells, often of- withten with smudged smudged basophilic basophilic nuclei nuclei marginated marginated at the at periphery the periphery of the of cell, the andcell, cholesteroland choles- cleftsterol (Figuresclefts (Figures4–6). The4–6). necrotic The necrotic areas areas were were big and big confluentand confluent focally focally with with geographical geograph- morphologyical morphology and basophilicand basophilic staining staining of debris of debris from from neutrophil neutrophil granulocytes granulocytes as well as well as palisadingas palisading histiocytes histiocytes along along the the brim. brim. There There were were scattered scattered microabscesses. microabscesses. The The inflam- inflam- matorymatory infiltrateinfiltrate waswas dominateddominated byby lymphocyteslymphocytes and,and, toto aa lesserlesser extent,extent, byby plasmaplasma cells,cells, ,eosinophils, andand neutrophilneutrophil granulocytes.granulocytes. ThereThere waswas aa littlelittle focusfocus ofof possiblepossiblecapillaritis, , butbut no no evident evident inflammation inflammation or or fibrinoid fibrinoid necrosis necrosis in in the the vessel vessel walls. walls. However, However, several several of of thethe larger larger vessels vessels were were involved involved including including granulomatous granulomatous inflammation inflammation with with destruction destruction ofof vesselvessel walls walls and and lumina, lumina, respectively. respectively. Finally, Finally, there there was was a smalla small area area of of xanthogranulo- xanthogranu- matouslomatous inflammation inflammation with with alveoli alveoli filled filled with with foamy foamy . macrophages. The inflammationThe inflammation was seenwas inseen close in relation close relation to bronchi, to bronchi, but there but were there no were no bronchiolitis obliterans, acuteobliterans, bronchioli- acute tis,bronchiolitis, bronchocentric bronchocentric granulomatosis, granulomatosis, or stenosis. Noor stenosis. foreign materialNo foreign was material identified. was Special identi- stainsfied. Special for fungi stains and for mycobacteria fungi and mycobacteria were negative, were and negative, there were and no there signs were of malignancy. no signs of Themalignancy. changes themselvesThe changes were themselves not pathognomonic were not pathognomonic for ANCA-associated for ANCA-associated vasculitis (AAV). vas- However, in lieu of the clinical presentation with joint pain, malaise, and constitutional culitis (AAV). However, in lieu of the clinical presentation with joint pain, malaise, and symptoms, absence of malignancy in EBUS, BAL, cytological examination of pleural ef- constitutional symptoms, absence of malignancy in EBUS, BAL, cytological examination fusion, and VATS in association with elevated PR3-ANCA and kidney biopsy showing of pleural effusion, and VATS in association with elevated PR3-ANCA and kidney biopsy pauci-immune extracapillary necrotizing glomerulonephritis (Figure7), the entire picture showing pauci-immune extracapillary necrotizing glomerulonephritis (Figure 7), the en- was consistent with GPA. The patient immediately started the standard treatment regimen tire picture was consistent with GPA. The patient immediately started the standard treat- for AAV with (1 g/kg/day) in combination with oral ment regimen for AAV with prednisolone (1 g/kg/day) in combination with oral cyclo- (100 mg/day), and the symptoms subsequently resolved with normalization of biochemical phosphamide (100 mg/day), and the symptoms subsequently resolved with normalization parameters. At follow-up, three months later, FDG-PET/CT showed complete resolution of biochemical parameters. At follow-up, three months later, FDG-PET/CT showed com- of all the prior FDG-avid lesions (Figure8A–C). Presently, at 14 months post-discharge, plete resolution of all the prior FDG-avid lesions (Figure 8A–C). Presently, at 14 months the patient is stable without extrarenal AAV activity, his creatinine has normalized to post-discharge, the patient is stable without extrarenal AAV activity, his creatinine has 81 µmol/L, creatinine clearance—to 91 mL/min, and proteinuria—to 2–3 g/day on the azathioprinenormalized to and 81 prednisolone μmol/L, creatinine maintenance clearance—to therapy. 91 mL/min, and proteinuria—to 2–3 g/day on the and prednisolone maintenance therapy.

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FigureFigure 4. Video-Assisted 4. Thoracoscopic Surgery (VATS) biopsy from the right lung’s middle lobe: Granulomatosis with Figure 4. Video-AssistedVideo-Assisted Thoracoscopic Thoracoscopic Surgery Surgery (VATS) (VATS) biopsy biopsy fromfrom the the right right lung’s lung’s middle middle lobe: lobe: Granulomatosis Granulomatosis with with Polyangiitis (GPA) with a multicore : a characteristic multinucleated giant cell with smudged basophilic nuclei PolyangiitisPolyangiitis (GPA) (GPA) with with a multicore a multicore giant giant cell: cell: a acharacteristic characteristic multinucleated giant giant cell cell with with smudged smudged basophilic basophilic nuclei nuclei marginated at the periphery of the cell. Magnification: ×40.× Haematoxylin and Eosin (HE) stain. marginatedmarginated at the at periphery the periphery of the of the cell. cell. Magnific Magnification:ation: ×40.40. Haematoxylin Haematoxylin andand EosinEosin (HE) (HE) stain. stain.

Figure 5. VATS biopsy from the right lung’s middle lobe: GPA blood vessels with vasculitis: destruction of larger vessel Figure 5. VATS biopsy from the right lung’s middle lobe: GPA blood vessels with vasculitis: destruction of larger vessel walls Figureand lumina 5. VATS due biopsy to inflammation. from the right Magnification: lung’s middle lobe: ×10. GPA HE bloodstain. vessels with vasculitis: destruction of larger vessel walls wallsand lumina and lumina due dueto inflammation. to inflammation. Magnification: Magnification: ×10.×10. HE HE stain. stain.

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FigureFigure 6. VATS 6. VATSbiopsy biopsy from fromthe right the rightlung’s lung’s middle middle lobe: lobe:GPA GPAnecrotizing necrotizing : granuloma: necrot necrotizingizing granulomatous granulomatous inflam- mationFigureinflammation and 6. cholesterol VATS and biopsy cholesterolclefts. from The the clefts.necros right Theis lung’s is necrosisbasophilic, middle is basophilic, lobe:with GPAgeographical withnecrotizing geographical morphology. granuloma: morphology. Magn necrotification:izing Magnification: granulomatous ×10. HE× stain.10. inflam- mationHE stain. and cholesterol clefts. The necrosis is basophilic, with geographical morphology. Magnification: ×10. HE stain.

Figure 7. Left kidney: the biopsy showed crescentic glomerulonephritis with focal fibrinoid necro- sis ofFigureFigure the glomerular 7. 7.Left Left kidney: kidney: tufts the endthe biopsy biopsy extracapillary showed showed crescentic proliferation crescentic glomerulonephritis gl omerulonephritisin five out with of seven focal with fibrinoidglomeruli. focal necrosis fibrinoid Immuno- necro- fluorescenceofsis the of glomerular the was glomerular negative tufts end tufts and extracapillary endconsistent extracapillary proliferationwith pauci-immune proliferation in five out ANCA-associatedin of five seven out glomeruli. of seven glomerulonephri- Immunofluo- glomeruli. Immuno- tis. rescencefluorescence was negative was negative and consistent and consistent with pauci-immune with pauci-immune ANCA-associated ANCA-associated glomerulonephritis. glomerulonephri- tis.

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(B)

(A) (C)

FigureFigure 8. 8.(A (A) MIP) MIP FDG-PET FDG-PET showing showing complete complete resolution resolution of allof all the the prior prior FDG-avid FDG-avid lesions, lesions, (B) transaxial(B) transaxial CT imageCT image showing show- resolutioning resolution of the of prior the tumorprior tumor atelectasis atelectasis complex, complex, (C) same (C) assame (B) as fused (B) fused with FDG-PET. with FDG-PET.

4.4. Discussion Discussion GPAGPA and and microscopic polyangiitis (MPA) (MPA) confine confine the major the major subgroups subgroups of AAV, of which AAV, representswhich represents a complex a complex autoimmune autoimmune disease disease of multifactorial of multifactorial etiology etiology that can that affect can affect all organall systems systems with with preferential preferential involvement involvement of theof the skin, skin, respiratory respiratory tract, tract, and and kidneys. kidneys. PulmonaryPulmonary involvement involvement in in AAV AAV is is a a frequent frequent finding finding and and with with varying varying radiographic radiographic presentations,presentations, i.e., i.e., multiple multiple lung lung nodulinoduli ofof varyingvarying size, solid and and often often cavitary cavitary lesions lesions or ordiffuse diffuse ground-glass ground-glass opacities opacities and and fibrosis fibrosis [14,15]. [14,15 The]. Thecurrent current case, case,however, however, was excep- was exceptionaltional in comparison in comparison with with many many previous previouslyly described described pulmonary pulmonary presentations presentations as asthe theFDG-PET/CT FDG-PET/CT scan, scan, in addition in addition to lung to lung lesions, lesions, identified identified enlarged enlarged thoracic thoracic lymph lymph nodes nodesin both in hila, both mediastinum, hila, mediastinum, neck and neck pleural and effusion. pleural effusion.Furthermore, Furthermore, FDG-avid lesions FDG-avid were lesionsobserved were in observedthe prostate in theand, prostate to some and, exte tont, somein the extent, parotid in glands. the parotid The spread glands. pattern The spreadwas very pattern suggestive was very of metastatic suggestive lung of metastatic cancer, which lung was cancer, subsequently which was ruled subsequently out by tis- ruledsue . out by tissue However, biopsies. despite However, a tentative despite malignant a tentative diagnosis malignant based diagnosis on the initial based radio- on thelogical initial findings, radiological standard findings, diagnostic standard procedures diagnostic with procedures EBUS and with cytological EBUS and examination cytological examinationof pleural effusion of pleural performed effusion performedin this case in failed this case to confirm failed to cancer confirm diagnosis. cancer diagnosis. This was Thisrather was unusual rather unusual considering considering the extent the extentand localization and localization of the of thoracic the thoracic lesions. lesions. A small A smallFDG-avid FDG-avid lymph lymph node nodeon the on neck the is neck often is oftena nonspecific a nonspecific finding finding and, in and, case in we case need we to needrule toout rule a malignant out a malignant disease, disease, biopsy biopsy from primary from primary thoracic thoracic lesions lesions with more with moreclear malig- clear malignantnant features features is preferred is preferred to avoid to avoid the therisk risk of prolonging of prolonging the the diagnostic diagnostic process. process. At At the thesame same time, time, FDG-avid FDG-avid lesions lesions were were also also seen seen in in both both parotid parotid glands, glands, which which often often and and in inmost most cases cases represent represent benign benign Wa Warthin’srthin’s tumors. tumors. Therefore, Therefore, surgical surgical biopsy, biopsy, which which is oth- is erwise not a standard diagnostic procedure, was necessary to provide sufficient tissue to

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otherwise not a standard diagnostic procedure, was necessary to provide sufficient tissue to determine histological diagnosis and rule out malignancy. This case report contributes to the previously described importance of surgical biopsy in patients with multiorgan involvement where the ultimate diagnosis is difficult to settle [16]. Evaluating the PET/CT description in the context of metastatic lung cancer, one would expect the patient to be more clinically affected than described. Our case report also confirms the previously reported findings that FDG-PET/CT cannot differentiate between malignant and inflammatory lesions in GPA [17]. Although such radiographical findings might be more indicative of malignancy, the current case, however, underscores the importance of tissue biopsy-based diagnosis with a broad differential diagnostic approach in the initial work-up in patients suspected to have a malignant disease. As reported in a series of 87 open-lung biopsies revealing GPA, 72% had concomitant renal involvement [18], as also confirmed in our case. A hallmark of GPA is the presence of granulomatous inflammation; however, granulomas rarely appear in kidney biopsies in which GPA and MPA share the same lesions. In our lung biopsy, there were no signs of malignancy; however, the findings were not pathognomonic for GPA either as several differential diagnoses can cause necrotizing granulomatous inflammation in the . Accordingly, renal biopsy was performed without further delay revealing pauci-immune extracapillary necrotizing glomerulonephritis consistent with ANCA-associated glomerulonephritis. Thus, considering the clinical, pathological, and biochemical findings, the indisputable diagnosis of GPA was finally settled and the standard treatment with a steroid and cyclophosphamide was initiated and the patient responded with the total remission of all the parameters and today has an almost normal renal function with the estimated glomerular filtration rate (eGFR) of 91 mL/min. Differential diagnosis in AAV patients includes malignancies (both solid tumors and hematological malignancies), infectious diseases, e.g., and , and autoimmune diseases, such as systemic (SLE) and [19,20]. AAV as a paraneoplastic phenomenon seems to be an uncommon presentation of hema- tological malignancies. However, their coexistence was previously observed, e.g., in a case where initial presentation with cutaneous vasculitis led to the diagnosis of [21,22]. Pulmonary malignancies are not usually reported at AAV diagnosis in patients with pulmonary symptoms. However, a case with probable GPA was reported to be misdiagnosed as lung cancer based on fine-needle aspiration biopsy, and responded to chemotherapy [23]. The authors emphasize though that diagnosis based on needle biopsy may not be sufficient and these two diagnoses might coexist. Conversely, there is an extensive number of malignancy studies during AAV follow-up [24–28]. The course of the disease with frequent relapses can be a challenge as lung tumors can be difficult to distinguish from pulmonary AAV activity [29]. In this study, the authors screened PubMed for relevant publications on AAV and pulmonary malignancies and found six GPA patients with associated lung cancer; only one was diagnosed during the first year of GPA diagnosis. There have been reported cases of AAV with lung cancer within two years after onset and many years later (8–10 years). In a Norwegian cohort of 419 AAV patients, the calculated standardized incidence ratios (SIRs) were 1.09 for all cancer types; however, they were not significantly increased. At the same time, non-melanoma skin cancer (NMSC), post- transplant cancer, and hematologic cancer associated with immunosuppressive treatment were significantly elevated [25]. A meta-analysis of observational studies in AAV patients (six studies with a total of 2578 patients) showed higher pooled SIRs of 1.74 [27]. Lower SIRs than previously were reported when follow-up data from the European Vasculitis Study Group clinical trial were analyzed and compared with previous studies, which was contributed to lower total exposure to cyclophosphamide. Presently, a persistent increased risk of overall malignancy, bladder cancer, and pancreatic cancer, as well as a markedly increased risk of squamous cutaneous skin cancer (SCC), was confirmed [28]. On a positive note, there was no increase in the incidence of cancers other than SCC for those treated with <10 g cyclophosphamide, which is a reference standard for the total cyclophosphamide Clin. Pract. 2021, 11 301

exposure when used for inductive immunosuppressive treatment in AAV, including in our hospital.

5. Conclusions Multidisciplinary and cooperative approaches remain paramount in the assessment of abnormal thoracic lesions, including pertinent planning and timing of subsequent diagnostic steps, to facilitate relevant treatment without unnecessary delays. In this case, standard FDG-PET/CT imaging initially gave rise to the suspicion of lung cancer. However, subsequent VATS and kidney biopsy in combination with positive PR3-ANCA refuted malignancy and confirmed the diagnosis of GPA.

Author Contributions: Conceptualization, E.M.U. and W.M.S.; methodology, E.M.U., K.E.N.-K.; software, J.E., B.C.-E., and J.O.L.; validation, E.M.U., K.E.N.-K., and W.M.S.; formal analysis, J.E., B.C.- E., J.O.L., E.M.U., K.E.N.-K., and W.M.S; investigation, J.E., B.C.-E., and J.O.L.; resources, J.E., B.C.-E., and J.O.L., data curation, E.M.U.; writing—original draft preparation, E.M.U.; writing—review and editing, E.M.U., K.E.N.-K., and W.M.S.; visualization, E.M.U., J.O.L.; supervision, W.M.S.; project administration, W.M.S. All authors have read and agreed to the published version of the manuscript. Funding: This research received no external funding. Institutional Review Board Statement: Not applicable. Informed Consent Statement: Written informed consent was obtained from the patient to publish this paper. Conflicts of Interest: The authors declare no conflict of interest.

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