Catastrophic antiphospholipid syndrome as a complication of systemic sclerosis

A 62-year-old man with a history of systemic sclerosis was admitted with diffuse alveolar hemorrhage and acute kidney injury without clinical data suggestive of glomerulonephritis. Laboratory tests showed anemia, leukocytosis with neutrophilia, thrombocytopenia, elevated serum creatinine and metabo...

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Main Authors: D.J. Manzella, L. Vicente, A.A. Pérez de la Hoz, R.J. Zamora, G. De Rosa, A.A. Pisarevsky
Format: Article
Language:English
Published: PAGEPress Publications 2019-07-01
Series:Reumatismo
Subjects:
Online Access:https://www.reumatismo.org/index.php/reuma/article/view/1095
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spelling doaj-80c443c82d1a402fa73f37291f21b6972020-11-25T00:16:16ZengPAGEPress PublicationsReumatismo0048-74492240-26832019-07-0171210.4081/reumatismo.2019.1095Catastrophic antiphospholipid syndrome as a complication of systemic sclerosisD.J. Manzella0L. Vicente1A.A. Pérez de la Hoz2R.J. Zamora3G. De Rosa4A.A. Pisarevsky5Department of Medicine, Hospital de Clínicas José de San Martín, University of Buenos Aires, Buenos AiresDepartment of Medicine, Hospital de Clínicas José de San Martín, University of Buenos Aires, Buenos AiresDepartment of Medicine, Hospital de Clínicas José de San Martín, University of Buenos Aires, Buenos AiresDepartment of Medicine, Hospital de Clínicas José de San Martín, University of Buenos Aires, Buenos AiresDepartment of Pathology, Hospital de Clínicas José de San Martín, University of Buenos Aires, Buenos AiresDepartment of Medicine, Hospital de Clínicas José de San Martín, University of Buenos Aires, Buenos Aires A 62-year-old man with a history of systemic sclerosis was admitted with diffuse alveolar hemorrhage and acute kidney injury without clinical data suggestive of glomerulonephritis. Laboratory tests showed anemia, leukocytosis with neutrophilia, thrombocytopenia, elevated serum creatinine and metabolic acidosis. Antinuclear antibodies were positive at a titer of 1/640 (speckled, 1/160; nucleolar, 1/320) while rheumatoid factor, anti Scl-70, anti-centromere, anti-neutrophil cytoplasmic antibody and anti-glomerular basement membrane antibodies were negative and serum complement levels were within normal range. During the following days, the patient developed multiple organ failure and, eventually, died. Lupus anticoagulant was revealed positive after the patient’s death, suggesting a catastrophic antiphospholipid syndrome. Clinical data and autopsy were consistent with this diagnosis. https://www.reumatismo.org/index.php/reuma/article/view/1095Systemic sclerosiscatastrophic antiphospholipid syndromepulmonary renal syndromemultiple organ failure.
collection DOAJ
language English
format Article
sources DOAJ
author D.J. Manzella
L. Vicente
A.A. Pérez de la Hoz
R.J. Zamora
G. De Rosa
A.A. Pisarevsky
spellingShingle D.J. Manzella
L. Vicente
A.A. Pérez de la Hoz
R.J. Zamora
G. De Rosa
A.A. Pisarevsky
Catastrophic antiphospholipid syndrome as a complication of systemic sclerosis
Reumatismo
Systemic sclerosis
catastrophic antiphospholipid syndrome
pulmonary renal syndrome
multiple organ failure.
author_facet D.J. Manzella
L. Vicente
A.A. Pérez de la Hoz
R.J. Zamora
G. De Rosa
A.A. Pisarevsky
author_sort D.J. Manzella
title Catastrophic antiphospholipid syndrome as a complication of systemic sclerosis
title_short Catastrophic antiphospholipid syndrome as a complication of systemic sclerosis
title_full Catastrophic antiphospholipid syndrome as a complication of systemic sclerosis
title_fullStr Catastrophic antiphospholipid syndrome as a complication of systemic sclerosis
title_full_unstemmed Catastrophic antiphospholipid syndrome as a complication of systemic sclerosis
title_sort catastrophic antiphospholipid syndrome as a complication of systemic sclerosis
publisher PAGEPress Publications
series Reumatismo
issn 0048-7449
2240-2683
publishDate 2019-07-01
description A 62-year-old man with a history of systemic sclerosis was admitted with diffuse alveolar hemorrhage and acute kidney injury without clinical data suggestive of glomerulonephritis. Laboratory tests showed anemia, leukocytosis with neutrophilia, thrombocytopenia, elevated serum creatinine and metabolic acidosis. Antinuclear antibodies were positive at a titer of 1/640 (speckled, 1/160; nucleolar, 1/320) while rheumatoid factor, anti Scl-70, anti-centromere, anti-neutrophil cytoplasmic antibody and anti-glomerular basement membrane antibodies were negative and serum complement levels were within normal range. During the following days, the patient developed multiple organ failure and, eventually, died. Lupus anticoagulant was revealed positive after the patient’s death, suggesting a catastrophic antiphospholipid syndrome. Clinical data and autopsy were consistent with this diagnosis.
topic Systemic sclerosis
catastrophic antiphospholipid syndrome
pulmonary renal syndrome
multiple organ failure.
url https://www.reumatismo.org/index.php/reuma/article/view/1095
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