<?xml version="1.0" encoding="ISO-8859-1"?><article xmlns:mml="http://www.w3.org/1998/Math/MathML" xmlns:xlink="http://www.w3.org/1999/xlink" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance">
<front>
<journal-meta>
<journal-id>2976-0526</journal-id>
<journal-title><![CDATA[Portuguese Kidney Journal (PKJ)]]></journal-title>
<abbrev-journal-title><![CDATA[PKJ]]></abbrev-journal-title>
<issn>2976-0526</issn>
<publisher>
<publisher-name><![CDATA[Sociedade Portuguesa de Nefrologia]]></publisher-name>
</publisher>
</journal-meta>
<article-meta>
<article-id>S2976-05262024000100058</article-id>
<article-id pub-id-type="doi">10.71749/pkj.6</article-id>
<title-group>
<article-title xml:lang="en"><![CDATA[Membranous Nephropathy as a Manifestation of IgG4&#8209;Related Disease (IgG4&#8209;RD): A Case Report]]></article-title>
</title-group>
<contrib-group>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Quaresma]]></surname>
<given-names><![CDATA[Marta]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Cunha]]></surname>
<given-names><![CDATA[Teresa Stock da]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Ferreira]]></surname>
<given-names><![CDATA[Joel]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Góis]]></surname>
<given-names><![CDATA[Mário]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Azevedo]]></surname>
<given-names><![CDATA[Ana]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
</contrib-group>
<aff id="Af1">
<institution><![CDATA[,Hospital de Vila Franca de Xira Internal Medicine Department ]]></institution>
<addr-line><![CDATA[Vila Franca de Xira ]]></addr-line>
<country>Portugal</country>
</aff>
<aff id="Af2">
<institution><![CDATA[,Hospital de Vila Franca de Xira Nephrology Department ]]></institution>
<addr-line><![CDATA[Vila Franca de Xira ]]></addr-line>
<country>Portugal</country>
</aff>
<aff id="Af3">
<institution><![CDATA[,Centro Hospitalar e Universitario de Lisboa Central Hospital de Curry Cabral Laboratory of Renal Morphology]]></institution>
<addr-line><![CDATA[Lisbon ]]></addr-line>
<country>Portugal</country>
</aff>
<pub-date pub-type="pub">
<day>00</day>
<month>03</month>
<year>2024</year>
</pub-date>
<pub-date pub-type="epub">
<day>00</day>
<month>03</month>
<year>2024</year>
</pub-date>
<volume>38</volume>
<numero>1</numero>
<fpage>58</fpage>
<lpage>63</lpage>
<copyright-statement/>
<copyright-year/>
<self-uri xlink:href="http://www.scielo.pt/scielo.php?script=sci_arttext&amp;pid=S2976-05262024000100058&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://www.scielo.pt/scielo.php?script=sci_abstract&amp;pid=S2976-05262024000100058&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://www.scielo.pt/scielo.php?script=sci_pdf&amp;pid=S2976-05262024000100058&amp;lng=en&amp;nrm=iso"></self-uri><abstract abstract-type="short" xml:lang="en"><p><![CDATA[Abstract IgG4&#8209;related disease (IgG4&#8209;RD) is an immune&#8209;mediated, inflammatory condition that can affect multiple organs, marked by lymphoplasmocytic infiltration and IgG4&#8209;positive plasma cells. Elevated serum IgG4 is common but not essential. Sole renal manifestation is rare, usually appearing as tubulointerstitial nephritis (TIN) or, less commonly, membranous nephropathy (MN). We present a case of an 87&#8209;year&#8209;old man with a medical history of hypertension, dyslipidemia, ischemic cardiomyopathy, and COPD. The patient presented with nephrotic syndrome (proteinuria/creatininuria ratio (P/C ratio) of 11 g/g) and acute kidney injury (creatinine 4 mg/dL). Urinalysis indicated proteinuria and haematuria without dysmorphic erythrocytes. Investigation revealed normochromic normocytic anaemia, hypoalbuminemia, and no evidence of monoclonal gammopathy or hypocomplementemia. Serologies for HIV, hepatitis B, and hepatitis C were negative. Antinuclear, anti&#8209;neutrophil, and anti&#8209;phospholipase A2 antibodies (anti&#8209;PLA2r) were also negative. Treatment including anti&#8209;proteinuric and anticoagulation therapies was initiated. Kidney biopsy displayed membranous nephropathy and interstitial nephritis; immunofluorescence showed parietal granular deposits of IgG&#8209;&#954; and IgG&#8209;&#955;; immunohistochemistry revealed lymphocytes, plasma cells (with more than 10 IgG4&#8209;positive cells per high&#8209;power field), and IgG4 deposits on the glomerular basement membrane. Serum IgG subclass levels detected a slight elevation of IgG4. Other organ involvements hinting at IgG4&#8209;RD were ruled out. Assuming IgG4&#8209;RD with renal involvement therapy with prednisolone (40 mg/day) was started and after one month the patient demonstrated a significant improvement in kidney function, anaemia, hypoalbuminemia, and proteinuria. Although renal manifestation as the first and/or only manifestation of IgG4&#8209;RD is rare, should be suspected in renal cases showing MN with or without TIN, especially when anti&#8209;PLA2R is negative.]]></p></abstract>
<kwd-group>
<kwd lng="en"><![CDATA[Glomerulonephritis, Membranous]]></kwd>
<kwd lng="en"><![CDATA[Immunoglobulin G4&#8209;Related Disease]]></kwd>
</kwd-group>
</article-meta>
</front><back>
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