Sites Menu > APS - Catastrophic Antiphospholipid Syndrome


APS - Catastrophic Antiphospholipid Syndrome


Catastrophic antiphospholipid syndrome: A rare cause of disseminated microvascular thrombotic injury – a case report with pathological and molecular correlative studies

Pathology International, Volume 55 Issue 3 Page 144 - March 2005. Catastrophic antiphospholipid syndrome (CAPS) is a severe and rare variant of antiphospholipid syndrome (APS) characterized by acute multiorgan failure due to small vessel thrombi in patients with positive antiphospholipid antibodies. We report a fatal case of catastrophic antiphospholipid syndrome in a young woman with a history of polymyositis and Hodgkin lymphoma. The patient was admitted to hospital because of severe foot pain following several weeks of skin ulcerations. Doppler ultrasonography showed evidence of arterial ischemia of the both lower extremities. Despite anticoagulation, immunosuppression, plasmapheresis and antibiotic therapy, she developed cutaneous gangrene, retroperitoneal hematoma, ileus, and acute respiratory and renal failure that resulted in death. Autopsy showed multifocal vascular injury and microthrombi with associated hemorrhages and infarcts in multiple organs. The patient had normal levels of functional protein C and protein S and a normal level of plasma homocysteine. Tests for common thromophilic gene mutations including prothrombin 20210, factor V Leiden 1691, and methylene tetrahydrofolate reductase 677 were negative. To our knowledge, this is the first CAPS patient with molecular studies for genetic prothrombotic mutations. Our report showed that there was no association between the development of CAPS and inherited thromophilia.

Catastrophic antiphospholipid syndrome in a 14-year-old child.

Pediatr Nephrol. 2005 Apr;20(4):519-21. Epub 2005 Feb 17. Antiphospholipid syndrome (APS) is an autoimmune disease. Less than 1% of patients with APS present with life-threatening catastrophic APS (CAPS). We report here a case of CAPS in a young girl with cardiac, gastrointestinal and renal involvement. Although the management was complicated, the outcome was better than expected. We suggest that CAPS be included in the differential diagnosis of acute renal failure in children with multi-organ involvement and prolonged phospholipid-dependent coagulation time and promptly treated with immunomodulating agents and anticoagulants.

Laboratory studies on pathophysiology of the catastrophic antiphospholipid syndrome.

Autoimmun Rev, December 1, 2006; 6(2): 68-71. The 'catastrophic' variant of the antiphospholipid syndrome (APS) is characterized by a diffuse thrombotic microvasculopathy. In contrast to the classical APS, single venous or arterial medium-to-large blood vessel occlusions are uncommon. The mechanisms of catastrophic APS are not clearly understood. In addition, there are no studies on pathophysiologic mechanisms of catastrophic APS. The clinical manifestations of catastrophic APS probably depend on (a) the organs affected by the thrombotic events and extent of the thrombosis and (b) manifestations of the systemic inflammatory response syndrome which are presumed to be due to excessive cytokine release from affected and necrotic tissues. The evident relationship between APS and infection may enable us to explain the development of catastrophic APS using the sepsis model. This is because catastrophic APS is characterized by multiple microvascular thrombotic events, of rapid onset, and causing multiorgan failure, a picture suggestive of septic shock, in which, there is a massive, acute inflammatory response.

Catastrophic secondary antiphospholipid syndrome with peripheral nervous system involvement: a case report.

Acta Med Okayama. 2004 Apr;58(2):107-10. A 34-year-old woman was admitted to our emergency room with a high fever, abdominal pain, dyspnea and confusion. High fever and abdominal pain had first occured after a cystocele operation 5 months earlier. Later, congestive heart failure with mural thrombus formation, peripheral polyneuropathy and ischemic cerebrovascular accident were identified in clinical follow-ups, and multiple arterial and venous thromboses were seen on cranial and abdominal magnetic resonance imaging angiography. The patient's symptoms improved with anticoagulant treatment. Antiphospholipid syndrome with elevated serum anticardiolipin IgG levels was diagnosed, and ischemic peripheral polyneuropathy with axonal degeneration was determined by sural nerve biopsy. In antiphospholipid syndrome, elevated anticardiolipin antibodies appear to be the most common acquired blood protein defect causing thrombosis. Disseminated vascular thrombosis in catastrophic antiphospholipid syndrome can result in multiorgan failure with increased morbidity and mortality. It rarely occurs secondary to various infections as in the case of our patient, who suffered postoperative intraabdominal infection. It is important to note that peripheral nervous system involvement is rare in antiphospholipid syndrome.

Catastrophic Antiphospholipid Syndrome (CAPS)

Juan Javier Lichauco, M.D., Jayashree Sinha, M.D.,, and Peter Barland, M.D. February 2002

Long term outcome of catastrophic antiphospholipid syndrome survivors

Annals of the Rheumatic Diseases 2003;62:530-533. Conclusion: Sixty six per cent of patients who survive an initial catastrophic APS event remained symptom free with anticoagulation during an average follow up of 67.2 months. Twenty six per cent of the survivors developed further APS related events and the mortality rate of these patients was about 25%.

Catastrophic antiphospholipid syndrome presenting as dilated cardiomyopathy with bilateral branch retinal artery thrombosis.

Int J Clin Pract. 2000 Oct;54(8):550-1. Cardiac manifestation of antiphospholipid syndrome (APS) is mainly in the form of left-sided valvular insufficiency, intra-cardiac thrombi or coronary artery occlusion. Dilated cardiomyopathy is a rare but important cardiac manifestation of APS, and responds well to adequate anticoagulation and steroids. We describe a case in which APS presented with dilated cardiomyopathy and bilateral retinal artery thrombosis.

Catastrophic antiphospholipid syndrome in cancer.

Haematologia (Budap). 2000; 30(4):303-11. Antiphospholipid syndrome is characterized by the presence of antiphospholipid antibodies resulting in arterial and venous thromboembolism. Apart from primary cases, this syndrome is often associated with autoimmune diseases. Around 50 cases of catastrophic antiphospholipid antibody syndrome have been reported as yet. Authors describe the first case of catastrophic antiphospholipid syndrome associated with gastric cancer. Apart from presenting the clinical case, authors also discuss the possible pathomechanism of this associated disorder including the role of immunological factors, as well as antiphospholipid antibodies.

CAPS REGISTRY

Last updated: 18 September 2004

Catastrophic antiphospholipid antibody syndrome

Pediatric Nephrology, Volume 20, Number 7 / July, 2005, Pages 998-999. Antiphospholipid antibody syndrome (APS) is characterized by recurrent thrombosis with the presence of circulating antiphospholipid antibodies. A diagnosis of APS requires the presence of at least one clinical and one laboratory criteria (detection of aCL IgG or IgM antibodies or the presence of lupus anticoagulant on two or more consecutive occasions 6 weeks apart). A severe, rapidly progressive form characterized by clinical involvement of at least three different organ systems with histopathological evidence of small and large vessel occlusion is termed catastrophic antiphospholipid syndrome. Early recognition of APS is crucial since aggressive management can result in a favorable outcome.

Registry Improves Understanding of Catastrophic Antiphospholipid Syndrome

(2006) 2, 81-89 - Nature Clinical Practice Rheumatology. The most important clinical feature of CAPS to recognize is that, despite the high risk early on of this potentially devastating syndrome, the long-term prognosis of those patients who do survive the initial onset appears to be excellent.

Catastrophic Antiphospholipid Syndrome and Sepsis. A Common Link?


Imaging findings in the rare catastrophic variant of the primary antiphospholipid syndrome

European Radiology, Volume 12, Number 3 / March, 2002, Pages 545-548. We report imaging findings in a case of the rare catastrophic variant of antiphospholipid syndrome (CAPS) characterized by widespread microvascular occlusions, which may lead to multiple organ failure. We present a case of a 66-year-old woman with bone marrow necrosis, acute acalculous cholecystitis (AAC), focal liver necrosis, subtle patchy splenic infarctions, and bilateral adrenal infarction. The demonstration of multiple microvascular organ involvement (three or more) is crucial for the diagnosis of the catastrophic variant of APS. This can be performed radiologically intra-vitam. Imaging can even reveal subclinical microinfarctions, which are often only diagnosed at autopsy.





 

 

The APS Foundation of America, Inc. website and forums are both volunteer run and funded by donations to the APSFA.

Website hosted by Dreamhost. Website created and maintained by Heidi P.

DISCLAIMER: APS Foundation of America, Inc. website is not intended to replace standard doctor-patient visits, physical examination, and medical testing. Information given to members is only an opinion. All information should be confirmed with your personal doctor. Always seek the advice of a trained physician in person before seeking any new treatment regarding your medical diagnosis or condition. Any information received from APS Foundation of America, Inc. website is not intended to diagnose, treat, or cure. This site is for informational purposes only. Please note that we will be listing all donor or purchaser's names on the Donor page of our foundation site. If you do not want your name listed, please contact us to opt out. If you think you may have a medical emergency, call your doctor or 911 immediately.

APS Foundation of America, Inc. will be building a database with your email, name and address information for future mailings. Your information will be kept confidential and not sold to any third parties. You may opt out at anytime by emailing us.

APSFA ©2005-2016 | APSFA Privacy Policy | APSFA Advertising Policy | 501(c)3 Public Charity EIN #203085295