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

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.

• Authors: Fabrizio Conti, Roberta Priori, Cristiano Alessandri, Roberta Misasi, Antonella Capozzi, Monica Pendolino, Simona Truglia, Silvia Frisenda, Maurizio Sorice, Guido Valesini
• Journal: Clinical and experimental rheumatology (Clin Exp Rheumatol) 2017 Jul-Aug Vol. 35 Issue 4 Pg. 678-680 ISSN: 0392-856X [Print] Italy
• PMID: 28516871 (Publication Type: Case Reports, Journal Article)

Chemical References

• Antibodies, Anticardiolipin
• Antibodies, Antiphospholipid
• Glucocorticoids
• Immunoglobulins, Intravenous
• Immunologic Factors
• Vimentin

Topics

• Adult
• Amputation, Surgical
• Antibodies, Anticardiolipin (immunology)
• Antibodies, Antiphospholipid (immunology)
• Antiphospholipid Syndrome (complications, diagnosis, immunology, therapy)
• Arterial Occlusive Diseases (diagnostic imaging, etiology, immunology)
• Blood Component Removal
• Chromatography, Thin Layer
• Computed Tomography Angiography
• Enzyme-Linked Immunosorbent Assay
• Female
• Glucocorticoids (therapeutic use)
• Humans
• Immunoglobulins, Intravenous (therapeutic use)
• Immunologic Factors (therapeutic use)
• Popliteal Artery (diagnostic imaging)
• Tibial Arteries (diagnostic imaging)
• Vimentin (immunology)