Catastrophic antiphospholipid syndrome as a complication of systemic sclerosis

  • D.J. Manzella | dmanzella@fmed.uba.ar Department of Medicine, Hospital de Clínicas José de San Martín, University of Buenos Aires, Buenos Aires, Argentina.
  • L. Vicente Department of Medicine, Hospital de Clínicas José de San Martín, University of Buenos Aires, Buenos Aires, Argentina.
  • A.A. Pérez de la Hoz Department of Medicine, Hospital de Clínicas José de San Martín, University of Buenos Aires, Buenos Aires, Argentina.
  • R.J. Zamora Department of Medicine, Hospital de Clínicas José de San Martín, University of Buenos Aires, Buenos Aires, Argentina.
  • G. De Rosa Department of Pathology, Hospital de Clínicas José de San Martín, University of Buenos Aires, Buenos Aires, Argentina.
  • A.A. Pisarevsky Department of Medicine, Hospital de Clínicas José de San Martín, University of Buenos Aires, Buenos Aires, Argentina.

Abstract

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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Published
2019-07-09
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Section
Case Reports
Keywords:
Systemic sclerosis, catastrophic antiphospholipid syndrome, pulmonary renal syndrome, multiple organ failure.
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How to Cite
Manzella, D., Vicente, L., Pérez de la Hoz, A., Zamora, R., De Rosa, G., & Pisarevsky, A. (2019). Catastrophic antiphospholipid syndrome as a complication of systemic sclerosis. Reumatismo, 71(2), 92-98. https://doi.org/10.4081/reumatismo.2019.1095