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Case Reports

 

Diagnosis of catastrophic anti-phospholipid syndrome in a patient tested negative for conventional tests

1, 2, 3, 4, 5, 6, 7, 8, 9, 10

 

  1. Reumatologia, Dipartimento di Medicina Interna e Specialità Mediche, Sapienza Università di Roma, Italy.
  2. Reumatologia, Dipartimento di Medicina Interna e Specialità Mediche, Sapienza Università di Roma, Italy.
  3. Reumatologia, Dipartimento di Medicina Interna e Specialità Mediche, Sapienza Università di Roma, Italy.
  4. Dipartimento di Medicina Sperimentale, Sapienza Università di Roma, Italy.
  5. Dipartimento di Medicina Sperimentale, Sapienza Università di Roma, Italy.
  6. Reumatologia, Dipartimento di Medicina Interna e Specialità Mediche, Sapienza Università di Roma, Italy.
  7. Reumatologia, Dipartimento di Medicina Interna e Specialità Mediche, Sapienza Università di Roma, Italy.
  8. Reumatologia, Dipartimento di Medicina Interna e Specialità Mediche, Sapienza Università di Roma, Italy.
  9. Dipartimento di Medicina Sperimentale, Sapienza Università di Roma, Italy.
  10. Reumatologia, Dipartimento di Medicina Interna e Specialità Mediche, Sapienza Università di Roma, Italy. guido.valesini@uniroma1.it

CER10069
2017 Vol.35, N°4
PI 0678, PF 0680
Case Reports

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PMID: 28516871 [PubMed]

Received: 05/11/2016
Accepted : 20/01/2017
In Press: 08/05/2017
Published: 13/07/2017

Abstract

Catastrophic antiphospholipid syndrome (CAPS) is a severe variant of APS, characterised by clinical evidence of multiple organ involvement developing over a very short period of time, histopathological evidence of multiple small vessel occlusions and laboratory confirmation of the presence of aPL (lupus anticoagulant and/or anticardiolipin antibodies and/or anti-Beta2-glcyoprotein I antibodies). Here we report a case of a 39-year-old woman patient who developed a CAPS which was negative to the conventional aPL but positive for aPL in thin layer chromatography immunostaining and vimentin/cardiolipin antibodies by ELISA test. The patient was treated with high doses of glucocorticoids, intravenous immunoglobulins plasma exchange and immunoadsorbent apheresis with a significant improvement of the ischaemic lesions of the hands even though the necrosis of the feet progressively worsened. As a result, the patient underwent partial surgical amputation of the feet. To our knowledge, this is the first ever reported case of CAPS diagnosed by means of thin layer chromatography immunostaining and vimentin/cardiolipin antibody ELISA test.

Rheumatology Article