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1 and 2</a>&#41;&#46; Treatment was initiated with a bolus of 500<span class="elsevierStyleHsp" style=""></span>mg&#47;m<span class="elsevierStyleSup">2</span> cyclophosphamide and immunoglobulins at 2<span class="elsevierStyleHsp" style=""></span>g&#47;kg&#44; spread across five doses&#46; Five days later&#44; frank haemoptysis&#44; anaemia and significant respiratory effort occurred&#44; prompting a transfer to ICU&#44; where orotracheal intubation and mechanical ventilation were performed&#46; The patient also required aspiration due to massive bleeding&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0020" class="elsevierStylePara elsevierViewall">During the patient&#39;s stay in the ICU&#44; six sessions of plasmapheresis were performed on alternate days&#44; and treatment with corticosteroids was maintained&#46; A clinical and analytical improvement was seen over the subsequent days and extubation was performed 13 days later&#44; with normalised kidney function and no new bleeding episodes&#46; Finally&#44; a kidney biopsy was performed in which 15 glomeruli were observed&#44; four of which were sclerosed while the others showed the presence of diffuse segmental lesions in the form of endocapillary and mesangial proliferation and glomerular necrotic lesions&#46; Interstitium with mild fibrosis and patchy inflammatory infiltrate&#46; Positive immunofluorescence in the face of granular mesangial IgA &#40;&#43;&#43;&#41;&#44; IgM &#40;&#43;&#43;&#41; and C3 &#40;&#43;&#43;&#41;&#46; After three months in hospital&#44; the patient was discharged on a monthly immunoglobulin treatment and a decreasing corticosteroid regimen&#46; Due to the risk of superinfection&#44; combined treatment with other immunosuppressants was postponed until the pulmonary haematoma had resolved&#46;</p><p id="par0025" class="elsevierStylePara elsevierViewall">At the last hospital visit&#44; from a clinical point of view the patient presented without dyspnoea&#44; and clear improvements were seen in his lung X-ray and laboratory tests&#58; creatinine 1&#46;2<span class="elsevierStyleHsp" style=""></span>mg&#47;dl&#44; GFR 61<span class="elsevierStyleHsp" style=""></span>ml&#47;min&#44; urine with minimal microscopic haematuria &#40;15&#8211;20 red blood cells&#47;field&#41; with a urine protein&#58;creatinine ratio of 238&#46;6<span class="elsevierStyleHsp" style=""></span>mg&#47;g&#44; and no anaemia or leukocytosis&#46;</p><p id="par0030" class="elsevierStylePara elsevierViewall">HSP is a form of leukocytoclastic vasculitis that affects the small vessels and&#44; on rare occasions&#44; the medium vessels&#46; Its symptoms are characterised by non-thrombocytopenic palpable purpura &#40;100&#37; of cases&#41;&#44; arthralgia in the lower limbs &#40;82&#37;&#41;&#44; gastrointestinal involvement &#40;50&#8211;75&#37;&#41; and renal involvement &#40;20&#8211;50&#37;&#41;&#46; Musculoskeletal&#44; neurological&#44; pulmonary&#44; cardiac and ocular conditions have been described less frequently&#46;<a class="elsevierStyleCrossRefs" href="#bib0050"><span class="elsevierStyleSup">1&#44;2</span></a> The incidence in adults is around 1&#46;3 cases per 100&#44;000&#46;<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">3</span></a></p><p id="par0035" class="elsevierStylePara elsevierViewall">The EULAR&#47;PRINTO&#47;PRES diagnostic criteria &#40;2008&#41; for HSP include&#44; as a mandatory criterion&#44; the existence of non-thrombocytopenic palpable purpura&#44; predominantly in the lower limbs and&#44; in case of atypical distribution&#44; proven IgA deposition in the biopsy&#46; In addition&#44; at least one of the following characteristics is required&#58; diffuse abdominal pain&#44; biopsy with IgA deposition&#44; arthritis or arthralgia and renal involvement &#40;haematuria or proteinuria&#41;&#46;<a class="elsevierStyleCrossRef" href="#bib0065"><span class="elsevierStyleSup">4</span></a></p><p id="par0040" class="elsevierStylePara elsevierViewall">Renal involvement usually manifests in the form of mild glomerulonephritis&#44; with microscopic haematuria and proteinuria&#44; and a biopsy showing IgA nephropathy&#46;<a class="elsevierStyleCrossRefs" href="#bib0070"><span class="elsevierStyleSup">5&#44;6</span></a></p><p id="par0045" class="elsevierStylePara elsevierViewall">Lung involvement in HSP is extremely rare and includes&#44; above all&#44; haemorrhage or interstitial disease&#46; Diffuse alveolar haemorrhage&#44; which may present immediately following the HSP diagnosis&#44; or even several years later&#44; has an unpredictable clinical evolution and is associated with high rates of mortality&#46;<a class="elsevierStyleCrossRefs" href="#bib0080"><span class="elsevierStyleSup">7&#44;8</span></a></p><p id="par0050" class="elsevierStylePara elsevierViewall">Chest X-rays are usually non-specific and computed tomography is required to confirm the findings&#46; The severity of the situation warrants the use of intensive immunosuppressive therapy<a class="elsevierStyleCrossRef" href="#bib0090"><span class="elsevierStyleSup">9</span></a>&#59; treatment combined with glucocorticoids and cyclophosphamide&#44; azathioprine&#44; methotrexate or rituximab is common&#46; Plasmapheresis has also been noted as a therapeutic option&#46;</p><p id="par0055" class="elsevierStylePara elsevierViewall">The most common aetiologies that coexist with alveolar haemorrhage and glomerulonephritis are anti-neutrophil cytoplasmic antibody-associated vasculitis and anti-glomerular basement membrane antibody disease&#46; Although uncommon&#44; HSP should be considered among the differential diagnoses in a patient with pulmonary haemorrhage and renal involvement&#46;</p></span>"
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Letter to the Editor
Pulmonary haemorrhage in a patient with IgA nefropathy
Hemorragia pulmonar en paciente con nefropatía IgA
Lara Belmar Vegaa,,
Corresponding author
belmarvega@outlook.es

Corresponding author.
, Carlos Fernández-Díazb,, Natalia Palmou Fontanab, Emilio Rodrigo Calabiaa, Luis Martin Penagosa, Manuel Arias Rodrígueza, Gema Fernández Fresnedoa
a Servicio de Nefrología, Hospital Universitario Marqués de Valdecilla, Santander, Spain
b Servicio de Reumatología, Hospital Universitario Marqués de Valdecilla, Santander, Spain
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GFR 41<span class="elsevierStyleHsp" style=""></span>ml&#47;min&#41; as well as leukocytosis with a left shift&#46; Following diuretic and antibiotic treatment&#44; an improvement in kidney function occurred &#40;Cr 0&#46;9<span class="elsevierStyleHsp" style=""></span>mg&#47;dl&#44; GFR 82<span class="elsevierStyleHsp" style=""></span>ml&#47;min&#41; and the leukocytosis decreased&#46; A few days later&#44; the patient presented again deterioration of kidney function &#40;Cr 2&#46;1<span class="elsevierStyleHsp" style=""></span>mg&#47;dl&#41;&#44; anaemia &#40;Hb 7&#46;5<span class="elsevierStyleHsp" style=""></span>g&#47;dl&#41; along with purpuric lesions of the lower limbs&#46; The tests performed showed&#58; normal albumin&#44; cholesterol and lactate dehydrogenase&#59; an ESR of 52<span class="elsevierStyleHsp" style=""></span>mm&#44; urine element and sediment with &#43;&#43;&#43;proteins&#44; &#43;&#43;&#43;&#43;Hb&#44; &#62;100 red blood cells&#47;field&#44; 24-hour urine protein of 1870<span class="elsevierStyleHsp" style=""></span>mg&#59; IgA 437&#46;00<span class="elsevierStyleHsp" style=""></span>mg&#47;dl&#44; normal remaining immunoglobulins and complement&#44; antinuclear antibodies&#44; anti-neutrophil cytoplasmic antibodies&#44; anti-glomerular basement membrane antibodies and negative viral serology testing&#46; Haptoglobins &#60; 25&#44; blood smear without schistocytes&#44; positive direct Coombs test&#46; Urological ultrasound with no pathological findings&#46;</p><p id="par0015" class="elsevierStylePara elsevierViewall">In light of suspected vasculitis&#44; treatment was initiated with three boli of methylprednisolone 500<span class="elsevierStyleHsp" style=""></span>mg on consecutive days&#44; followed by prednisone at 1<span class="elsevierStyleHsp" style=""></span>mg&#47;kg every 24 hours&#46; 72 hours later&#44; symptoms of haemoptysis and anaemia appeared&#59; a chest X-ray and chest CT scan were performed&#44; showing findings suggestive of a bilateral alveolar haemorrhage and the presence of intraparenchymal pulmonary haematoma &#40;<a class="elsevierStyleCrossRefs" href="#fig0005">Figs&#46; 1 and 2</a>&#41;&#46; Treatment was initiated with a bolus of 500<span class="elsevierStyleHsp" style=""></span>mg&#47;m<span class="elsevierStyleSup">2</span> cyclophosphamide and immunoglobulins at 2<span class="elsevierStyleHsp" style=""></span>g&#47;kg&#44; spread across five doses&#46; Five days later&#44; frank haemoptysis&#44; anaemia and significant respiratory effort occurred&#44; prompting a transfer to ICU&#44; where orotracheal intubation and mechanical ventilation were performed&#46; The patient also required aspiration due to massive bleeding&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0020" class="elsevierStylePara elsevierViewall">During the patient&#39;s stay in the ICU&#44; six sessions of plasmapheresis were performed on alternate days&#44; and treatment with corticosteroids was maintained&#46; A clinical and analytical improvement was seen over the subsequent days and extubation was performed 13 days later&#44; with normalised kidney function and no new bleeding episodes&#46; Finally&#44; a kidney biopsy was performed in which 15 glomeruli were observed&#44; four of which were sclerosed while the others showed the presence of diffuse segmental lesions in the form of endocapillary and mesangial proliferation and glomerular necrotic lesions&#46; Interstitium with mild fibrosis and patchy inflammatory infiltrate&#46; Positive immunofluorescence in the face of granular mesangial IgA &#40;&#43;&#43;&#41;&#44; IgM &#40;&#43;&#43;&#41; and C3 &#40;&#43;&#43;&#41;&#46; After three months in hospital&#44; the patient was discharged on a monthly immunoglobulin treatment and a decreasing corticosteroid regimen&#46; Due to the risk of superinfection&#44; combined treatment with other immunosuppressants was postponed until the pulmonary haematoma had resolved&#46;</p><p id="par0025" class="elsevierStylePara elsevierViewall">At the last hospital visit&#44; from a clinical point of view the patient presented without dyspnoea&#44; and clear improvements were seen in his lung X-ray and laboratory tests&#58; creatinine 1&#46;2<span class="elsevierStyleHsp" style=""></span>mg&#47;dl&#44; GFR 61<span class="elsevierStyleHsp" style=""></span>ml&#47;min&#44; urine with minimal microscopic haematuria &#40;15&#8211;20 red blood cells&#47;field&#41; with a urine protein&#58;creatinine ratio of 238&#46;6<span class="elsevierStyleHsp" style=""></span>mg&#47;g&#44; and no anaemia or leukocytosis&#46;</p><p id="par0030" class="elsevierStylePara elsevierViewall">HSP is a form of leukocytoclastic vasculitis that affects the small vessels and&#44; on rare occasions&#44; the medium vessels&#46; Its symptoms are characterised by non-thrombocytopenic palpable purpura &#40;100&#37; of cases&#41;&#44; arthralgia in the lower limbs &#40;82&#37;&#41;&#44; gastrointestinal involvement &#40;50&#8211;75&#37;&#41; and renal involvement &#40;20&#8211;50&#37;&#41;&#46; Musculoskeletal&#44; neurological&#44; pulmonary&#44; cardiac and ocular conditions have been described less frequently&#46;<a class="elsevierStyleCrossRefs" href="#bib0050"><span class="elsevierStyleSup">1&#44;2</span></a> The incidence in adults is around 1&#46;3 cases per 100&#44;000&#46;<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">3</span></a></p><p id="par0035" class="elsevierStylePara elsevierViewall">The EULAR&#47;PRINTO&#47;PRES diagnostic criteria &#40;2008&#41; for HSP include&#44; as a mandatory criterion&#44; the existence of non-thrombocytopenic palpable purpura&#44; predominantly in the lower limbs and&#44; in case of atypical distribution&#44; proven IgA deposition in the biopsy&#46; In addition&#44; at least one of the following characteristics is required&#58; diffuse abdominal pain&#44; biopsy with IgA deposition&#44; arthritis or arthralgia and renal involvement &#40;haematuria or proteinuria&#41;&#46;<a class="elsevierStyleCrossRef" href="#bib0065"><span class="elsevierStyleSup">4</span></a></p><p id="par0040" class="elsevierStylePara elsevierViewall">Renal involvement usually manifests in the form of mild glomerulonephritis&#44; with microscopic haematuria and proteinuria&#44; and a biopsy showing IgA nephropathy&#46;<a class="elsevierStyleCrossRefs" href="#bib0070"><span class="elsevierStyleSup">5&#44;6</span></a></p><p id="par0045" class="elsevierStylePara elsevierViewall">Lung involvement in HSP is extremely rare and includes&#44; above all&#44; haemorrhage or interstitial disease&#46; Diffuse alveolar haemorrhage&#44; which may present immediately following the HSP diagnosis&#44; or even several years later&#44; has an unpredictable clinical evolution and is associated with high rates of mortality&#46;<a class="elsevierStyleCrossRefs" href="#bib0080"><span class="elsevierStyleSup">7&#44;8</span></a></p><p id="par0050" class="elsevierStylePara elsevierViewall">Chest X-rays are usually non-specific and computed tomography is required to confirm the findings&#46; The severity of the situation warrants the use of intensive immunosuppressive therapy<a class="elsevierStyleCrossRef" href="#bib0090"><span class="elsevierStyleSup">9</span></a>&#59; treatment combined with glucocorticoids and cyclophosphamide&#44; azathioprine&#44; methotrexate or rituximab is common&#46; Plasmapheresis has also been noted as a therapeutic option&#46;</p><p id="par0055" class="elsevierStylePara elsevierViewall">The most common aetiologies that coexist with alveolar haemorrhage and glomerulonephritis are anti-neutrophil cytoplasmic antibody-associated vasculitis and anti-glomerular basement membrane antibody disease&#46; 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