Diagnosis Of Catastrophic Antiphospholipid Syndrome Research Articles

Antiphospholipid Patients Admitted in the Intensive Care Unit: What Must The Rheumatologist Know?

Antiphospholipid syndrome (APS) is a rare systemic autoimmune disorder that can escalate into a 'thrombotic storm' called the catastrophic antiphospholipid syndrome (CAPS), frequently requiring ICU admission for multiple organ failure. This review aims to offer insight and recent evidence on critically-ill APS patients. The CAPS classification criteria define this condition as the involvement of at least three organs/systems/tissues within less than a week, caused by small vessel thrombosis, in patients with elevated antiphospholipid antibodies levels. These criteria do not encompass the full spectrum of critically-ill thrombotic APS patients and they need to be cautiously used for the bedside diagnosis of CAPS. Thrombocytopenia is the laboratory hallmark of CAPS, sometimes dropping below 20G/L, but a complete thrombotic microangiopathy pattern is infrequent. Anticoagulation is the pivotal treatment for APS and CAPS, associated with improved outcome. Triple therapy - the combination of anticoagulation, high-dose corticosteroids, and either plasma exchange or intravenous immunoglobulins - remains the standard treatment for CAPS patients. Eculizumab, an anti-C5 monoclonal antibody, may be useful in refractory patients. Despite significant progress, CAPS mortality rate remains high. Its diagnosis and management are complex, requiring a close multidisciplinary cross talk between APS specialists and intensivists.

Diagnosis and Management of Catastrophic Antiphospholipid Syndrome and the Potential Impact of the 2023 ACR/EULAR Antiphospholipid Syndrome Classification Criteria.

Catastrophic antiphospholipid syndrome (CAPS) is a rare and life-threatening condition characterized by the persistence of antiphospholipid antibodies and occurrence of multiple vascular occlusive events. CAPS currently remains a diagnostic challenge and requires urgent treatment. The diagnosis of CAPS is made difficult by classification criteria used as diagnostic criteria in clinical practice, knowledge derived from retrospective data and case reports, confounding clinical and biological features, and its rapid onset and mortality. The absence of prospective studies of CAPS limits the strength of evidence for guideline treatment protocols. This comprehensive review summarizes the current understanding of the disease, and discusses how the 2023 ACR/EULAR Antiphospholipid Syndrome Classification Criteria impact the definition and therapeutic management of CAPS, which is considered the most severe form of APS. The correct integration of 2023 ACR/EULAR APS classification criteria is poised to facilitate CAPS diagnosis, particularly in critical situations, offering a promising avenue for improved outcomes.

Catastrophic antiphospholipid syndrome after pregnancy complicated by hemolysis, elevated liver enzymes and low platelets syndrome.

Antiphospholipid syndrome (APLS) is rarely complicated by catastrophic antiphospholipid syndrome (CAPS). Peripartum CAPS is rarer still and can masquerade as other obstetric disorders. A high degree of suspicion is critical for early diagnosis and specific management given the significant morbidity and mortality associated with this disorder. We report a case of a 27-year-old at 22 week's gestation with a history of APLS found to have severe hemolysis, elevated liver enzymes and low platelets (HELLP) syndrome, resulting in termination of pregnancy. Further workup revealed the diagnosis of CAPS followed by prompt treatment with triple therapy leading to clinical improvement. CAPS should be considered within the differential in an obstetric patient with a history of APLS who has evidence of multiorgan involvement with macro- or microvascular thrombosis. Although this may mimic alternative disorders, prompt diagnosis is imperative for appropriate therapy and reduction in maternal morbidity and mortality.

Catastrophic antiphospholipid syndrome presented as ruptured papillary muscle during puerperium in a patient with systemic lupus erythematosus

Catastrophic antiphospholipid syndrome (CAPS) is a rare and serious phenomenon that requires prompt recognition and treatment. The authors present the case of a puerperal woman with systemic lupus erythematosus (SLE) admitted to the emergency room with headache, blurred vision, thoracic pain, and purpuric lesions on both ears. Echocardiogram revealed global decrease in left ventricular function while cardiac and inflammatory markers were elevated. Three days after admission she developed cardiogenic shock due to rupture of mitral papillary muscle which required emergent cardiac surgery, with replacement of the mitral valve; treatment with anticoagulation, high-dose glucocorticoids, and intravenous immunoglobulins were initiated. Cardiac and brain MRI revealed signs of ischemic lesions in both organs. Histopathology analysis of the placenta and papillary muscle showed signs of ischemia secondary to microvascular thrombosis. Based on the clinical demonstration of thrombosis in three organs, and the presence of lupus anticoagulant antibodies, a diagnosis of probable CAPS was established. This case highlights the importance of a high level of suspicion of CAPS, particularly in patients with risk factors, and the value of immediate adequate treatment. Moreover, the rupture of a papillary muscle with histologically consistent signs of antiphospholipid syndrome expands the spectrum of involvement of this disease and should be considered as a rare but life-threatening possibility in patients with myocardial injury.

Bilateral adrenal hemorrhage in APLS (anti-phospholipid syndrome) patient on Novel Anticoagulant: a case report

Background: Antiphospholipid syndrome is a prothrombotic state characterized by the existence of persistent antiphospholipid antibodies. The key sequence of events is focal microthrombosis and post-infarction hemorrhage in the adrenal glands. Use of anticoagulant therapy is often used as a prevention and treatment among patients. Case presentation: We describe a case of 40 year old female who visited Emergency Room with complain of severe right upper quadrant pain for two days associated with nausea and vomiting. Initial laboratory investigations of blood, liver, and renal functions were normal. CT scan of abdomen revealed diffused enlargement of the right adrenal gland and showed relatively less enhancement indicating hemorrhage at the right side which later developed bilateral during admission. A day after admission, platelet count was 60,000 accompanied with worsened abdominal pain lead to diagnosis of catastrophic Anti-phospholipid syndrome (APLS). Conclusion: APS may lead to a variety of clinical manifestations due to venous and/or arterial thrombosis, so prescribing novel anticoagulants for patients with the APS is selective, and multiple factors are needed to be considered for a successful treatment.

Management of Catastrophic Antiphosphopholipid Syndrome with Eculizumab

Catastrophic antiphospholipid syndrome (CAPS) is a rare, often fatal phenomenon. Patients present with a wide range of symptomatology including thrombotic microangiopathy, cytopenias and end organ damage. The mortality rate of CAPS is as high as 33% in spite of the use of combination therapies including steroids, anticoagulation, plasma exchange (PEX) and intravenous immunoglobulin (Cervera CA 2009; Espinosa G 2011; Bucciarelli S 2006). CAPS is believed to be a disorder of complement-mediated inflammation which results in tissue injury. The proposed mechanism of the thrombotic microangiopathyisthe interaction of the coagulation cascade and complement (Mehdi AA 2010). Multiple murine-based studies demonstrate the contribution of C5a to antiphospholipid antibody-mediated intravascular thrombosis (Fischetti F 2005; Pierangeli SS 2005; Giannakopoulos B 2013). Eculizumab is a humanized monoclonal antibody that binds to C5 and inhibits its cleavage to C5a and C5b. It is FDA approved for the treatment of the complement mediated disorders paroxysmal nocturnal hemoglobinuria and atypical hemolytic uremic syndrome.We present 3 cases of CAPS treated with eculizumab at our institution. Their individual diagnostic criteria and background characteristics are outlined in Table 1.Case 1: 41-year-old male with history of antiphospholipid syndrome on chronic anticoagulation presented with fevers and ventilatory-dependent respiratory failure. Upon transfer to our institution, he developed thrombocytopenia and left hand mottling; doppler revealed a radial artery thrombus. Biopsy of the involved area of skin indicated thrombotic microangiopathy. Pulse dose steroids and unfractionated heparin were initiated. He was extubated within 24 hours and fever resolved. He remained dyspneic and mildly thrombocytopenic and therefore rituximab was initiated. Oxygen requirements, liver dysfunction, and thrombocytopenia resolved. As the prednisone dose was tapered, new microangiopathic lesions were noted. The decision was made to start eculizumab which is ongoing as a maintenance dose every two weeks with no clinical recurrence of CAPS.Case 2: 68-year-old male presented with priapism. On day 2 of hospitalization, he was found to have left lower extremity (LLE) DVTs and received unfractionated heparin. On day 4, he developed fever and thrombocytopenia and was noted to have a cold LLE. Angiography demonstrated occlusion of the posterior tibial artery and heparin was discontinued in favor of an alteplase drip. The patient was subsequently transferred to our institution. Doppler demonstrated multiple DVTs in both lower extremities. Evaluation for HIT was negative. Lupus anticoagulant (LA) was strongly positive raising concern for CAPS. Steroids and PEX were initiated. However, emergency fasciotomy of the RLE was needed to relieve compartment syndrome. Skin biopsy demonstrated microangiopathy. Due to lack of clinical improvement, PEX was discontinued and eculizumab was initiated. On day 3 post-eculizumab initiation, there was evidence of regression of skin mottling. Eculizumab was redosed on day 5. Steroids were tapered and he received 2 additional doses of eculizumab. No additional thrombotic complications occurred. He eventually died during this hospitalization due to septic shock.Case 3: 37-year-old female presented with fever, hypoxia, and painful feet. She was hypotensive and labs demonstrated leukopenia, thrombocytopenia, elevated liver enzymes, and acute kidney injury. Vasopressors and antibiotics were initiated. Over the next 24 hours she developed mottling of her feet, face and breasts. The diagnosis of CAPS was made based on clinical criteria and a positive LA. Unfractionated heparin, steroids and eculizumab were initiated. Dialysis was started due to renal failure. Skin biopsy showed microangiopathy. Marked improvement of skin mottling was noted after 3 doses of eculizumab and renal function normalized after 7 doses. She was discharged with a slow steroid taper, therapeutic enoxaparin, and maintenance eculizumab.Complement blockade with eculizumab is safe and effective in patients with CAPS. These cases suggest that utilizing therapy to inhibit the complement pathway may be an integral component in treating CAPS more effectively. Future directions should include a randomized clinical trial to evaluate eculizumab as part of combination therapy for CAPS. [Display omitted] DisclosuresBroome:True North Therapeutics: Honoraria; Alexion Pharmaceuricals: Honoraria.

Catastrophic antiphospholipid syndrome (CAPS) precipitated by cardiac surgery: A case report and review of the literature

Background: A devastating complication of antiphospholipid syndrome (APS) is catastrophic antiphospholipid syndrome(CAPS), which is extremely rare, difficult to diagnose, and portends a poor prognosis with high mortality. Intra-abdominal andobstetric surgeries have been regarded as precipitating factors in the development of CAPS in patients with pre-existing APS. Wepresent the unique case of a patient who developed CAPS as an immediate complication after valvular replacement surgery.Case presentation: A 57-year-old female was admitted for acute decompensated heart failure secondary to mitral valve stenosis.Her past medical history was suspicious for APS, but she was not formally diagnosed or started on anticoagulant therapy.She ultimately underwent mitral valve replacement. One week post-operatively, she developed intractable abdominal pain,progressive renal failure and thrombocytopenia, and behavioral changes over the course of several days. Extensive investigation forinfection, thrombocytopenic thrombotic purpura (TTP), heparin-induced thrombocytopenia (HIT) and disseminated intravascularcoagulation (DIC) did not explain her rapid clinical deterioration. She did, however, fulfill the criteria for probable CAPS, given that she had rapid involvement of four organ systems and persistent antiphospholipid antibodies. She was started on bothplasmapharesis and hemodialysis 12 days postoperatively, and showed significant improvement in her mentation, abdominal pain,thrombocytopenia, and renal function.Conclusion: This case illustrates the high degree of clinical suspicion and understanding of precipitating factors that are requiredto make a diagnosis of CAPS; furthermore, it delineates the life-saving impact of effective treatment. The correlation of CAPSand surgery has been observed in several reports. However, the current literature posits that CAPS is seldom a complication ofcardiac surgery, and more frequently an immediate complication after abdominal or obstetric surgeries. For this reason, this caseis novel in that it represents one of the few cases in which CAPS was triggered by cardiac surgery involving cardiopulmonarybypass (CPB).

129 Catastrophic antiphospholipid syndrome and pregnancy: A case report

Introduction Association between antiphospholipid antibodies and poor gestational outcomes is recognized for decades. For the diagnosis of Catastrophic Antiphospholipid Syndrome (CAPS) is necessary high clinical suspicion and intensive care support is essential to save the patients in this really catastrophic disorder. Objectives Description of a case report and literature review of CAPS in gestation, looking updates in relation to the early diagnosis and appropriate management of these patients. Methods Case report of CAPS during pregnancy and literature review on Pubmed. Results Z.M.S.S, 33, G5P2, previously diagnosed with primary APS sought care at Hospital Sao Paulo on February 7, 2014, with a 34 weeks pregnancy, presenting hypertension, lower limb edema associated with laboratory findings of proteinuria and thrombocytopenia - Hellp syndrome class II (platelet 57.000). After pressure control, anticonvulsant therapy with magnesium sulfate, she was submitted to an emergency C/S with general anestesia, giving birth to a newborn female, weighing 2130 grams, Apgar 8/8. Evolved rapidly with coagulation disorder, severe epigastric pain radiating to the lower back, in association with sudden onset of pain in the left upper limb, having been referred to intensive care unit where she stayed until February 26. During research, we identified multiple vascular occlusion sites (hepatic microcirculation thrombosis, renal cortical and left brachial vein). The paciente underwent to immunosuppressive therapy with methylprednisolone, immunoglobulin and anticoagulation, with progressive improvement, and was home discharged on March 4, 2014 in good health. Catastrophic Antiphospholipid Syndrome (CAPS), also known as Asherson Syndrome, it’s a potentially fatal variant, that affects less than 1% of patients with antiphospholipid syndrome (APS). It was first described in 1992 by Asherson, is characterized by multiple vascular occlusive events, mainly affecting small vessels. Affects concomitantly different tissues, with progressive and rapid deterioration, leading to multiple organ failure and high mortality. Usually presents thrombocytopenia, hemolytic anemia associated with antiphospholipid antibodies in high titers. It may be the first manifestation of APS in a large percentage of cases. Among the triggers are infections, surgery, trauma, anticoagulation interruption and pregnancy. Clinical support is essential and includes removal of provocative factor, corticosteroid therapy, anticoagulation, intravenous immunoglobulin and plasmapheresis. Conclusion Although less than 1% of patients with APS develop this condition and despite all of the recommended therapies, this potentially fatal outcome (near 50%) shows their importance in modern medicine. Finally, we would like to thank the support of our Intensive Care Unit and Reumatology teams at UNIFESP.

Treatment of catastrophic antiphospholipid syndrome.

Catastrophic antiphospholipid syndrome (CAPS) is a severe manifestation of antiphospholipid syndrome (APS). Although affecting only 1% of patients with APS, the condition is frequently fatal if not recognized and treated early. Here, we will review the current approach to diagnosis and treatment of CAPS. Data from the international 'CAPS registry', spearheaded by the European Forum on Antiphospholipid Antibodies, have improved our understanding of at-risk patients, typical clinical features, and precipitating diagnoses. Current guidelines also continue to support the role of anticoagulants and glucocorticoids as foundation therapy in all patients. Finally, new basic science and case series suggest that novel therapies, such as rituximab and eculizumab, warrant further study. Attention to associated diagnoses, such as infection and systemic lupus erythematosus (SLE), is critical at the time of diagnosis. All patients should be treated with anticoagulants, corticosteroids, and possibly plasma exchange. In patients with SLE, cyclophosphamide should be considered. In refractory or relapsing cases, new therapies, such as rituximab and possibly eculizumab, may be options, but need further study.

A case of catastrophic antiphospholipid syndrome: first report with advanced cardiac imaging using MRI

This present case pertains to a 48-year-old woman with a history of antiphospholipid syndrome, who presented with progressive fatigue, generalized weakness, and orthopnea acutely. She had a prior diagnosis of antiphospholipid syndrome with recurrent deep vein thromboses (DVTs) and repeated demonstration of lupus anticoagulants. She presented in cardiogenic shock with markedly elevated troponin and global myocardial dysfunction on echocardiography, and cardiac catheterization revealed minimal disease. Cardiac magnetic resonance imaging was performed, which revealed findings of perfusion defects and microvascular obstruction, consistent with the pathophysiology of catastrophic antiphospholipid syndrome (CAPS). Diagnosis was made based on supportive imaging, including head magnetic resonance imaging (MRI) revealing multifocal, acute strokes; microvascular thrombosis in the dermis; and subacute renal infarctions. The patient was anticoagulated with intravenous unfractionated heparin and received high-dose methylprednisolone, plasmapheresis, intravenous immunoglobulin, and one dose each of rituximab and cyclophosphamide. She convalesced with eventual myocardial recovery after a complicated course. The diagnosis of CAPS relies on the presence of (1) antiphospholipid antibodies and (2) involvement of multiple organs in a microangiopathic thrombotic process with a close temporal association. The myocardium is frequently affected, and heart failure, either as the presenting symptom or cause of death, is common. Despite echocardiographic evidence of myocardial dysfunction in such patients, MRIs of CAPS have not previously been reported. This case highlights the utility in assessing the involvement of the myocardium by the microangiopathic process with MRI. Because the diagnosis of CAPS requires involvement in multiple organ systems, cardiac MRI is likely an underused tool that not only reaffirms the pathophysiology of CAPS, but could also clue clinicians in to the possibility of a diffuse thrombotic process.

Catastrophic APS in the context of other thrombotic microangiopathies.

The catastrophic antiphospholipid syndrome (CAPS) is a rare disease that affects 1% of cases with antiphospholipid syndrome (APS). CAPS can mimic or overlap with different thrombotic diseases; many patients present with a microthrombotic storm or thrombotic microangiopathic hemolytic anemia (TMHA). Thus, the differential diagnosis of CAPS includes thrombotic thrombocytopenic purpura (TTP), typical and atypical hemolytic uremic syndrome (HUS), systemic infections, malignancies, pregnancy-related disorders, malignant hypertension, heparin-induced thrombocytopenia, and drug-induced thrombotic microangiopathies. Antiphospholipid antibody (aPL) positivity has been proposed as the clue in this differential diagnosis; however, aPL can also occur in healthy people and in those with infections or malignancies. Thus, the differential diagnosis of an aPL-positive patient presenting with a microthrombotic storm is broad; the workup should include a special attention to signs of infection and disseminated malignant disease, assessing the funduscopic signs of malignant hypertension, testing ADAMTS13 activity and anti-heparin-platelet factor 4 (HPF4) antibodies, and searching previous exposure to certain drugs. This article aims to review the main diseases included in the differential diagnosis of CAPS in the context of other thrombotic microangiopathies.

Particular catastrophic antiphospholipid syndrome, on the sole surgical site after breast reduction

A 20-year-old woman treated with vitamin K antagonist for antiphospholipid syndrome (APS) (pulmonary embolisms at age 15) was admitted for breast reduction after bridging therapy. At 2 days post-surgery haematomas appeared on the surgical site and anticoagulant therapy was withheld. She developed a skin and breast necrosis leading to the diagnosis of catastrophic APS. Despite medical treatment (anticoagulant therapy, corticosteroids and intravenous immunoglobulins) and surgery, necrosis continued. After 2 weeks of negative-pressure wound therapy (V.A.C.(®) Therapy™) the patient improved, mammary tissues were alive, well vascularised and budding. Breast reconstruction was then initiated. Artificial dermis graft (MatriDerm(®) 2 mm) was applied, and 3 weeks later the apposition of split-thickness skin graft on it. Six months later, results of the surgery were good and the patient was satisfied.

Catastrophic antiphospholipid syndrome presenting with sudden renal failure: The lesson lies in vascular lesions.

Catastrophic antiphospholipid syndrome presenting with sudden renal failure: The lesson lies in vascular lesions.

Unusual Hepatic Manifestation: Catastrophic Antiphospholipid Syndrome Mimicking Liver Abscesses

Purpose: Catastrophic Antiphospholipid Syndrome (CAPS) is a rare systemic variant of Antiphospholipid Syndrome (APS). Clinically, patients often appear septic, however infection is not the insulting etiology. A 28 year old female presented with right upper quadrant pain and mildly elevated transaminases. Her history included detectable antiphospholipid antibodies and a previous pregnancy complicated with pre-eclampsia. Abdominal ultrasound revealed cholelithiasis and she underwent a laparoscopic cholecystectomy. Postoperatively, she was noted with high fevers, persistent abdominal pain and worsening transaminitis. CAT scan of the abdomen revealed multiple hypodense lesions within the liver concerning for abscesses and she was initiated on intravenous antibiotics. After initial improvement, she returned with abdominal pain, fevers, and continually rising transaminases, progressing into fulminate liver failure with pancytopenia and hypoxia. Repeat imaging revealed worsening hepatic lesions. Trans-jugular biopsy found areas of sharply demarcated hepatocellular necrosis with fibrin deposition consistent with microthrombi within the liver. These histologic findings combined with multi-organ failure in the setting of antiphospholipid antibodies supported the diagnosis of CAPS. Treatment with high dose steroids, anticoagulation, intravenous immunoglobulin and plasmaphoresis lead to a dramatic improvement in her condition. CAPS, first described in 1992 by Asherson, occurs in less than 1% of patients with APS and carries a high mortality rate (>50%). Gastrointestinal manifestations occur in 30% of CAPS cases with hepatic involvement being most common. Diagnosis requires involvement of at least three organ systems, histopathologic findings of small vessel occlusion, and the presence of antiphospholipid antibodies. Our case underscores the importance of quick recognition and aggressive treatment of CAPS. This diagnosis should be considered in any patient with positive antiphospholipid antibodies who appears septic and progressing into multi-organ failure with no discernible infectious etiology.Figure 1: Worsening hepatic lesions seen on CAT scan thought to represents infection but were found to be infarction.

Early diagnosis and successful treatment of catastrophic antiphospholipid syndrome complicated by multiple organ failure

A 50-year-old woman was emergently admitted because of rapidly progressive unconsciousness, renal failure, hepatic dysfunction, hemolytic anemia, thrombocytopenia, and high-grade fever in July, 2008. Based on clinical and laboratory findings, we made a tentative diagnosis of thrombotic thrombocytopenic purpura (TTP) and immediately initiated the plasma exchange (PE). Despite the PE, she developed panperitonitis due to multiple intestinal perforation and massive splenic infarction within a week after the admission. Thrombosis of arterioles at perforated portion in the resected small and large intestines was histologically confirmed. Therefore, we made a definite diagnosis of catastrophic antiphospholipid syndrome (CAPS) based on the presence of antiphosphatidylserine-prothrombin complex antibodies (aPS/PT) throughout the course and lupus anticoagulant that was revealed positive on one occasion, and multiple thrombotic lesions. The underlying disease of CAPS appeared to be lupus erythematosus because of her clinical history and laboratory findings such as persistent leukopenia. Although it has been reported that CAPS causes systemic thrombosis at microvessels mostly within a week from the onset and the mortality rate in this disorder is as high as 50%, we successfully treated her in combination with high-dose corticosteroid, anticoagulation, concentrated human IgG, surgical procedures, and hemodialysis in addition to PE. Early diagnosis of CAPS and immediate start of PE may have contributed to the successful treatment.

Catastrophic antiphospholipid syndrome in a patient with Down syndrome

We report a patient with Down syndrome, under treatment with carbamazepine, levopromazine and clonazepam. After urinary infection he developed glans necrosis requiring excision of prepuce. Six hours post surgery he presented right-hand ischemia followed by arterial and venous thrombosis of the right thoracic extremity. Later, he progressed to a compartment syndrome and presented ischemia of toes. All the clinical manifestations developed over a week. Anticardiolipin (aCL) antibodies, lupus anticoagulant and perinuclear antineutrophil antibodies were positive. Anticoagulant and immunosuppressive treatment were initiated. Owing to the failure of both treatments, the patient underwent amputation of right hand and a toe. Histopathology revealed recent and old thrombosis of medium- and small-sized vessels without vasculitis. Diagnosis of catastrophic antiphospholipid syndrome (CAPS) was made. At present, the patient continues on oral anticoagulants, IgG aCL remains positive, and no further episodes of thromboses have been observed after 4 years of follow-up. To our knowledge, this is the first case of CAPS in a patient with Down syndrome.

A Case of Catastrophic Antiphospholipid Syndrome in an Adolescent Girl With Parvovirus B19 Infection

Antiphospholipid syndrome is an autoimmune disease characterized by recurrent thrombosis and the presence of antiphospholipid antibodies. Clinical presentations are dependent on the affected vessels and organs. The most common presentation of antiphospholipid syndrome is arterial or venous thrombosis. An unusual presentation of the disease is characterized by microvascular thrombosis with multiorgan involvement, which is termed catastrophic antiphospholipid syndrome. The diagnosis of catastrophic antiphospholipid syndrome can be difficult because of the heterogeneity of the different clinical forms. Clinical manifestations of catastrophic antiphospholipid syndrome are complex with multiple organ involvement, resulting in renal insufficiency, heart failure, acute respiratory distress syndrome, and liver involvement. Early diagnosis and aggressive therapies are essential in this condition because of the extremely high mortality rate. Herein, the case of a 14-year-old girl with catastrophic antiphospholipid syndrome that was previously misdiagnosed as a vasculitis related to parvovirus B19 infection is presented.

Catastrophic antiphospholipid syndrome mimicking a malignant pancreatic tumour – a case report

The catastrophic antiphospholipid syndrome is characterised by rapid onset thromboses, often resistant to conventional anticoagulant treatment, and resulting in life threatening multiple organ dysfunction. The diagnosis of catastrophic antiphospholipid syndrome may be difficult, predominantly due to its frequently atypical presentation. We report a case of a 35-year-old female who presented with a pancreatic tumour and extensive thromboses. Following a storm of ischemic events due to thrombotic occlusions in spite of therapeutic heparin dose, the suspicion of catastrophic antiphospholipid syndrome emerged. The patient was successfully treated with anticoagulants, immunoglobulins, plasmapheresis and rituximab. The present report shows that the use of the diluted Russell's viper venom time can be helpful in providing additional information on the lupus anticoagulants antibody status, allowing careful monitoring of lupus anticoagulants conversion and hence response to therapy.

Catastrophic antiphospholipid syndrome and pulmonary embolism in a 3-year-old child

We report a rare example of catastrophic antiphospholipid syndrome (CAPS) in a young child. A 3-year-old girl with no previous medical history presented with extensive and recurrent thromboses. The diagnosis of CAPS was based on the occurrence of cardiopulmonary embolism in the child with a high titre of autoantibodies directed against phospholipids and beta-2-glycoprotein 1. In spite of a relatively rapid diagnosis and multiple treatments, the outcome was unfavourable. Multimodality imaging, including both ultrasonography and spiral CT, allowed close follow-up of the thromboses.

Unusual abdominal manifestations of catastrophic antiphospholipid syndrome

A 40-year-old woman with newly diagnosed Graves’ disease was admitted for carbimazole-induced agranulocytosis. Upon recovery of leucopenia, she developed acute onset of thrombocytopenia, renal failure and microangiopathic haemolytic anaemia. Contrast-enhanced computed tomography of the abdomen (left panel) performed for deranged liver enzymes showed a complete splenic infarct (yellow arrow) and multiple hepatic nodules (red arrows), which were initially thought to be abscesses. Ultrasound-guided fine needle aspiration and core liver biopsy of these nodules identified solid hepatic lesions inconsistent with abscesses. Histology yielded necrotic tissue with no morphological or microbiological evidence of fungal or bacterial infection. Lupus anticoagulant detection by both activated partial thromboplastin time and dilute Russell’s viper venom test with respective confirmatory tests was positive on four separate occasions within 1 month. Immunoassays for anticardiolipin IgG and IgM were, however, negative. A probable diagnosis of catastrophic antiphospholipid syndrome (CAPS) was made. Treatment with unfractionated heparin, intravenous immunoglobulin and plasmaphaeresis was initiated. Severe haematochezia and intra-abdominal sepsis unresponsive to conservative measures (including broad spectrum antibacterial and empiric antifungal therapy) ensued. This necessitated an exploratory laparotomy, which revealed that the spleen had liquefied with coagulative necrosis and had eroded into the splenic flexure of the colon. Additional findings included a gangrenous gall bladder and necrotic liver nodules. Splenectomy, cholecystectomy and segmental colonic resection were performed. The patient developed a right pleural effusion (right panel), which drained fluid and necrotic tissue. Recurrent uncontrolled bleeding from the gastrointestinal tract necessitated a second laparotomy, which revealed necrotic liver nodules eroding into and possibly perforating the gastric fundus, and a thinned-out diaphragm allowing the translocation of abdominal contents into the pleural cavity. Histologic sections of the resected spleen and liver showed complete infarction with the presence of thrombi occluding the larger blood vessels. Small vessel occlusion, however, was not apparent because of the extensive infarction. Cultures of resected tissues and pleural fluid yielded mixed bacterial and fungal infection, which probably occurred secondary to CAPS. The patient unfortunately succumbed to her illness. This case illustrates that CAPS can lead to acute multi-organ failure and can be associated with infections, both of which can result in challenging surgical management issues. Early recognition is essential for effective life-saving treatment to be instituted.