Grand Rounds from International Lupus Centres `Catastrophic' antiphospholipid syndrome
Identifieur interne : 001787 ( Istex/Corpus ); précédent : 001786; suivant : 001788Grand Rounds from International Lupus Centres `Catastrophic' antiphospholipid syndrome
Auteurs : P J Maddison ; C. Thorpe ; J R C. Seale ; W. Ahmed ; G S WhiteleySource :
- Lupus [ 0961-2033 ] ; 2000-09.
English descriptors
- Teeft :
- Active phase, Anticardiolipin, Anticardiolipin antibodies, Anticardiolipin antibody, Antiphospholipid, Antiphospholipid antibodies, Antiphospholipid syndrome, Arthritis rheum, Asherson, Catastrophic antiphospholipid syndrome, Clinical course, Clinical features, Clinical picture, Coagulation, Deep venous thrombosis, Distress syndrome, Erythematosus, Gastrointestinal features, Gwynedd rheumatology service, High degree, High dose, Hypercoagulable disorder, Intensive care, Intensive care unit, Liver function tests, Lung compliance, Lupus, Microangiopathic haemolytic anaemia, Motor neuropathy, Multiple organs, Neurological manifestations, Platelet count, Previous history, Progressive failure, Rheumatoid factor, Rheumatol, Syndrome, Systemic, Systemic lupus erythematosus, Thrombosis, Thrombotic, Thrombotic storm, Transient improvement, Vascular endothelium, Ysbyty gwynedd.
Abstract
When `catastrophic' is applied as an adjective to the antiphospholipid syndrome, it implies a characteristic presentation due to predominantly small blood vessel thrombosis leading to rapidly progressive failure of multiple organs and a frequently fatal outcome. We present the case of a 48year-old woman who presented with the `catastrophic' antiphospholipid syndrome without previous history of coagulation disorder or connective tissue disease that illustrates the difficulties in diagnosing and managing this disorder. We also review the factors that have been reported to have a role in the development of this condition and show how this case throws light on its pathogenesis.
Url:
DOI: 10.1177/096120330000900702
Links to Exploration step
ISTEX:BAF90CF8A87D9533D95296AEFB8D09F05CAF0C38Le document en format XML
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Ahmed</name></author><author wicri:is="90%"><name sortKey="Whiteley, G S" sort="Whiteley, G S" uniqKey="Whiteley G" first="G S" last="Whiteley">G S Whiteley</name><affiliation><mods:affiliation>Gwynedd Rheumatology Service, Ysbyty Gwynedd, Bangor, UK</mods:affiliation></affiliation></author></titleStmt><publicationStmt><idno type="wicri:source">ISTEX</idno><idno type="RBID">ISTEX:BAF90CF8A87D9533D95296AEFB8D09F05CAF0C38</idno><date when="2000" year="2000">2000</date><idno type="doi">10.1177/096120330000900702</idno><idno type="url">https://api.istex.fr/ark:/67375/M70-CTNRRGTT-N/fulltext.pdf</idno><idno type="wicri:Area/Istex/Corpus">001787</idno><idno type="wicri:explorRef" wicri:stream="Istex" wicri:step="Corpus" wicri:corpus="ISTEX">001787</idno></publicationStmt><sourceDesc><biblStruct><analytic><title level="a" type="main" xml:lang="en">Grand Rounds from International Lupus Centres `Catastrophic' antiphospholipid syndrome</title><author wicri:is="90%"><name sortKey="Maddison, P J" sort="Maddison, P J" uniqKey="Maddison P" first="P J" last="Maddison">P J Maddison</name><affiliation><mods:affiliation>Gwynedd Rheumatology Service, Ysbyty Gwynedd, Bangor LL57 2PW, UK.</mods:affiliation></affiliation><affiliation><mods:affiliation>E-mail: peter@madd10.freeserve.co.uk</mods:affiliation></affiliation></author><author wicri:is="90%"><name sortKey="Thorpe, C" sort="Thorpe, C" uniqKey="Thorpe C" first="C" last="Thorpe">C. Thorpe</name></author><author wicri:is="90%"><name sortKey="Seale, J R C" sort="Seale, J R C" uniqKey="Seale J" first="J R C" last="Seale">J R C. Seale</name></author><author wicri:is="90%"><name sortKey="Ahmed, W" sort="Ahmed, W" uniqKey="Ahmed W" first="W" last="Ahmed">W. Ahmed</name></author><author wicri:is="90%"><name sortKey="Whiteley, G S" sort="Whiteley, G S" uniqKey="Whiteley G" first="G S" last="Whiteley">G S Whiteley</name><affiliation><mods:affiliation>Gwynedd Rheumatology Service, Ysbyty Gwynedd, Bangor, UK</mods:affiliation></affiliation></author></analytic><monogr></monogr><series><title level="j">Lupus</title><idno type="ISSN">0961-2033</idno><idno type="eISSN">1477-0962</idno><imprint><publisher>Sage Publications</publisher><pubPlace>Sage CA: Thousand Oaks, CA</pubPlace><date type="published" when="2000-09">2000-09</date><biblScope unit="volume">9</biblScope><biblScope unit="issue">7</biblScope><biblScope unit="page" from="484">484</biblScope><biblScope unit="page" to="488">488</biblScope></imprint><idno type="ISSN">0961-2033</idno></series></biblStruct></sourceDesc><seriesStmt><idno type="ISSN">0961-2033</idno></seriesStmt></fileDesc><profileDesc><textClass><keywords scheme="Teeft" xml:lang="en"><term>Active phase</term><term>Anticardiolipin</term><term>Anticardiolipin antibodies</term><term>Anticardiolipin antibody</term><term>Antiphospholipid</term><term>Antiphospholipid antibodies</term><term>Antiphospholipid syndrome</term><term>Arthritis rheum</term><term>Asherson</term><term>Catastrophic antiphospholipid syndrome</term><term>Clinical course</term><term>Clinical features</term><term>Clinical picture</term><term>Coagulation</term><term>Deep venous thrombosis</term><term>Distress syndrome</term><term>Erythematosus</term><term>Gastrointestinal features</term><term>Gwynedd rheumatology service</term><term>High degree</term><term>High dose</term><term>Hypercoagulable disorder</term><term>Intensive care</term><term>Intensive care unit</term><term>Liver function tests</term><term>Lung compliance</term><term>Lupus</term><term>Microangiopathic haemolytic anaemia</term><term>Motor neuropathy</term><term>Multiple organs</term><term>Neurological manifestations</term><term>Platelet count</term><term>Previous history</term><term>Progressive failure</term><term>Rheumatoid factor</term><term>Rheumatol</term><term>Syndrome</term><term>Systemic</term><term>Systemic lupus erythematosus</term><term>Thrombosis</term><term>Thrombotic</term><term>Thrombotic storm</term><term>Transient improvement</term><term>Vascular endothelium</term><term>Ysbyty gwynedd</term></keywords></textClass><langUsage><language ident="en">en</language></langUsage></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">When `catastrophic' is applied as an adjective to the antiphospholipid syndrome, it implies a characteristic presentation due to predominantly small blood vessel thrombosis leading to rapidly progressive failure of multiple organs and a frequently fatal outcome. We present the case of a 48year-old woman who presented with the `catastrophic' antiphospholipid syndrome without previous history of coagulation disorder or connective tissue disease that illustrates the difficulties in diagnosing and managing this disorder. We also review the factors that have been reported to have a role in the development of this condition and show how this case throws light on its pathogenesis.</div></front></TEI><istex><corpusName>sage</corpusName><keywords><teeft><json:string>antiphospholipid</json:string><json:string>lupus</json:string><json:string>antiphospholipid syndrome</json:string><json:string>anticardiolipin</json:string><json:string>rheumatol</json:string><json:string>asherson</json:string><json:string>erythematosus</json:string><json:string>catastrophic antiphospholipid syndrome</json:string><json:string>antiphospholipid antibodies</json:string><json:string>thrombosis</json:string><json:string>coagulation</json:string><json:string>systemic lupus erythematosus</json:string><json:string>thrombotic</json:string><json:string>syndrome</json:string><json:string>anticardiolipin antibodies</json:string><json:string>clinical picture</json:string><json:string>platelet count</json:string><json:string>intensive care unit</json:string><json:string>lung compliance</json:string><json:string>arthritis rheum</json:string><json:string>anticardiolipin antibody</json:string><json:string>rheumatoid factor</json:string><json:string>progressive failure</json:string><json:string>microangiopathic haemolytic anaemia</json:string><json:string>distress syndrome</json:string><json:string>motor neuropathy</json:string><json:string>liver function tests</json:string><json:string>high degree</json:string><json:string>transient improvement</json:string><json:string>hypercoagulable disorder</json:string><json:string>clinical course</json:string><json:string>intensive care</json:string><json:string>previous history</json:string><json:string>ysbyty gwynedd</json:string><json:string>clinical features</json:string><json:string>gastrointestinal features</json:string><json:string>active phase</json:string><json:string>vascular endothelium</json:string><json:string>thrombotic storm</json:string><json:string>high dose</json:string><json:string>multiple organs</json:string><json:string>gwynedd rheumatology service</json:string><json:string>neurological manifestations</json:string><json:string>deep venous thrombosis</json:string><json:string>systemic</json:string></teeft></keywords><author><json:item><name>P J Maddison</name><affiliations><json:string>Gwynedd Rheumatology Service, Ysbyty Gwynedd, Bangor LL57 2PW, UK.</json:string><json:string>E-mail: peter@madd10.freeserve.co.uk</json:string></affiliations></json:item><json:item><name>C Thorpe</name></json:item><json:item><name>J R C Seale</name></json:item><json:item><name>W Ahmed</name></json:item><json:item><name>G S Whiteley</name><affiliations><json:string>Gwynedd Rheumatology Service, Ysbyty Gwynedd, Bangor, UK</json:string></affiliations></json:item></author><subject><json:item><lang><json:string>eng</json:string></lang><value>catastrophic antiphospholipid syndrome (CAPS)</value></json:item><json:item><lang><json:string>eng</json:string></lang><value>antiphospholipid antibodies</value></json:item><json:item><lang><json:string>eng</json:string></lang><value>acute respiratory distress syndrome (ARDS)</value></json:item><json:item><lang><json:string>eng</json:string></lang><value>critical illness neuropathy</value></json:item></subject><articleId><json:string>10.1177_096120330000900702</json:string></articleId><arkIstex>ark:/67375/M70-CTNRRGTT-N</arkIstex><language><json:string>eng</json:string></language><originalGenre><json:string>research-article</json:string></originalGenre><abstract>When `catastrophic' is applied as an adjective to the antiphospholipid syndrome, it implies a characteristic presentation due to predominantly small blood vessel thrombosis leading to rapidly progressive failure of multiple organs and a frequently fatal outcome. We present the case of a 48year-old woman who presented with the `catastrophic' antiphospholipid syndrome without previous history of coagulation disorder or connective tissue disease that illustrates the difficulties in diagnosing and managing this disorder. 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We present the case of a 48year-old woman who presented with the `catastrophic' antiphospholipid syndrome without previous history of coagulation disorder or connective tissue disease that illustrates the difficulties in diagnosing and managing this disorder. We also review the factors that have been reported to have a role in the development of this condition and show how this case throws light on its pathogenesis.</p></abstract><textClass><keywords scheme="keyword"><list><head>keywords</head><item><term>catastrophic antiphospholipid syndrome (CAPS)</term></item><item><term>antiphospholipid antibodies</term></item><item><term>acute respiratory distress syndrome (ARDS)</term></item><item><term>critical illness neuropathy</term></item></list></keywords></textClass></profileDesc><revisionDesc><change when="2000-09">Published</change></revisionDesc></teiHeader></istex:fulltextTEI><json:item><extension>txt</extension><original>false</original><mimetype>text/plain</mimetype><uri>https://api.istex.fr/ark:/67375/M70-CTNRRGTT-N/fulltext.txt</uri></json:item></fulltext><metadata><istex:metadataXml wicri:clean="corpus sage not found" wicri:toSee="no header"><istex:xmlDeclaration>version="1.0" encoding="UTF-8"</istex:xmlDeclaration><istex:docType PUBLIC="-//NLM//DTD Journal Publishing DTD v2.3 20070202//EN" URI="journalpublishing.dtd" name="istex:docType"></istex:docType><istex:document><article article-type="research-article" dtd-version="2.3" xml:lang="EN"><front><journal-meta><journal-id journal-id-type="hwp">splup</journal-id><journal-id journal-id-type="publisher-id">LUP</journal-id><journal-title>Lupus</journal-title><issn pub-type="ppub">0961-2033</issn><publisher>
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<contrib-group><contrib contrib-type="author" xlink:type="simple"><name name-style="western"><surname>Maddison</surname><given-names>P J</given-names></name><aff>Gwynedd Rheumatology Service, Ysbyty Gwynedd, Bangor LL57 2PW, UK. <email xlink:type="simple">peter@madd10.freeserve.co.uk</email>
</aff></contrib></contrib-group>
<contrib-group><contrib contrib-type="author" xlink:type="simple"><name name-style="western"><surname>Thorpe</surname><given-names>C</given-names></name></contrib></contrib-group>
<contrib-group><contrib contrib-type="author" xlink:type="simple"><name name-style="western"><surname>Seale</surname><given-names>J R C</given-names></name></contrib></contrib-group>
<contrib-group><contrib contrib-type="author" xlink:type="simple"><name name-style="western"><surname>Ahmed</surname><given-names>W</given-names></name></contrib></contrib-group>
<contrib-group><contrib contrib-type="author" xlink:type="simple"><name name-style="western"><surname>Whiteley</surname><given-names>G S</given-names></name><aff>Gwynedd Rheumatology Service, Ysbyty Gwynedd, Bangor, UK</aff></contrib></contrib-group>
<pub-date pub-type="ppub"><month>09</month><year>2000</year></pub-date><volume>9</volume><issue>7</issue><fpage>484</fpage><lpage>488</lpage><abstract>
<p>When `catastrophic' is applied as an adjective to the antiphospholipid syndrome, it implies a characteristic presentation due to predominantly small blood vessel thrombosis leading to rapidly progressive failure of multiple organs and a frequently fatal outcome. We present the case of a 48year-old woman who presented with the `catastrophic' antiphospholipid syndrome without previous history of coagulation disorder or connective tissue disease that illustrates the difficulties in diagnosing and managing this disorder. We also review the factors that have been reported to have a role in the development of this condition and show how this case throws light on its pathogenesis.</p>
</abstract><kwd-group>
<kwd>catastrophic antiphospholipid syndrome (CAPS)</kwd>
<kwd>antiphospholipid antibodies</kwd>
<kwd>acute respiratory distress syndrome (ARDS)</kwd>
<kwd>critical illness neuropathy</kwd>
</kwd-group><custom-meta-wrap><custom-meta xlink:type="simple"><meta-name>sagemeta-type</meta-name><meta-value>Journal Article</meta-value></custom-meta><custom-meta xlink:type="simple"><meta-name>search-text</meta-name><meta-value> Grand Rounds from International Lupus Centres 'Catastrophic' antiphospholipid syndrome PJ Maddison1*, C Thorpe1, JRC Seale1, W Ahmed1 and GS Whiteley1 1 Gwynedd Rheumatology Service, Ysbyty Gwynedd, Bangor, UK When 'catastrophic' is applied as an adjective to the antiphospholipid syndrome, it implies a characteristic presentation due to predominantly small blood vessel thrombosis leading to rapidly progressive failure of multiple organs and a frequently fatal outcome. We present the case of a 48- year-old woman who presented with the 'catastrophic' antiphospholipid syndrome without previous history of coagulation disorder or connective tissue disease that illustrates the dif®culties in diagnosing and managing this disorder. We also review the factors that have been reported to have a role in the development of this condition and show how this case throws light on its pathogenesis. Lupus (2000) 9, 484±488. Keywords: catastrophic antiphospholipid syndrome (CAPS); antiphospholipid antibodies; acute respiratory distress syndrome (ARDS); critical illness neuropathy Introduction The antiphospholipid syndrome (APS), or 'Hughes syndrome', describes patients with a hypercoagulable disorder resulting in increased foetal loss and recurrent arterial or venous thromboses often asso- ciated with cytopenia, particularly thrombocytopenia, in whom antiphospholipid antibodies (APL) can be demonstrated.1 The clinical consequences of throm- bosis, which is the main complication of APS, depends on the site and extent of the process. In most cases it is the larger vessels that are involved,2 particularly thrombosis of the intracranial arteries leading to cerebral ischaemia or the deep veins of the lower extremities sometimes leading to pulmonary embolism. These events usually occur in isolation or, if recurrent, over months or years. Much less commonly, there is diffuse thrombotic occlusion of predominantly small vessels leading to damage and dysfunction of multiple organs, concurrently or sequentially over days or weeks.3 The term 'catastrophic' antiphospholipid syndrome (CAPS) has been coined for this serious and often fatal complication. Patients frequently present to the intensive care unit with the challenging differential diagnosis of multisystem failure. The experience of others reported in the literature3 emphasizes that the diagnosis of CAPS requires a high degree of clinical awareness but, once the condition is suspected clinically, the diagnosis is generally con®rmed by the demonstration of antiphospholipid antibodies in the serum andaor lupus anticoagulant activity in plasma. We present a 48-year-old Caucasian female who was admitted to our intensive care unit with acute respiratory distress syndrome (ARDS), evidence of a coagulopathy and severe neurological complications. Although the clinical picture pointed to the diagnosis of CAPS, APL were not detected in the acute phase although increasing titres of anticardiolipin antibody were identi®ed after several weeks during her recovery. Not only does this case highlight the dif®culties of diagnosis and management of such patients but also adds to the circumstantial evidence that antiphospholipid antibodies have a direct pathogenic role in the thrombosis. Case history Our patient, a 48-year-old Caucasian female, pre- sented in June 1998 with a four-month history of *Correspondence: PJ Maddison, Gwynedd Rheumatology Service, Ysbyty Gwynedd, Bangor LL57 2PW, UK. E-mail: peter@madd10.freeserve.co.uk Received 13 March 2000; accepted 12 June 2000 Lupus (2000) 9, 484±488 ß 2000 Macmillan Publishers Ltd All rights reserved 0961-2033/00 $15.00 www.nature.com/lup progressively severe lower abdominal cramping pain and diarrhoea, sometimes mixed with fresh blood. Her previous medical history included a severe depressive illness in 1967, treated with ECT followed by recurrent episodes of anxiety and depression. In 1995 she had been thoroughly investigated for abdominal discomfort, distension and ¯atulence. Investigations including both upper and lower gastrointestinal endoscopy were normal and the diagnosis of irritable bowel syndrome was made. She also had a long history, of at least 15 years' duration, of generalized musculoskeletal pain affect- ing her neck, shoulder girdle, lower back, hips and knees together with severe fatigue. These symptoms were investigated in 1997. The only signi®cant ®nding was of tender points and the clinical picture was consistent with ®bromyalgia. Laboratory inves- tigations were normal, including an ESR of 10 mmah, platelets of 224 Kaml and normal liver function tests, except that rheumatoid factor was detected (RA latex test) in high titre (800 iual). The ANA was negative (Hep2 cells). In April 1998 she was admitted with pleuritic, left-sided chest pain. There were no abnormalities on clinical examination. Investigations showed a slightly raised ESR of 27 mmah, haemo- globin 10.5 gadl, platelets 176 Kaml and abnormal transaminases (AST 57 IUal [normal: 10 ± 32]; ALT 104 IUal [normal: 10 ± 32]). A VQ scan showed no evidence of perfusion mismatch and an ultrasound scan demonstrated that the deep leg veins were patent. The symptoms rapidly resolved without treatment and she was discharged. She had a normal obstetric history with three normal pregnancies between 1971 and 1976. There were no miscarriages. However, aged 20 y, she had had a left deep venous thrombosis while on a high oestrogen contraceptive pill. On this occasion, the working diagnosis was ulcerative colitis. Colonoscopy demonstrated granular and friable mucosa with discrete ulcers involving the rectum and descending colon. Biopsy of rectal mucosa showed mild, nonspeci®c in¯ammation in the lamina propria with no evidence of crypt abscesses or granulomata. She was treated with corticosteroid enemas, mesalazine and, subsequently, prednisolone 60 mgad to which azathioprine was added but she failed to respond. Over the next three months, the clinical picture was dominated by severe abdominal pain, bloody diarrhoea and progressive loss of weight. Endoscopy con®rmed progression of ulceration to involve the whole of the large bowel. Repeat biopsies showed the same nonspeci®c histological picture. During this time, the ESR rose to 70 mmah, and the AST and ALT to 258 IUaml and 717 IUaml respectively. A liver biopsy showed no abnormality. In December 1998 she had a subtotal colectomy, end ileostomy and closure of the rectal stump because of her deteriorating condition and failure to respond to medical treatment. Prednisolone was tailed off and subsequently discontinued. Histology showed multi- ple ulcers throughout the length of the colon. At the base of the largest ulcers there were small muscular arteries showing almost total obliteration of their lumen by organizing thrombus. Post-operatively, she developed severe lower abdominal pain and bleeding from the surgical wound. Platelets had fallen to 72 Kaml. Prothrombin time (PT) (21 s [control: 13 s]) and partial thromboplastin time (PTT) (42 s [control: 31 s]) were prolonged. D-dimer levels were greater than 0.5 mgaml but less than 1.0 mgaml, not typical of disseminated intravascular coagulation (DIC). The blood ®lm showed no evidence of microangiopathic haemolytic anaemia. After one week she had surgical removal of a large pelvic haematoma. Post-opera- tively she went into respiratory failure and was transferred to the Intensive Care Unit. She demonstrated the picture of severe ARDS with extensive shadowing of both lung ®elds on X-ray and very poor oxygenation and lung compliance, requiring long-term ventilation. There was persistent thrombo- cytopenia, ranging from 28 ± 70 Kaml with bleeding from wounds, puncture sites and vagina. There was no evidence of DIC or microangiopathic haemolytic anaemia. She developed a motor neuropathy with complete ¯accid paralysis of her limbs, but with sparing of sensation, associated with changes of severe axonal motor neuropathy on nerve conduction tests. The liver function tests were persistently abnormal with raised alkaline phosphatase (4409 IUaml) and gGT (1400 IUal) as well as the high levels of transaminases. The clinical picture was also complicated by infection including MRSA septicaemia and pneumonia. During this episode of infection she required inotropic support of her heart but, otherwise, there was no evidence of signi®cant myocardial or renal dysfunction and she was not hypertensive. The diagnosis of CAPS was suspected but APL were not detected, either by ELISA for anticardiolipin antibodies or a lupus anticoagulant (LAC) using the dilute Russell viper venom time. Empirically, she was treated with three 'pulses' of i.v. methylprednisolone 1 g on successive days. This was followed by a transient improvement in lung compliance and oxygenation (Figure 1). Two weeks later the 'pulses' of methylprednisolone were repeated followed by hydrocorticone, initially 200 mg daily then in a slowly reducing dose later converting to prednisolone. Gradual but steady improvement in her respiratory function occurred. This was now accompanied by Catastrophic antiphospholipid syndrome PJ Maddison et al 485 Lupus resolution of the thrombocytopenia. During the recovery phase anticardiolipin antibodies were de- tected, initially a low titre of IgM antibodies and subsequently a moderate titre of IgG and IgM antibodies. Up to this point she had not been fully anticoagulated. Subcutaneous heparin was adminis- tered during the ®rst week of intensive care but discontinued because of bleeding. Once APL were detected, anticoagulation was achieved with warfarin, aiming for an INR of between 2 ± 3. The convales- cence phase was interrupted by the development of symmetrical polyarthritis predominantly affecting her small joints which did not respond to nonsteroidal anti-in¯ammatory drugs. The arthritis resolved after the addition of hydroxychloroquine 400 mgad. At follow-up in January 2000 she was well on prednisolone 5 mg daily, hydroxychloroquine 400 mg daily and warfarin. She was now functionally independent and her strength was returning. Platelet count and liver function tests were normal. Discussion This case illustrates the diagnostic and management challenge posed by CAPS. It also highlights the possible role of APL in its pathogenesis. The term 'catastrophic' has not found universal approval,4 but it does draw attention to a distinctive presentation characterized by rapidly progressive multi-organ fail- ure due to predominantly small vessel coagulopathy that is unusual in patients with APL.5 It also highlights the frequently poor prognosis. Diagnosis requires a high index of suspicion and CAPS often has to be distinguished from multisystem disorders that might be associated with coagulopathy including DIC, thrombotic thrombocytopaenic pur- pura and systemic rheumatic diseases associated with disseminated vaculopathy such as SLE and primary vasculitis. Our case did not demonstrate the typical laboratory features of DIC although they have been found in up to 28% of patients with CAPS.3 A slight increase in D-dimer was noted early on but not in the range associated with severe DIC and ®brinogen levels were, in fact, increased. Similarly, there were no features of microangiopathy such as the presence of schistocytes on the peripheral blood smear. Apart from a positive test for rheumatoid factor, there was no other indication that our patient had an underlying systemic rheumatic disease. Furthermore, apart from the history of deep venous thrombosis 30 y before while she was taking the oral contraceptive pill, there had been no prior features to suggest the presence of APS. This is not uncommon in patients presenting with CAPS. In the review of Asherson et al,3 22 of 50 Figure 1 Platelet count and lung compliance (mlacmH2O) during the patient's clinical course in intensive care. There was a transient improvement in compliance after the ®rst administration of IV methylprednisolone (MP), with a progressive recovery of both compliance and FiO2 (fraction of inspired oxygen, required to keep Pa O2 greater than 10 kPa) after the second administration of methylprednisolone 2 weeks later. During recovery, the platelet count returned to normal and anticardiolipin antibodies were detected for the ®rst time. Catastrophic antiphospholipid syndrome PJ Maddison et al 486 Lupus cases had no previous history of APS features and less than 50% had an associated autoimmune rheumatic disease such as SLE. The clinical features described in our case were typical of CAPS. Asherson et al reported3 that the most common manifestations seen in patients with CAPS were a renal microangiopathy usually accom- panied by severe hypertension and pulmonary in- volvement, often a full blown picture of ARDS. While renal impairment was not a major feature in our patient, and she was never hypertensive, her clinical course was dominated initially by gastrointestinal manifestations and subsequently by typical and persistent ARDS requiring respiratory support for three months. Gastrointestinal features, either present clinically or demonstrated at postmortem examina- tion, are not uncommon in CAPS, present in about 38%.3,6,7 In some cases there is thrombotic occlusion of major mesenteric vessels leading to bowel infarc- tion. By contrast, the clinical and histological features demonstrated by this case suggest that predominantly small vessel thrombosis accounted for the diffuse colonic involvement. A similar picture has been reported previously in which colonic ulceration associated with microvascular thrombosis was de- tected at postmortem examination.7 Elevation of liver enzymes, a marked feature in our patient, is also well recognized in CAPS, occurring in at least a third of patients, and associated with hepatic microthrombi.3,8 The diffuse and predominantly motor neuropathy, which resulted in complete quadriplegic paralysis in our case, showed the clinical and electrophysiological features of so-called 'critical illness neuropathy'.9 The mechanism of axonal injury in this condition is unknown. However, there is speculation that injury to the microcirculation of distal nerves causes ischaemia and axonal degeneration. It is likely, therefore, that this was also a manifestation of CAPS in this patient although there were other important associated factors such as severe sepsis. A wide variety of central and peripheral neurological features have been described in patients with APL10 ± 12 but, to our knowledge, this is the ®rst description of 'critical illness neuropathy' in the setting of CAPS. We used a combination of an ELISA for anticar- diolipin and a functional assay for the presence of LAC which is generally accepted as a con®rmatory screen for APS.13 Since the demonstration that the APL that characterize APS react with phospholipid binding proteins, especially b2 glycoprotein 1 (b2GP1),14 ± 17 assays for anti-b2GP1 have been developed which have a high degree of speci®city for APS.18,19 There is a small proportion of patients with APL who are negative in tests for cardiolipin and LAC but anti-b2GP1 positive. The failure to detect anticardiolipin antibody and LAC during the active phase of disease in this patient, with the occurrence of APL during the recovery phase, has occasionally been described in other patients presenting with CAPS.20,21 The phenomenon can be explained by antibody consumption and has been documented for other potentially pathogenic autoantibodies in, for example, SLE.22,23 The absence of APL contributed to the clinical conundrum in this case. It is possible that we might have detected anti-b2GP1 had the assay been available. However, we doubt this since a fall in titre of anti-b2GP1 commonly occurs at the time of a thrombotic episode.24 This was observed more commonly than concurrent falls in anticardiolipin titre. The pathogenic potential of APL has been demon- strated unquestionably in animal models and impli- cated in both pregnancy loss and other features of APS in these animals.25 The demonstration of falling antibody titres during the active phase of the thrombotic process is good circumstantial evidence of a direct role in pathogenesis in humans. However, why patients presenting with CAPS develop diffuse small vessel thrombosis rather than the usual picture of isolated large vessel occlusion is obscure. There are recent reviews of the pathogenesis of CAPS which emphasise that multiple factors probably interact.26,27 Central to the process, however, is the increased susceptibility to thrombosis due to the presence of APL which is probably genetically determined.28 It is possible that the particular pro®le of APL in an individual in¯uences the clinical pattern of disease. There is evidence to suggest that APL and, particu- larly anti-b2GP1, bind to vascular endothelium and not only interfere with the kinetics of phospholipid- dependent coagulation but also activate endothelial cells.29 Frequently, CAPS is associated with trigger factors which potentiate these effects on vascular endothelium and the coagulation process.3,30 These include surgery, even minor investigational proce- dures, and infection, both of which occurred in our patient and which are potent inducers of tissue damage and increased cytokine production. Further- more, products of infection, by acting as super- antigens or by molecular mimicry can further stimulate the autoimmune response to phospho- lipids.27 Golden and Belmont31 have emphasized the potential importance of leukothrombosis arising from intravascular activation of complement, neutrophils and endothelium, which occurs in endotoxic shock and may play an important role in CAPS and its manifestations such as ARDS. Also, the presence of thrombus itself may potentiate the coagulation process in patients with a hypercoagulable disorder. As described by Kitchens,21 an increase in coagulation Catastrophic antiphospholipid syndrome PJ Maddison et al 487 Lupus products such as thrombin ± antithrombin complexes occurs when there are fresh clots enhancing further thrombosis and a decline in the levels of naturally occurring inhibitors of thrombosis such as ATIII, protein C and protein S. This process, termed 'thrombotic storm' by Kitchens, may have operated in our patient. It can be minimized by appropriate anticoagulation. CAPS is a serious complication with a mortality in reported cases of approximately 50%.3 The optimal management of CAPS is not known. Most cases have been treated by corticosteroids, anticoagulation, plasmapheresis, high dose intravenous immunoglobu- lin and cyclophosphamide, usually in some form of combination of these. A few patients have received anti®brinolytic and other therapies.32 In the review by Asherson et al,3 the best outcome (70% survival) was seen in patients treated with a combination of corticosteroids, anticoagulation, plasmapheresis anda or immunoglobulin. The addition of cyclophospha- mide was associated with a lower rate of survival, although these patients may have had more severe disease. The survival of our patient was predomi- nantly due to high quality intensive care. Initially, the gastrointestinal features failed to respond to high dose daily prednisolone. However, her subsequent recovery dated from being given megadose i.v. corticosteroids. Experience from a larger number of cases suggests that steroids alone are insuf®cient and that methods designed to achieve prompt reduction in APL titres, such as plasmapheresis, are required. However, in this unusual case, serum antibodies were undetectable and these techniques were not employed. 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</ref-list></back></article></istex:document></istex:metadataXml><mods version="3.6"><titleInfo lang="en"><title>Grand Rounds from International Lupus Centres `Catastrophic' antiphospholipid syndrome</title></titleInfo><titleInfo type="alternative" lang="en" contentType="CDATA"><title>Grand Rounds from International Lupus Centres `Catastrophic' antiphospholipid syndrome</title></titleInfo><name type="personal"><namePart type="given">P J</namePart><namePart type="family">Maddison</namePart><affiliation>Gwynedd Rheumatology Service, Ysbyty Gwynedd, Bangor LL57 2PW, UK.</affiliation><affiliation>E-mail: peter@madd10.freeserve.co.uk</affiliation></name><name type="personal"><namePart type="given">C</namePart><namePart type="family">Thorpe</namePart></name><name type="personal"><namePart type="given">J R C</namePart><namePart type="family">Seale</namePart></name><name type="personal"><namePart type="given">W</namePart><namePart type="family">Ahmed</namePart></name><name type="personal"><namePart type="given">G S</namePart><namePart type="family">Whiteley</namePart><affiliation>Gwynedd Rheumatology Service, Ysbyty Gwynedd, Bangor, UK</affiliation></name><typeOfResource>text</typeOfResource><genre type="research-article" displayLabel="research-article" authority="ISTEX" authorityURI="https://content-type.data.istex.fr" valueURI="https://content-type.data.istex.fr/ark:/67375/XTP-1JC4F85T-7">research-article</genre><originInfo><publisher>Sage Publications</publisher><place><placeTerm type="text">Sage CA: Thousand Oaks, CA</placeTerm></place><dateIssued encoding="w3cdtf">2000-09</dateIssued><copyrightDate encoding="w3cdtf">2000</copyrightDate></originInfo><language><languageTerm type="code" authority="iso639-2b">eng</languageTerm><languageTerm type="code" authority="rfc3066">en</languageTerm></language><abstract lang="en">When `catastrophic' is applied as an adjective to the antiphospholipid syndrome, it implies a characteristic presentation due to predominantly small blood vessel thrombosis leading to rapidly progressive failure of multiple organs and a frequently fatal outcome. We present the case of a 48year-old woman who presented with the `catastrophic' antiphospholipid syndrome without previous history of coagulation disorder or connective tissue disease that illustrates the difficulties in diagnosing and managing this disorder. We also review the factors that have been reported to have a role in the development of this condition and show how this case throws light on its pathogenesis.</abstract><subject><genre>keywords</genre><topic>catastrophic antiphospholipid syndrome (CAPS)</topic><topic>antiphospholipid antibodies</topic><topic>acute respiratory distress syndrome (ARDS)</topic><topic>critical illness neuropathy</topic></subject><relatedItem type="host"><titleInfo><title>Lupus</title></titleInfo><genre type="journal" authority="ISTEX" authorityURI="https://publication-type.data.istex.fr" valueURI="https://publication-type.data.istex.fr/ark:/67375/JMC-0GLKJH51-B">journal</genre><identifier type="ISSN">0961-2033</identifier><identifier type="eISSN">1477-0962</identifier><identifier type="PublisherID">LUP</identifier><identifier type="PublisherID-hwp">splup</identifier><part><date>2000</date><detail type="volume"><caption>vol.</caption><number>9</number></detail><detail type="issue"><caption>no.</caption><number>7</number></detail><extent unit="pages"><start>484</start><end>488</end></extent></part></relatedItem><identifier type="istex">BAF90CF8A87D9533D95296AEFB8D09F05CAF0C38</identifier><identifier type="ark">ark:/67375/M70-CTNRRGTT-N</identifier><identifier type="DOI">10.1177/096120330000900702</identifier><identifier type="ArticleID">10.1177_096120330000900702</identifier><recordInfo><recordContentSource authority="ISTEX" authorityURI="https://loaded-corpus.data.istex.fr" valueURI="https://loaded-corpus.data.istex.fr/ark:/67375/XBH-0J1N7DQT-B">sage</recordContentSource></recordInfo></mods><json:item><extension>json</extension><original>false</original><mimetype>application/json</mimetype><uri>https://api.istex.fr/ark:/67375/M70-CTNRRGTT-N/record.json</uri></json:item></metadata><serie></serie></istex></record>Pour manipuler ce document sous Unix (Dilib)
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