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    "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">We report the case of a 57-year-old man with cough&#44; dyspnea&#44; hemoptysis&#44; and epistaxis&#44; and no history of exposure to drugs&#44; toxic substances or chemicals&#46; He had previously been diagnosed with IgG4-related disease &#40;IgG4-RD&#41; by lacrimal gland biopsy&#44; and also had chronic gastritis&#44; autoimmune hypothyroidism&#44; hypergonadotropic hypogonadism&#44; chronic anemia&#44; and chronic kidney disease stage V&#46; On physical examination&#44; he was afebrile&#44; tachypneic&#44; and bleeding from the nose&#46; Pulmonary auscultation revealed bilateral wet crackles&#46;</p><p id="par0010" class="elsevierStylePara elsevierViewall">Labs&#58; RBC 3&#46;12&#47;mm<span class="elsevierStyleSup">3</span>&#44; Hct&#58; 29&#46;5&#37;&#44; Hb&#58; 9&#46;1<span class="elsevierStyleHsp" style=""></span>g&#47;dL&#44; WBC&#58; 15&#44;360&#47;mm<span class="elsevierStyleSup">3</span> &#40;82&#37; neutrophils&#41;&#44; Platelets&#58; 277&#44;000&#44; Glucose 109<span class="elsevierStyleHsp" style=""></span>mg&#47;dL&#44; Creatinine&#58; 12&#46;46<span class="elsevierStyleHsp" style=""></span>mg&#47;dL&#44; Urea&#58; 161<span class="elsevierStyleHsp" style=""></span>mg&#47;dL&#44; CPK&#58; 72<span class="elsevierStyleHsp" style=""></span>U&#47;L&#44; Na&#58; 142<span class="elsevierStyleHsp" style=""></span>mEquiv&#46;&#47;L&#44; K&#58; 5&#46;6<span class="elsevierStyleHsp" style=""></span>mEquiv&#46;&#47;L&#44; PRO-BNP&#58; 22&#44;644<span class="elsevierStyleHsp" style=""></span>pg&#47;mL&#44; CRP&#58; 20&#46;91<span class="elsevierStyleHsp" style=""></span>mg&#47;dL&#44; PCT&#58; 0&#46;929<span class="elsevierStyleHsp" style=""></span>ng&#47;dL&#46; Arterial blood gases &#40;FiO<span class="elsevierStyleInf">2</span>&#58; 0&#46;21&#41;&#58; pH 7&#46;47&#44; pCO<span class="elsevierStyleInf">2</span>&#58; 34&#46;4<span class="elsevierStyleHsp" style=""></span>mmHg&#44; pO<span class="elsevierStyleInf">2</span>&#58; 59<span class="elsevierStyleHsp" style=""></span>mmHg&#44; HCO<span class="elsevierStyleInf">3</span><span class="elsevierStyleSup">&#8722;</span> 25&#46;4&#46; Normal liver function and coagulation&#46; Chest X-ray with bilateral alveolar pattern&#46; Chest&#44; cranial&#44; and upper airway CT confirmed alveolar consolidation&#44; with thickening of left maxillary sinus mucosa &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>a&#41;&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0015" class="elsevierStylePara elsevierViewall">Fiberoptic bronchoscopy revealed active bleeding in the left bronchial tree and bronchoalveolar lavage showed more than 15&#37; hemosiderophages&#46; DLCO&#58; 120&#37; KCO&#58; 154&#37;&#44; elevated&#46;</p><p id="par0020" class="elsevierStylePara elsevierViewall">Autoimmune testing positive for p-ANCA &#40;Anti-MPO&#58; 33<span class="elsevierStyleHsp" style=""></span>IU&#47;mL&#41; and negative for ANAs&#44; ENAs&#44; C3&#58; 83<span class="elsevierStyleHsp" style=""></span>mg&#47;dL&#44; C4&#58; 30<span class="elsevierStyleHsp" style=""></span>mg&#47;dL&#44; AMA&#44; AML&#44; Anti-MBG and cryoglobulins&#46; Serum IgG&#58; 934<span class="elsevierStyleHsp" style=""></span>mg&#47;dL and IgG4&#58; 38<span class="elsevierStyleHsp" style=""></span>mg&#47;dL&#44; normal&#46;</p><p id="par0025" class="elsevierStylePara elsevierViewall">Nasal biopsy &#40;inferior and middle turbinate&#41; showed 2 medium-caliber vascular structures with walls occupied by polymorphonuclear neutrophils&#44; showing fibrinoid degeneration&#44; consistent with a diagnosis of leukocytoclastic vasculitis &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>b&#41;&#46; Specific immunohistochemical staining was positive for IgG4 &#40;25 IgG4-positive plasma cells per high power field&#41; together with lymphoplasmocytic infiltrate and vascular congestion &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>c&#41;&#46; The patient was diagnosed with microscopic polyangitis-type vasculitis with p-ANCA&#44; alveolar hemorrhage and acute renal failure&#44; and IgG4-RD&#46;</p><p id="par0030" class="elsevierStylePara elsevierViewall">Treatment consisted of cyclophosphamide&#44; prednisone &#40;70<span class="elsevierStyleHsp" style=""></span>mg&#47;kg&#47;day&#41;&#44; plasmapheresis &#40;alternating days&#41; and hemodialysis&#46; The patient&#39;s progress was satisfactory&#44; with no recurrences of alveolar hemorrhage and negativization of p-ANCA&#46; Renal function deterioration required continued hemodialysis&#46; Four years after diagnosis&#44; he received a renal transplant and has shown favorable progress at 1 year&#46;</p><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">Discussion</span><p id="par0035" class="elsevierStylePara elsevierViewall">ANCA-associated vasculitis &#40;AAV&#41;-IgG4-RD overlap syndrome is a new entity that was first identified in 2017&#44;<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">1</span></a> although these findings were described in 2011 in patients with tubulointerstitial nephritis with IgG4-RD and vasculitis&#46;<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">2</span></a></p><p id="par0040" class="elsevierStylePara elsevierViewall">The study by Danlos F-X et al&#46;<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">1</span></a> included 18 patients with a mean age of 55 years&#44; with AAV and IgG4-RD according to Chapel Hill criteria &#40;2013&#41;<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">3</span></a> and the CDC international criteria for IgG4-RD &#40;possible&#44; probable&#44; and definitive&#41;&#46;<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">2</span></a> The diagnosis was concomitant in 72&#37; of the series&#59; in 17&#37;&#44; AAV preceded IgG4-RD and in 11&#37;&#44; IgG4-RD preceded AAV&#46; The diagnosis of IgG4-RD was established as definitive in 28&#37;&#44; probable in 28&#37;&#44; and possible in 44&#37;&#46;<a class="elsevierStyleCrossRefs" href="#bib0030"><span class="elsevierStyleSup">1&#44;2</span></a></p><p id="par0045" class="elsevierStylePara elsevierViewall">Plasma replacement plus glucocorticoids was questioned in the PEXIVAS study&#44;<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">4</span></a> because of its lack of effectiveness in reducing mortality and preserving renal function&#44; but the ACR 2021 consensus statement on the management of AAV recommends its use if there is a high risk of severe renal failure&#46; Rituximab may be used as a first-line treatment in both entities&#46;<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">5</span></a> Multicenter studies are needed to improve knowledge and understanding of this new entity&#46;</p></span></span>"
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