“Catastrophic” Antiphospholipid Syndrome

Abstract

We thank Drs Gómez-Puerta and colleagues for their insights into our case, a 31-year-old woman with active SLE who presented with global cognitive dysfunction, and their emphasis on the newly described entity of CAPS. We agree that the multiorgan involvement in our patient, most likely secondary to her underlying SLE, makes CAPS a diagnostic probability. During the initial work-up, assays for anti-phospholipid antibodies, in addition to the lupus anticoagulant, were negative. However, this point was not mentioned in our article because of space constraints. Inhibitors of factor XII were not detected; prolonged incubation of the patient's plasma with normal plasma showed no progressive decrease of factor XII activity over time.1Feinstein DI Inhibitors of blood coagulation.in: Hoffman R Benz Jr, EJ Shattil SJ Hematology: Basic Principles and Practice. 3rd ed. Churchill-Livingstone, Philadelphia, Pa2000: 1970Google Scholar Moreover, our patient's clinical presentation, as well as the diffusion-weighted magnetic resonance images and apparent diffusion coefficient maps, were consistent with vasogenic edema. In contrast, neurologic disorders associated with simple APS are primarily focal2Levine SR Brey RL Neurological aspects of antiphospholipid antibody syndrome.Lupus. 1996; 5: 347-353PubMed Google Scholar as a result of parenchymal ischemia or infarction (unless rostral brainstem ischemia from rostral basilar artery thrombosis causes a “top-of-the-basilar syndrome”). In patients with simple APS, diffusion-weighted magnetic resonance images and apparent diffusion coefficient maps would reveal cytotoxic edema.3Ay H Buonanno FS Rordorf G et al.Normal diffusion-weighted MRI during stroke-like deficits.Neurology. 1999; 52: 1784-1792Crossref PubMed Google Scholar Nevertheless, we did recommend repeat determinations of antiphospholipid antibody levels in 6 months. Our patient remained well under the care of her primary physician, and no further evaluation was obtained. The Editor welcomes letters and comments, particularly pertaining to recently published articles in Mayo Clinic Proceedings, as well as letters reporting original observations and research. Letters pertaining to a recently published Proceedings article should be received no later than 1 month after the article's publication. A letter should be no longer than 500 words, contain no more than 5 references and 1 table or figure, be signed by no more than 3 authors, be in double-spaced, typewritten format, and not be published or submitted elsewhere. The letter must be signed and include the correspondent's full address, telephone and fax numbers, and e-mail address (if available). It is assumed that appropriate letters will be published, at the Editor's discretion, unless the writer indicates otherwise. The Editor reserves the right to edit letters in accordance with Proceedings style and to abridge them if necessary. Letters may be submitted by surface mail to Letters to the Editor, Mayo Clinic Proceedings, Room 770 Siebens Building, Rochester, MN 55905; by fax to (507) 284-0252; or by e-mail to [email protected] (Note: Authors who submit letters by fax or e-mail must also send a copy by surface mail.) The Editor welcomes letters and comments, particularly pertaining to recently published articles in Mayo Clinic Proceedings, as well as letters reporting original observations and research. Letters pertaining to a recently published Proceedings article should be received no later than 1 month after the article's publication. A letter should be no longer than 500 words, contain no more than 5 references and 1 table or figure, be signed by no more than 3 authors, be in double-spaced, typewritten format, and not be published or submitted elsewhere. The letter must be signed and include the correspondent's full address, telephone and fax numbers, and e-mail address (if available). It is assumed that appropriate letters will be published, at the Editor's discretion, unless the writer indicates otherwise. The Editor reserves the right to edit letters in accordance with Proceedings style and to abridge them if necessary. Letters may be submitted by surface mail to Letters to the Editor, Mayo Clinic Proceedings, Room 770 Siebens Building, Rochester, MN 55905; by fax to (507) 284-0252; or by e-mail to [email protected] (Note: Authors who submit letters by fax or e-mail must also send a copy by surface mail.) “Catastrophic” Antiphospholipid SyndromeMayo Clinic ProceedingsVol. 78Issue 4PreviewTo the Editor: “Catastrophic” antiphospholipid syndrome (CAPS), an unusual but often fatal complication of the antiphospholipid syndrome (APS), is characterized by multiorgan failure due to microvascular thrombosis in 3 or more organs.1 The article by Dy and Swaroop2 described a 31-year-old woman with a history of systemic lupus erythematosus (SLE) who had an acute episode of renal failure accompanied by frank proteinuria (9 g/24 h), hypertension, and renal biopsy-proven active grade 4 glomerulonephritis. Full-Text PDF