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Blood gas test showed severe hypoxemia&#46; Urinalysis showed haematuria &#40;200&#47;mm3&#41; and proteinuria 300<span class="elsevierStyleHsp" style=""></span>mg&#47;dL&#44; without casts&#46; Chest X-ray revealed bilateral diffuse opacities &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>&#41;&#44; and chest CT suggested pulmonary hemorrhage&#46; Ultrasonogram showed normal sized kidneys with increased echogenicity&#46; She required mechanical ventilation and hemodialysis&#46; Bronchofibroscopy revealed alveolar hemorrhage&#44; and plasmapheresis was initiated&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0015" class="elsevierStylePara elsevierViewall">Complementary investigation showed nephrotic range proteinuria &#40;4&#46;7<span class="elsevierStyleHsp" style=""></span>g&#47;24<span class="elsevierStyleHsp" style=""></span>h&#41;&#46; Serum protein electrophoresis showed an alpha2 spike and hypogamaglobulinemia&#46; Serum complement was normal and serology for lupus&#44; vasculitis and cryoglobulinemia&#44; as well as for human immunodeficiency virus&#44; hepatitis B and C infections were negative&#46; Echocardiogram revealed a type II diastolic dysfunction&#46; A renal biopsy was performed and revealed nodular glomerulosclerosis&#46; Immunofluorescence revealed linear staining for kappa light chains along the tubular basement membrane and also in the glomerulus&#44; allowing the diagnosis of light chain deposits disease &#40;LCDD&#41; &#40;<a class="elsevierStyleCrossRefs" href="#fig0010">Figs&#46; 2 and 3</a>&#41;&#46; Serum immunofixation revealed a kappa light chain band&#44; and urine immunofixation revealed Bence-Jones kappa&#46; Bone marrow biopsy and aspirate showed normocellular marrow with 10&#37; monoclonal plasmocytosis&#46; Therefore&#44; multiple myeloma was diagnosed&#46; She received chemotherapy and an autologous hematopoietic cell transplant&#44; achieving maintained complete hematological response&#46; At one-year of follow-up&#44; she remains dialysis-dependent&#46;</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia><elsevierMultimedia ident="fig0015"></elsevierMultimedia><p id="par0020" class="elsevierStylePara elsevierViewall">The initial presentation led us to consider an immunological cause for the pulmonary renal syndrome&#44; despite the negative immunological results&#44; which might occur in 10&#8211;20&#37; of the PRS of immunological origin&#46; The unexpected nodular glomerulosclerosis on the kidney biopsy led us to further investigate an hematological disease&#44; because together with diabetes mellitus and smoking&#44; light or heavy chain deposits disease is one of the main causes of nodular glomerulosclerosis&#46; The investigation of the hematological disease was consistent with a diagnosis of multiple myeloma&#44; according to the International Myeloma Working Group criteria&#46;<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">2</span></a></p><p id="par0025" class="elsevierStylePara elsevierViewall">LCDD is a rare renal manifestation of plasma cell disorders&#46;<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">3</span></a> LCDD is a systemic disease with renal&#44; cardiac&#44; pulmonary&#44; hepatic and gastrointestinal involvement&#46;<a class="elsevierStyleCrossRefs" href="#bib0045"><span class="elsevierStyleSup">3&#8211;5</span></a> Renal involvement is the most frequent and manifests as nephrotic syndrome and renal insufficiency&#44; typically rapidly progressive&#46; Renal biopsy typically reveals nodular glomerulosclerosis and thickening of the tubular basement membrane&#46; In 80&#37; of the cases it is characterized by the deposition of kappa light chains along the glomerular capillaries&#44; nodules and the tubular basement membrane&#46; Electron microscopy reveals granular deposits&#46;<a class="elsevierStyleCrossRefs" href="#bib0050"><span class="elsevierStyleSup">4&#44;6</span></a></p><p id="par0030" class="elsevierStylePara elsevierViewall">Although we cannot conclude on the cause of the pulmonary hemorrhage&#44; because of the lack of pulmonary biopsy&#44; we speculate on pulmonary tissue LC deposition&#44; as this seems to be a case of systemic LC deposition&#58; kidney&#44; lung&#44; heart&#44; skin and articulations&#46; This case highlights that LCDD should be kept in mind in the differential diagnosis of PRS&#46;</p></span>"
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        "texto" => "<p id="par0035" class="elsevierStylePara elsevierViewall">The authors would like to acknowledge Helena Viana&#44; MD and Fernanda Carvalho&#44; MD&#44; who were responsible for the kidney biopsy results and supplied the images&#46;</p>"
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Letter to the Editor
Pulmonary-renal syndrome as a clinical expression of multiple myeloma
Síndrome pulmón-riñón como una expresión clínica de mieloma múltiple
Joana Gameiro
Autor para correspondencia
joana.estrelagameiro@gmail.com

Corresponding author.
, Sofia Jorge, José António Lopes, António Gomes da Costa
Service of Nephrology and Renal Transplantation, Department of Medicine Centro Hospitalar Lisboa Norte, EPE Av. Prof. Egas Moniz, 1649-035 Lisboa, Portugal
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Blood gas test showed severe hypoxemia&#46; Urinalysis showed haematuria &#40;200&#47;mm3&#41; and proteinuria 300<span class="elsevierStyleHsp" style=""></span>mg&#47;dL&#44; without casts&#46; Chest X-ray revealed bilateral diffuse opacities &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>&#41;&#44; and chest CT suggested pulmonary hemorrhage&#46; Ultrasonogram showed normal sized kidneys with increased echogenicity&#46; She required mechanical ventilation and hemodialysis&#46; Bronchofibroscopy revealed alveolar hemorrhage&#44; and plasmapheresis was initiated&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0015" class="elsevierStylePara elsevierViewall">Complementary investigation showed nephrotic range proteinuria &#40;4&#46;7<span class="elsevierStyleHsp" style=""></span>g&#47;24<span class="elsevierStyleHsp" style=""></span>h&#41;&#46; Serum protein electrophoresis showed an alpha2 spike and hypogamaglobulinemia&#46; Serum complement was normal and serology for lupus&#44; vasculitis and cryoglobulinemia&#44; as well as for human immunodeficiency virus&#44; hepatitis B and C infections were negative&#46; Echocardiogram revealed a type II diastolic dysfunction&#46; A renal biopsy was performed and revealed nodular glomerulosclerosis&#46; Immunofluorescence revealed linear staining for kappa light chains along the tubular basement membrane and also in the glomerulus&#44; allowing the diagnosis of light chain deposits disease &#40;LCDD&#41; &#40;<a class="elsevierStyleCrossRefs" href="#fig0010">Figs&#46; 2 and 3</a>&#41;&#46; Serum immunofixation revealed a kappa light chain band&#44; and urine immunofixation revealed Bence-Jones kappa&#46; Bone marrow biopsy and aspirate showed normocellular marrow with 10&#37; monoclonal plasmocytosis&#46; Therefore&#44; multiple myeloma was diagnosed&#46; She received chemotherapy and an autologous hematopoietic cell transplant&#44; achieving maintained complete hematological response&#46; At one-year of follow-up&#44; she remains dialysis-dependent&#46;</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia><elsevierMultimedia ident="fig0015"></elsevierMultimedia><p id="par0020" class="elsevierStylePara elsevierViewall">The initial presentation led us to consider an immunological cause for the pulmonary renal syndrome&#44; despite the negative immunological results&#44; which might occur in 10&#8211;20&#37; of the PRS of immunological origin&#46; The unexpected nodular glomerulosclerosis on the kidney biopsy led us to further investigate an hematological disease&#44; because together with diabetes mellitus and smoking&#44; light or heavy chain deposits disease is one of the main causes of nodular glomerulosclerosis&#46; The investigation of the hematological disease was consistent with a diagnosis of multiple myeloma&#44; according to the International Myeloma Working Group criteria&#46;<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">2</span></a></p><p id="par0025" class="elsevierStylePara elsevierViewall">LCDD is a rare renal manifestation of plasma cell disorders&#46;<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">3</span></a> LCDD is a systemic disease with renal&#44; cardiac&#44; pulmonary&#44; hepatic and gastrointestinal involvement&#46;<a class="elsevierStyleCrossRefs" href="#bib0045"><span class="elsevierStyleSup">3&#8211;5</span></a> Renal involvement is the most frequent and manifests as nephrotic syndrome and renal insufficiency&#44; typically rapidly progressive&#46; Renal biopsy typically reveals nodular glomerulosclerosis and thickening of the tubular basement membrane&#46; In 80&#37; of the cases it is characterized by the deposition of kappa light chains along the glomerular capillaries&#44; nodules and the tubular basement membrane&#46; Electron microscopy reveals granular deposits&#46;<a class="elsevierStyleCrossRefs" href="#bib0050"><span class="elsevierStyleSup">4&#44;6</span></a></p><p id="par0030" class="elsevierStylePara elsevierViewall">Although we cannot conclude on the cause of the pulmonary hemorrhage&#44; because of the lack of pulmonary biopsy&#44; we speculate on pulmonary tissue LC deposition&#44; as this seems to be a case of systemic LC deposition&#58; kidney&#44; lung&#44; heart&#44; skin and articulations&#46; This case highlights that LCDD should be kept in mind in the differential diagnosis of PRS&#46;</p></span>"
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        "texto" => "<p id="par0035" class="elsevierStylePara elsevierViewall">The authors would like to acknowledge Helena Viana&#44; MD and Fernanda Carvalho&#44; MD&#44; who were responsible for the kidney biopsy results and supplied the images&#46;</p>"
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