Antiphospholipid Syndrome and Patent Foramen Ovale: A Case Report and Literature Review.

Antiphospholipid syndrome (APS) is a systemic autoimmune disease characterized by arterial, venous, or microvascular thrombosis, pregnancy morbidity, or non-thrombotic manifestations in patients with persistent antiphospholipid antibodies (aPL). Catastrophic APS is a rare and severe form of APS that is defined by the presence of multiple vascular occlusive events. When a patent foramen ovale (PFO) is present, paradoxical embolization can occur, simultaneously leading to arterial and venous thrombosis. We present a complex clinical case of a patient who presented with multiple arterial and venous thrombotic events with positive aPL. The suspicion of catastrophic APS was removed when a PFO was found in a transesophageal echocardiogram, justifying paradoxical embolization. This emphasizes the importance of searching for PFO in patients with APS presenting with simultaneous venous and arterial thrombosis for management and prognosis purposes.

AngioJet thrombectomy with extracorporeal membrane oxygenation support for an acute large-scale pulmonary embolism with bilateral atrial thrombosis: a case report of catastrophic antiphospholipid syndrome.

Background: Catastrophic Antiphospholipid Syndrome (CAPS), a severe systemic autoimmune disorder, predominantly causes life-threatening multi-organ failure, with a high mortality rate. It primarily affects small vessels, seldom impacting large vessels. Notably, acute massive pulmonary embolism (PE) with bilateral atrial thrombosis is an exceptional occurrence in CAPS. Acute pulmonary embolism (PE) is a common cardiovascular disease that progresses rapidly and has a high mortality rate. Acute massive PE combined with bilateral atrial thrombosis has an even higher mortality rate. PE treatments primarily include pharmaceuticals, catheter interventions, and surgical measures, with integrated treatment strategies demonstrating promising outcomes in clinical practice. Extracorporeal membrane oxygenation (ECMO) can provide cardiopulmonary support for the treatment of high-risk PE patients and is a proven therapeutic measure. Methods: This report presents the case of a 52-year-old male admitted due to fever and sudden onset of impaired consciousness, with cardiac ultrasound and pulmonary artery CT angiography revealing an acute large-scale pulmonary embolism accompanied by bilateral atrial thrombosis, with the condition rapidly worsening and manifesting severe respiratory and circulatory failure. With ECMO support, the patient underwent a thrombectomy using an AngioJet intervention. The diagnosis of CAPS was confirmed through clinical presentation and laboratory examination, and treatment was adjusted accordingly. Results: The patient made a successful recovery and was subsequently discharged from the hospital. Conclusion: In CAPS patients, the rare instance of acute massive PE accompanied by bilateral atrial thrombosis significantly risks severe respiratory and circulatory failure, adversely affecting prognosis. Early initiation of ECMO therapy is crucial, offering a vital opportunity to address the root cause. In this case report the patient was successfully treated with an AngioJet thrombectomy supported by ECMO.

The Relentless Recurrence of Diffuse Alveolar Hemorrhage in Catastrophic Antiphospholipid Syndrome and Lupus: A Therapeutic Challenge.

Diffuse alveolar hemorrhage (DAH), a rare complication of coexisting antiphospholipid syndrome (APS) and systemic lupus erythematosus (SLE), poses significant diagnostic and therapeutic challenges, especially with recurrent episodes. We present a 27-year-old male with catastrophic APS and SLE who experienced acute respiratory failure and hemoptysis due to DAH. Despite aggressive therapy with immunosuppressants, plasma exchange, and anticoagulation, he had recurrent DAH episodes requiring repeated admissions. Early recognition, multidisciplinary management, and utilization of effective targeted therapies, such as intravenous immunoglobulin, in refractory cases are crucial for improving outcomes in this challenging complication.

Extensive Thrombosis in Catastrophic Antiphospholipid Syndrome in a Newly Diagnosed Systemic Lupus Erythematosus: A Case Report.

In this case report, we present the development of catastrophic antiphospholipid syndrome (CAPS), a rare and potentially fatal consequence of systemic lupus erythematosus (SLE), in a 33-year-old Micronesian woman. CAPS is characterized by extensive arterial thrombosis and multiorgan failure. The patient first showed signs of neuropsychiatric symptoms, brain infarctions on imaging, and severe hypoxic respiratory failure brought into the hospital by diffuse alveolar hemorrhage (DAH) along with lupus nephritis (LN). Blood urea nitrogen (BUN) and creatinine (Cr) were progressively elevated to 102/4.1 mg/dL, respectively. A urinalysis revealed microscopic hematuria and proteinuria with a urine protein/creatinine ratio of 6052 mg/g. She was also found to have had microangiopathic hemolytic anemia (MAHA) and severe venous thrombosis, both of which were indicative of CAPS. An aggressive approach, including immunosuppressive medication, therapeutic plasma exchange, and anticoagulation, had positive results, including renal recovery and the cessation of thrombotic episodes. This instance highlights how crucial it is to identify CAPS patients early and take appropriate action to improve patient outcomes for this difficult and sometimes deadly disorder.

Management of Anticoagulation and Antifibrinolytics in Catastrophic Antiphospholipid Syndrome.

Antiphospholipid syndrome (APS) is an autoimmune disorder that presents with hypercoagulability and results in a lab artifact of prolonged PTT. The most severe form is catastrophic antiphospholipid antibody syndrome (CAPS), which manifests as rapidly progressing thromboses in multiple organ systems leading to multi-organ ischemia. The mainstay management CAPS is anticoagulation and systemic corticosteroids. Antifibrinolytic agents have previously been thought to be relatively contraindicated in CAPS due to the pro-thrombotic nature of the disease; the complex coagulation profile of CAPS can make it difficult to assess the risks and benefits of antifibrinolytic therapy. Also, should a patient with CAPS require cardiopulmonary bypass (CPB) for surgery, it poses a unique challenge in providing appropriate anticoagulation in the setting of prolonged ACT. We present a case of a 32-year-old postpartum female with CAPS requiring heart transplant who safely received intraoperative antifibrinolytic therapy and was successfully anticoagulated during CPB after perioperative plasmapheresis.

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

Background: 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. Case: 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. Conclusion: 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.

Pediatric catastrophic antiphospholipid syndrome with extensive skin involvement.

Antiphospholipid syndrome (APS) is a state of hypercoagulability due to persistent antiphospholipid antibodies (aPLs) in the blood. Catastrophic APS (CAPS) is a severe form with higher morbidity and mortality in which there occurs widespread thrombosis in multiple organs and hence warrants early diagnosis and aggressive management. We report a case of pediatric CAPS with extensive cutaneous involvement precipitated by infection successfully treated with the combination of high dose systemic corticosteroids, antibiotics, long-term anticoagulation, and wound care.

First presentation of Graves' thyroid storm complicated by catastrophic antiphospholipid antibody syndrome: A case report.

Catastrophic antiphospholipid antibody syndrome is a rare and severe subtype of antiphospholipid syndrome with multisystemic organ failure due to thromboembolic events, resulting in high mortality rates. The association between catastrophic antiphospholipid antibody syndrome and autoimmune thyroid diseases is rarely reported in the literature. We report a case of a 35-year-old previously healthy female with Graves' thyroid storm, positive lupus antibodies, and probable catastrophic antiphospholipid antibody syndrome. Her hospital course was complicated by extensive venous thromboembolism, superior vena cava syndrome, thromboembolic strokes, and Takotsubo cardiomyopathy. Eventually, this led to an unfortunate death secondary to profound shock after 8 days despite emergent treatment. Our case report discusses the link between autoimmune thyroid disorders and catastrophic antiphospholipid antibody syndrome. We emphasize the difficulty in diagnosing catastrophic antiphospholipid antibody syndrome in extremely ill patients and stress the significance of considering it as a possible cause in thyrotoxicosis patients with multiple organ failure and hypercoagulability. Early recognition and prompt management are crucial in improving outcomes in these patients.

Primary catastrophic antiphospholipid syndrome in children with midbrain infarction: a case report.

Background: Catastrophic antiphospholipid syndrome (CAPS) is a multi-system autoimmune disease characterized by extensive thrombosis. Pediatric CAPS is extremely rare and associated with a high mortality rate, especially when midbrain infarction is involved. Hence, early diagnosis and prompt initiation of appropriate treatment for CAPS complicated by midbrain infarction are of utmost importance in achieving favorable outcomes. Case presentation: In this report, we present the case of a 14-year-old girl who presented with neurological symptoms and digestive system infection and was initially diagnosed with an "intracranial infection". After a series of rigorous diagnostic procedures, the patient was ultimately diagnosed with primary CAPS and was immediately transferred to the intensive care unit where she was treated with anticoagulation, glucocorticoids, intravenous immunoglobulin (IVIG) therapy, and multiple plasma infusions. Twenty-seven days after admission, the patient's condition improved with standardized treatment, and she was discharged and followed up regularly. Conclusion: This case report provides a description of the clinical features observed in a pediatric patient with CAPS and concurrent midbrain infarction, highlighting the crucial role of early diagnosis and timely treatment in influencing patient prognosis.

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

PURPOSE OF THE REVIEW: 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. RECENT FINDINGS: 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.

Cardiac involvement in the catastrophic antiphospholipid syndrome (CAPS): Lessons from the "CAPS registry".

OBJECTIVE: To analyze the demographic, clinical, and laboratory characteristics of catastrophic antiphospholipid syndrome (CAPS) patients with cardiac involvement, and to identify the factors associated with this cardiac involvement. MATERIAL AND METHODS: Based on the analysis of the "CAPS Registry", the demographic, clinical, and serological characteristics of patients with cardiac involvement were analyzed. Cardiac involvement was defined as heart failure, valvular disease, acute myocardial infarction, pericardial effusion, pulmonary arterial hypertension, systolic dysfunction, intracardiac thrombosis, and microvascular disease. Univariate and multivariate analysis was used for multiple comparisons. RESULTS: 749 patients (293 [39 %] women and mean age 38.1 ± 16.2 years) accounting for 778 CAPS events were included, of them 404 (52 %) had cardiac involvement. The main cardiac manifestations were heart failure in 185/377 (55 %), valve disease in 116/377 (31 %), and acute myocardial infarction in 104/378 (28 %). Of 58 patients with autopsy/biopsy, 48 (83 %) had cardiac thrombotic microangiopathy, Stroke (29% vs. 21 %, p = 0.012), transient cerebral vascular accident (2% vs. 1 %, p = 0.005), pulmonary infarction (26% vs. 3 %, p = 0.017), renal infarction (46% vs. 35 %, p = 0.006), acute kidney injury (70% vs. 53 %, p < 0.001), and livedo reticularis (24% vs. 17 %, p = 0.016) were significantly more frequent during CAPS events with versus without heart involvement. Multivariate analysis identified acute kidney injury (OR 1.068, IC 95 % 1.8-4.8, p < 0.001) as the only clinical characteristics that were, independently, associated with cardiac involvement in CAPS events. Cardiac involvement was not related to higher mortality. CONCLUSIONS: Cardiac involvement is frequent in CAPS, with association with kidney involvement, and it is not related to higher mortality. The presence of cardiac microthrombosis was demonstrated in most biopsies/autopsies performed.

What we know and what we don't know about catastrophic antiphospholipid syndrome.

Catastrophic antiphospholipid syndrome (CAPS) is a severe condition with high mortality. Since its description in 1992, an important effort has been made to improve and disseminate knowledge on CAPS. Most of our current knowledge comes from the studies performed using the CAPS Registry, a database created in 2000 to gather as many cases as possible in order to better define this disease. It has demonstrated that this condition has multiple faces and is often triggered by a precipitating factor that leads to a thrombotic microangiopathy and cytokine storm involving almost any organ of the body. Analysis of the CAPS Registry has also shown that patients receiving anticoagulation, glucocorticoids and plasma exchange and/or IVIG have a better prognosis. However, there are still many unresolved questions. In this review we summarize what is known and what is still a matter of research in this condition.

Catastrophic Antiphospholipid Syndrome.

Unlike classic APS, CAPS causes multiple microthrombosis due to an increased inflammatory response, known as a "thrombotic storm". CAPS typically develops after infection, trauma, or surgery and begins with the following symptoms: fever, thrombocytopenia, muscle weakness, visual and cognitive disturbances, abdominal pain, renal failure, and disseminated intravascular coagulation. Although the presence of antiphospholipid antibodies in the blood is one of the diagnostic criteria, the level of these antibodies can fluctuate significantly, which complicates the diagnostic process and can lead to erroneous interpretation of rapidly developing symptoms. Triple therapy is often used to treat CAPS, which includes the use of anticoagulants, plasmapheresis, and high doses of glucocorticosteroids and, in some cases, additional intravenous immunoglobulins. The use of LMWH is recommended as the drug of choice due to its anti-inflammatory and anticoagulant properties. CAPS is a multifactorial disease that requires not only an interdisciplinary approach but also highly qualified medical care, adequate and timely diagnosis, and appropriate prevention in the context of relapse or occurrence of the disease. Improved new clinical protocols and education of medical personnel regarding CAPS can significantly improve the therapeutic approach and reduce mortality rates.

Choroidal involvement in patients with catastrophic anti-phospholipid syndrome: Two case reports.

PURPOSE: To describe choroidal involvement in catastrophic antiphospholipid syndrome (CAPS). METHODS: We report here two cases of bilateral CAPS choroidopathy in two female patients. RESULTS: Case report 1: A thirty-five-year-old female patient, with history of primary anti-phospholipid syndrome (APS), treated with anticoagulants, presented an acute renal failure following a salpingectomy. She complained of bilateral acute blurred vision. Ophthalmologic evaluation revealed visual acuity (VA) of 5/10, extensive serous retinal (SRD) detachment, areas of hypofluorescence on fluorescein angiography (FA), and non-perfusion areas in the choriocapillaris, on optical coherence tomography angiography (OCT-A), in both eyes. Considering the diagnosis of probable CAPS, the patient received intravenous pulse steroids, plasmapheresis, intravenous anticoagulation and haemodialysis, with favourable evolution. Case report 2: A thirty-three-year-old female patient, with history of systemic lupus erythematosus (SLE) and secondary APS, treated with corticosteroids, immunosuppressive agents and anti-coagulation, presented a myocardiac infarction. She complained of bilateral acute blurred vision. Ophthalmologic evaluation revealed VA of 1/10 in the RE and 6/10 in LE, bilateral extensive SRD, leakage points on FA and non-perfusion areas in the choriocapillaris on OCT-A. Criteria of probable CAPS were fulfilled. Treatment with intravenous pulse steroids, anticoagulation and reanimation modalities allowed VA improvement. Alveolar haemorrhage and cardiogenic shock led to fatal evolution. CONCLUSION: Our case reports highlight the importance of early diagnosis and ophthalmic evaluation in CAPS. Multidisciplinary approach and rapid initiation of effective treatment, based on corticosteroids, anticoagulation and plasmapheresis, allow better vital and visual prognosis.

Cardiac arrest in the setting of probable catastrophic antiphospholipid syndrome in young patient with a history of COVID infection and polyglandular disorder-Case report.

Antiphospholipid syndrome is an autoimmune disorder characterized by arterial and venous thrombosis and recurrent spontaneous abortions due to the persistent presence of antiphospholipid antibodies. Probable Catastrophic antiphospholipid (Catastrophic antiphospholipid-like syndrome) is a life-threatening presentation of antiphospholipid syndrome which manifests as intravascular thrombosis, leading to rapid onset of symptoms and involvement of multiple organ systems. We present a case of a 28-year-old woman with a history of polyglandular autoimmune syndrome, systemic lupus erythematosus, provoked bilateral deep vein thrombosis in the setting of Severe Acute Respiratory Syndrome Coronavirus 2 (SARS-CoV-2) infection 2 years prior, and hypothyroidism who presents with a cardiac arrest in the setting of an acute ST-elevation myocardial infarction with thromboembolic occlusion of two coronary arteries simultaneously in the setting of noncompliance with anticoagulation for the past 1 week. Her presentation was further complicated by acute hypoxic respiratory failure due to diffuse alveolar hemorrhage during the hospital course with progressive multiorgan failure and eventual death. Catastrophic antiphospholipid is associated with high morbidity and mortality, thus a timely diagnosis and multidisciplinary approach to management is needed for evaluation and management.

Complement biomarkers in the antiphospholipid syndrome - Approaches to quantification and implications for clinical management.

Complement is a major driver of antiphospholipid syndrome (APS) and a promising therapeutic target in refractory and catastrophic APS. Complement testing in APS is largely limited to research settings, and reliable, rapid-turnaround biomarkers are needed to predict those at risk for adverse clinical outcomes and most likely to benefit from complement inhibition. We review complement biomarkers and their association with thrombosis and obstetric outcomes, including: (i) complement proteins and activation fragments in the fluid phase; (ii) assays that evaluate complement on cell membranes (e.g. in vivo cell-bound complement fragments, hemolytic assays, and ex vivo 'functional' cell-based assays, and (iii) sequencing of complement genes. Current studies highlight the inconsistencies in testing both between studies and various aPL/APS subgroups, suggesting that either cell-based testing or multiplex panels employing a combination of biomarkers simultaneously may be most clinically relevant. Standardization of complement assays is needed to ensure reproducibility and establish clinically relevant applications.

Pulmonary involvement in catastrophic antiphospholipid syndrome: A descriptive analysis from the "CAPS Registry".

OBJECTIVES: To describe the pulmonary involvement in patients with catastrophic antiphospholipid syndrome (CAPS), focusing on its relationship with extrapulmonary involvement, laboratory, radiological, and pathological findings. METHODS: This retrospective cross-sectional study includes all patients grouped in the "CAPS Registry". All cases were reviewed, and those with pulmonary thromboembolism (PE) and/or diffuse alveolar hemorrhage (DAH) were selected. Data on pulmonary and extrapulmonary clinical presentation, radiologic patterns, laboratory findings, associated autoimmune diseases, treatments, and outcomes were analyzed. Frequency distribution and measures of central tendency were used to describe the cohort. Comparison between groups regarding qualitative variables was undertaken by chi-square or Fisher exact test, while T-test for independent variables was used to compare groups regarding continuous variables. IBM-SPSS v.22 was used for data analysis. RESULTS: PE was reported in 129 (48.6 %) episodes, DAH in 75 (28.3 %) episodes, and overlap (DAH plus PE) in 7 (2.6 %) episodes. Bronchoalveolar lavage (BAL) was performed in 35 (4.9 %) CAPS episodes, and lung pathology samples were obtained in 84 (10.5 %) episodes (including autopsies). A significant relationship was observed between DAH and laboratory features of thrombotic microangiopathy (TMA). A meaningful relationship was also found between triple antiphospholipid antibody positivity and pathological TMA (26.5 %) as well as hypocomplementemia and DAH (24 %). CONCLUSIONS: Pulmonary involvement may include both TMA and non-thrombotic inflammation, which can be differentiated into three patterns: PE, DAH with systemic TMA with hypocomplementemia or DAH without systemic TMA with/without hypocomplementemia.

Síndrome antifosfolípidos catastrófico en México. Revisión de la bibliografía / Catastrophic antiphospholipid syndrome in Mexico. Literature review

El síndrome antifosfolípidos catastrófico (SAFC) es una entidad rara. Se han reportado aproximadamente 600 casos en todo el mundo, y se desconoce la prevalencia en México. Objetivo: Conocer la prevalencia estimada de SAFC en México. Material y métodos: Se realizó una búsqueda bibliográfica de casos clínicos aislados o series de casos en los diversos buscadores, utilizando los términos «síndrome antifosfolípidos catastrófico» y «México», en mayo de 2022. Resultados: Encontramos una serie de casos retrospectivos en necropsias que incluyeron 12 casos, dos reportes que incluyeron 2 casos cada uno, y también se encontraron reportes de 11 casos clínicos aislados; estas publicaciones se generaron entre 2003 y 2020. En total, se tienen datos de 27 casos de SAFC, de los cuales 16 corresponden al síndrome antifosfolípidos primario, 10 en asociación con lupus eritematoso sistémico y 1 caso de esclerosis sistémica. La tasa de prevalencia estimada en la población mexicana en 2022 es de 2 casos por cada 10.000.000 de habitantes. La mortalidad estimada fue del 68% en esta serie de casos. Conclusión: Los casos de SAFC en México están subreportados; sin embargo, identificarlos ayudará a mejorar las estrategias diagnósticas y terapéuticas que se utilizan actualmente en el país, incentivando la implementación de la triple terapia y, en casos refractarios, el uso de eculizumab, para reducir la mortalidad actual. (AU)

Catastrophic antiphospholipid syndrome (CAPS) is a rare entity, approximately 600 cases have been reported around the world, and the prevalence in Mexico is unknown. Objective: To determine the estimated prevalence of CAPS in Mexico. Material and methods: A literature search of isolated clinical cases or case series was conducted in diverse search engines, using the terms: «catastrophic antiphospholipid syndrome» and «Mexico» in May 2022. Results: We found a series of retrospective cases in autopsies that included 12 cases, two reports that included 2 cases each, and reports of 11 isolated clinical cases; these publications were generated between 2003 and 2020. In total, we collected data on 27 cases of CAPS, of which 16 correspond to primary antiphospholipid syndrome, 10 are associated with systemic lupus erythematosus, and 1 case corresponds to systemic sclerosis. The estimated prevalence rate in the Mexican population in 2022 is 2 cases per 10,000,000 inhabitants. The estimated mortality was 68% in this case series. Conclusion: Cases of catastrophic antiphospholipid syndrome in Mexico are underreported; identifying them will help improve current diagnostic and therapeutic strategies used in the country, encouraging the implementation of triple therapy and, in refractory cases, the use of eculizumab, to reduce current mortality. (AU)

Catastrophic Antiphospholipid Syndrome: A Complex Diagnosis in the Setting of Lupus.

This case report aims to highlight the importance of keeping catastrophic antiphospholipid syndrome (CAPS) high on the list of differentials in patients with lupus who present with digital ischemia and to understand the workup and treatment of the disease. Catastrophic antiphospholipid syndrome is a life-threatening variant of antiphospholipid syndrome (APS), and it is distinguished on the APS spectrum by its increased intensity and extent of thrombotic outcomes. Less than 1% of patients with APS develop CAPS and the demographic of patients affected are primarily females, 37 ± 14 years old, and have underlying primary APS or systemic lupus erythematosus (SLE). This is the case of a young female with lupus and end-stage renal disease secondary to lupus nephritis who presented to the emergency department for shortness of breath and bilateral leg swelling that eventually progressed to catastrophic antiphospholipid syndrome. She developed pulmonary embolisms, axillary hematoma, and bilateral lower extremity digital gangrene. The treatment course consisted of anticoagulation, steroids, intravenous immunoglobulin (IVIG), above-knee amputation, and eventually rituximab. Diagnosis and treatment of digital ischemia can be complex, especially, in the setting of lupus where the differential diagnosis is broad. A high index of suspicion for CAPS is essential for early diagnosis and treatment.