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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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.
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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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