<?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>S2976-05262025000100055</article-id>
<article-id pub-id-type="doi">10.71749/pkj.81</article-id>
<title-group>
<article-title xml:lang="en"><![CDATA[Amyloid Storm: An Uncommon Case of AA Amyloid Nephropathy]]></article-title>
</title-group>
<contrib-group>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Domingos]]></surname>
<given-names><![CDATA[Diogo]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
<xref ref-type="aff" rid="Aaf"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Laranjinha]]></surname>
<given-names><![CDATA[Ivo]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
<xref ref-type="aff" rid="Aaf"/>
<xref ref-type="aff" rid="A a"/>
<xref ref-type="aff" rid="A3"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Manso]]></surname>
<given-names><![CDATA[Rita Theias]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Matias]]></surname>
<given-names><![CDATA[Patrícia]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
<xref ref-type="aff" rid="Aaf"/>
<xref ref-type="aff" rid="A a"/>
<xref ref-type="aff" rid="A3"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Gonçalves]]></surname>
<given-names><![CDATA[Margarida]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
<xref ref-type="aff" rid="Aaf"/>
</contrib>
</contrib-group>
<aff id="Af1">
<institution><![CDATA[,Hospital de Santa Cruz - Unidade Local de Saúde de Lisboa Ocidental Nephrology Department ]]></institution>
<addr-line><![CDATA[Lisboa ]]></addr-line>
<country>Portugal</country>
</aff>
<aff id="Af2">
<institution><![CDATA[,Centro Clínico Académico de Lisboa (CCAL)  ]]></institution>
<addr-line><![CDATA[Lisboa ]]></addr-line>
<country>Portugal</country>
</aff>
<aff id="Af3">
<institution><![CDATA[,Universidade NOVA de Lisboa Faculdade de Ciências Médicas iNOVA4health, NOVA Medical School I]]></institution>
<addr-line><![CDATA[Lisboa ]]></addr-line>
<country>Portugal</country>
</aff>
<aff id="Af4">
<institution><![CDATA[,Hospital de Santa Cruz - Unidade Local de Saúde de Lisboa Ocidental Pathology Department ]]></institution>
<addr-line><![CDATA[Lisboa ]]></addr-line>
<country>Portugal</country>
</aff>
<pub-date pub-type="pub">
<day>00</day>
<month>03</month>
<year>2025</year>
</pub-date>
<pub-date pub-type="epub">
<day>00</day>
<month>03</month>
<year>2025</year>
</pub-date>
<volume>39</volume>
<numero>1</numero>
<fpage>55</fpage>
<lpage>59</lpage>
<copyright-statement/>
<copyright-year/>
<self-uri xlink:href="http://www.scielo.pt/scielo.php?script=sci_arttext&amp;pid=S2976-05262025000100055&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://www.scielo.pt/scielo.php?script=sci_abstract&amp;pid=S2976-05262025000100055&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://www.scielo.pt/scielo.php?script=sci_pdf&amp;pid=S2976-05262025000100055&amp;lng=en&amp;nrm=iso"></self-uri><abstract abstract-type="short" xml:lang="en"><p><![CDATA[Abstract AA amyloidosis is a rare disease caused by tissue accumulation of serum amyloid A (SAA), a protein produced in the liver in response to chronic inflammation or infection. Kidney manifestations of AA amyloidosis usually develop gradually. The authors report a case of a 77&#8209;year&#8209;old patient with a previous diagnosis of multifactorial chronic kidney disease and bronchiectasis, who developed sudden onset nephrotic syndrome and KDIGO stage 3 acute kidney injury shortly after the diagnosis of acute pyelonephritis. A kidney biopsy confirmed the diagnosis of renal AA amyloidosis. Progressive kidney dysfunction ensued, with the need to begin kidney replacement therapy eleven months after the initial diagnosis. This type of renal AA amyloidosis (referred to as &#8220;amyloid storm&#8221;) has been reported in patients with familial Mediterranean fever but was not previously described in AA amyloidosis of other etiologies. The prognosis is poor, especially after dialysis initiation, and more studies are needed to develop strategies to delay its progression and improve outcomes.]]></p></abstract>
<kwd-group>
<kwd lng="en"><![CDATA[Acute Kidney Injury]]></kwd>
<kwd lng="en"><![CDATA[Amyloidosis]]></kwd>
<kwd lng="en"><![CDATA[Nephrotic Syndrome]]></kwd>
<kwd lng="en"><![CDATA[Serum Amyloid A Protein]]></kwd>
</kwd-group>
</article-meta>
</front><back>
<ref-list>
<ref id="B1">
<label>1</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Dember]]></surname>
<given-names><![CDATA[LM]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Amyloidosis-associated kidney disease]]></article-title>
<source><![CDATA[J Am Soc Nephrol]]></source>
<year>2006</year>
<volume>17</volume>
<page-range>3458-71</page-range></nlm-citation>
</ref>
<ref id="B2">
<label>2</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Lachmann]]></surname>
<given-names><![CDATA[HJ]]></given-names>
</name>
<name>
<surname><![CDATA[Goodman]]></surname>
<given-names><![CDATA[HJ]]></given-names>
</name>
<name>
<surname><![CDATA[Gilbertson]]></surname>
<given-names><![CDATA[JA]]></given-names>
</name>
<name>
<surname><![CDATA[Gallimore]]></surname>
<given-names><![CDATA[JR]]></given-names>
</name>
<name>
<surname><![CDATA[Sabin]]></surname>
<given-names><![CDATA[CA]]></given-names>
</name>
<name>
<surname><![CDATA[Gillmore]]></surname>
<given-names><![CDATA[JD]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Natural history and outcome in systemic AA amyloidosis]]></article-title>
<source><![CDATA[N Engl J Med]]></source>
<year>2007</year>
<volume>356</volume>
<page-range>2361-71</page-range></nlm-citation>
</ref>
<ref id="B3">
<label>3</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Pepys]]></surname>
<given-names><![CDATA[MB]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Amyloidosis]]></article-title>
<source><![CDATA[Annu Rev Med]]></source>
<year>2006</year>
<volume>57</volume>
<page-range>223-41</page-range></nlm-citation>
</ref>
<ref id="B4">
<label>4</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Kukuy]]></surname>
<given-names><![CDATA[OL]]></given-names>
</name>
<name>
<surname><![CDATA[Beckerman]]></surname>
<given-names><![CDATA[P]]></given-names>
</name>
<name>
<surname><![CDATA[Dinour]]></surname>
<given-names><![CDATA[D]]></given-names>
</name>
<name>
<surname><![CDATA[Ben Zvi]]></surname>
<given-names><![CDATA[I]]></given-names>
</name>
<name>
<surname><![CDATA[Livneh]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Amyloid storm acute kidney injury and massive proteinuria, rapidly progressing to end-stage kidney disease in AA amyloidosis of familial Mediterranean fever]]></article-title>
<source><![CDATA[Rheumatology]]></source>
<year>2020</year>
<volume>60</volume>
<page-range>3235-42</page-range></nlm-citation>
</ref>
<ref id="B5">
<label>5</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Ayar]]></surname>
<given-names><![CDATA[Y]]></given-names>
</name>
<name>
<surname><![CDATA[Ersoy]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
<name>
<surname><![CDATA[Oksuz]]></surname>
<given-names><![CDATA[MF]]></given-names>
</name>
<name>
<surname><![CDATA[Ocakoglu]]></surname>
<given-names><![CDATA[G]]></given-names>
</name>
<name>
<surname><![CDATA[Vuruskan]]></surname>
<given-names><![CDATA[BA]]></given-names>
</name>
<name>
<surname><![CDATA[Yildiz]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Desfechos clínicos e sobrevida em pacientes com amiloidose AA]]></article-title>
<source><![CDATA[Rev Bras Reum]]></source>
<year>2017</year>
<volume>57</volume>
<page-range>535-44</page-range></nlm-citation>
</ref>
<ref id="B6">
<label>6</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Verine]]></surname>
<given-names><![CDATA[J]]></given-names>
</name>
<name>
<surname><![CDATA[Mourad]]></surname>
<given-names><![CDATA[N]]></given-names>
</name>
<name>
<surname><![CDATA[Desseaux]]></surname>
<given-names><![CDATA[K]]></given-names>
</name>
<name>
<surname><![CDATA[Vanhille]]></surname>
<given-names><![CDATA[P]]></given-names>
</name>
<name>
<surname><![CDATA[Noël]]></surname>
<given-names><![CDATA[LH]]></given-names>
</name>
<name>
<surname><![CDATA[Beau-fils]]></surname>
<given-names><![CDATA[H]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Clinical and histological characteristics of renal AA amyloidosis a retrospective study of 68 cases with a special interest to amyloid -associated inflammatory re-sponse]]></article-title>
<source><![CDATA[Hum Pathol]]></source>
<year>2007</year>
<volume>38</volume>
<page-range>1798-809</page-range></nlm-citation>
</ref>
<ref id="B7">
<label>7</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Erdogmus]]></surname>
<given-names><![CDATA[S]]></given-names>
</name>
<name>
<surname><![CDATA[Celebi]]></surname>
<given-names><![CDATA[ZK]]></given-names>
</name>
<name>
<surname><![CDATA[Akturk]]></surname>
<given-names><![CDATA[S]]></given-names>
</name>
<name>
<surname><![CDATA[Kumru]]></surname>
<given-names><![CDATA[G]]></given-names>
</name>
<name>
<surname><![CDATA[Duman]]></surname>
<given-names><![CDATA[N]]></given-names>
</name>
<name>
<surname><![CDATA[Ates]]></surname>
<given-names><![CDATA[K]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Profile of renal AA amyloidosis in older and younger individuals a single-centre experience]]></article-title>
<source><![CDATA[Amyloid]]></source>
<year>2018</year>
<volume>25</volume>
<page-range>115-9</page-range></nlm-citation>
</ref>
<ref id="B8">
<label>8</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Mirioglu]]></surname>
<given-names><![CDATA[S]]></given-names>
</name>
<name>
<surname><![CDATA[Uludag]]></surname>
<given-names><![CDATA[O]]></given-names>
</name>
<name>
<surname><![CDATA[Hurdogan]]></surname>
<given-names><![CDATA[O]]></given-names>
</name>
<name>
<surname><![CDATA[Kumru]]></surname>
<given-names><![CDATA[G]]></given-names>
</name>
<name>
<surname><![CDATA[Berke]]></surname>
<given-names><![CDATA[I]]></given-names>
</name>
<name>
<surname><![CDATA[Doumas]]></surname>
<given-names><![CDATA[SA]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[AA Amyloidosis A Contemporary View]]></article-title>
<source><![CDATA[Curr Rheumatol Rep]]></source>
<year>2024</year>
<volume>26</volume>
<page-range>248-59</page-range></nlm-citation>
</ref>
<ref id="B9">
<label>9</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Picken]]></surname>
<given-names><![CDATA[MM]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[The pathology of amyloidosis in classification a review]]></article-title>
<source><![CDATA[Acta Haematol]]></source>
<year>2020</year>
<volume>143</volume>
<page-range>322-34</page-range></nlm-citation>
</ref>
<ref id="B10">
<label>10</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Said]]></surname>
<given-names><![CDATA[SM]]></given-names>
</name>
<name>
<surname><![CDATA[Sethi]]></surname>
<given-names><![CDATA[S]]></given-names>
</name>
<name>
<surname><![CDATA[Valeri]]></surname>
<given-names><![CDATA[AM]]></given-names>
</name>
<name>
<surname><![CDATA[Leung]]></surname>
<given-names><![CDATA[N]]></given-names>
</name>
<name>
<surname><![CDATA[Cornell]]></surname>
<given-names><![CDATA[LD]]></given-names>
</name>
<name>
<surname><![CDATA[Fidler]]></surname>
<given-names><![CDATA[ME]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Renal amyloidosis origin and clinicopathologic correlations of 474 recent cases]]></article-title>
<source><![CDATA[Clin J Am Soc Nephrol]]></source>
<year>2013</year>
<volume>8</volume>
<page-range>1515-23</page-range></nlm-citation>
</ref>
<ref id="B11">
<label>11</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Hutten von]]></surname>
<given-names><![CDATA[H]]></given-names>
</name>
<name>
<surname><![CDATA[Mihatsch]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Lobeck]]></surname>
<given-names><![CDATA[H]]></given-names>
</name>
<name>
<surname><![CDATA[Rudolph]]></surname>
<given-names><![CDATA[B]]></given-names>
</name>
<name>
<surname><![CDATA[Eriksson]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Röcken]]></surname>
<given-names><![CDATA[C]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Prevalence and origin of amyloid in kidney biopsies]]></article-title>
<source><![CDATA[Am J Surg Pathol]]></source>
<year>2009</year>
<volume>33</volume>
<page-range>1198-205</page-range></nlm-citation>
</ref>
<ref id="B12">
<label>12</label><nlm-citation citation-type="journal">
<collab>Oliveira -Silva C.Marques N.Pinho A.Poínhos R.Bergantim R.Nunes AT</collab>
<article-title xml:lang=""><![CDATA[Lessons from four decades of systemic amyloidosis with renal involvement]]></article-title>
<source><![CDATA[Nephron]]></source>
<year>2023</year>
<volume>147</volume>
<page-range>158-69</page-range></nlm-citation>
</ref>
<ref id="B13">
<label>13</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Muchtar]]></surname>
<given-names><![CDATA[E]]></given-names>
</name>
<name>
<surname><![CDATA[Dispenzieri]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
<name>
<surname><![CDATA[Magen]]></surname>
<given-names><![CDATA[H]]></given-names>
</name>
<name>
<surname><![CDATA[Grogan]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Mauermann]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[McPhail]]></surname>
<given-names><![CDATA[ED]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Systemic amyloidosis from A (AA) to T (ATTR) a review]]></article-title>
<source><![CDATA[J Intern Med]]></source>
<year>2021</year>
<volume>289</volume>
<page-range>268-92</page-range></nlm-citation>
</ref>
<ref id="B14">
<label>14</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Keeling]]></surname>
<given-names><![CDATA[J]]></given-names>
</name>
<name>
<surname><![CDATA[Teng]]></surname>
<given-names><![CDATA[J]]></given-names>
</name>
<name>
<surname><![CDATA[Herrera]]></surname>
<given-names><![CDATA[GA]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[AL-amyloidosis and light-chain deposition disease light chains induce divergent phenotypic transformations of human mesangial cells]]></article-title>
<source><![CDATA[Lab Investig]]></source>
<year>2004</year>
<volume>84</volume>
<page-range>1322-38</page-range></nlm-citation>
</ref>
<ref id="B15">
<label>15</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Çelebi]]></surname>
<given-names><![CDATA[ZK]]></given-names>
</name>
<name>
<surname><![CDATA[Kiremitçi]]></surname>
<given-names><![CDATA[S]]></given-names>
</name>
<name>
<surname><![CDATA[Sadioglu]]></surname>
<given-names><![CDATA[RE]]></given-names>
</name>
<name>
<surname><![CDATA[Keven]]></surname>
<given-names><![CDATA[K]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Rapidly Progressive Renal Failure in AA Amyloidosis A New Clinical and Histopathological Entity for an Old Disease]]></article-title>
<source><![CDATA[Turk J Nephrol]]></source>
<year>2020</year>
<volume>29</volume>
<page-range>289-96</page-range></nlm-citation>
</ref>
<ref id="B16">
<label>16</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Merlini]]></surname>
<given-names><![CDATA[G]]></given-names>
</name>
<name>
<surname><![CDATA[Bellotti]]></surname>
<given-names><![CDATA[V]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Molecular Mechanisms of Amyloidosis]]></article-title>
<source><![CDATA[N Engl J Med]]></source>
<year>2003</year>
<volume>349</volume>
<page-range>583-96</page-range></nlm-citation>
</ref>
<ref id="B17">
<label>17</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Polverino]]></surname>
<given-names><![CDATA[E]]></given-names>
</name>
<name>
<surname><![CDATA[Goeminne]]></surname>
<given-names><![CDATA[PC]]></given-names>
</name>
<name>
<surname><![CDATA[McDonnell]]></surname>
<given-names><![CDATA[MJ]]></given-names>
</name>
<name>
<surname><![CDATA[Aliberti]]></surname>
<given-names><![CDATA[S]]></given-names>
</name>
<name>
<surname><![CDATA[Marshall]]></surname>
<given-names><![CDATA[SE]]></given-names>
</name>
<name>
<surname><![CDATA[Loebinger]]></surname>
<given-names><![CDATA[MR]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[European Respiratory Society guidelines for the management of adult bronchiectasis]]></article-title>
<source><![CDATA[Eur Respir J]]></source>
<year>2017</year>
<volume>50</volume>
<page-range>1700629</page-range></nlm-citation>
</ref>
<ref id="B18">
<label>18</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Malle]]></surname>
<given-names><![CDATA[E]]></given-names>
</name>
<name>
<surname><![CDATA[Steinmetz]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
<name>
<surname><![CDATA[Raynes]]></surname>
<given-names><![CDATA[JG]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Serum amyloid A (SAA) an acute phase protein and apolipoprotein]]></article-title>
<source><![CDATA[Atherosclerosis]]></source>
<year>1993</year>
<volume>102</volume>
<page-range>131-46</page-range></nlm-citation>
</ref>
<ref id="B19">
<label>19</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Karam]]></surname>
<given-names><![CDATA[S]]></given-names>
</name>
<name>
<surname><![CDATA[Haidous]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Royal]]></surname>
<given-names><![CDATA[V]]></given-names>
</name>
<name>
<surname><![CDATA[Leung]]></surname>
<given-names><![CDATA[N]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Renal AA amyloidosis presentation, diagnosis, and current therapeutic options: a review]]></article-title>
<source><![CDATA[Kidney Int]]></source>
<year>2023</year>
<volume>103</volume>
<page-range>473-84</page-range></nlm-citation>
</ref>
<ref id="B20">
<label>20</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Yilmaz]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Unsal]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
<name>
<surname><![CDATA[Sokmen]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Kaptanogullari]]></surname>
<given-names><![CDATA[OH]]></given-names>
</name>
<name>
<surname><![CDATA[Alkim]]></surname>
<given-names><![CDATA[C]]></given-names>
</name>
<name>
<surname><![CDATA[Kabukcuoglu]]></surname>
<given-names><![CDATA[F]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Renal Involvement in AA Amyloidosis Clinical Outcomes and Survival]]></article-title>
<source><![CDATA[Kidney Blood Press Res]]></source>
<year>2013</year>
<volume>37</volume>
<page-range>33-42</page-range></nlm-citation>
</ref>
</ref-list>
</back>
</article>
