Anesthetic implications of the catastrophic antiphospholipid syndrome

The antiphospholipid antibody syndrome (or the anticardiolipin antibody syndrome) is characterized by the presence of autoantibodies. Its major association is with systemic lupus erythematosus. 'Catastrophic' antiphospholipid syndrome (CAPS) is defined as an accelerated form of APS usually resulting in multiorgan failure and can be precipitated by surgery. We present the case of a 12-year-old male child who presented for enucleation of his left eye for fungal endopthalmitis. This patient had a history of CAPS 2 months before surgery with myocardial, gastrointestinal, renal and laryngeal involvement that improved on aggressive treatment and was subsequently managed on an outpatient basis for 2 months before presenting for enucleation. To the best of our knowledge, this is the first case of CAPS in a child reported in the anesthetic literature. Further aspects of this puzzling condition and its anesthesia implications are discussed.     

Catastrophic antiphospholipid antibody syndrome following initiation of hemodialysis

Antiphospholipid syndrome (APS) is associated with arterial and venous thrombosis, pregnancy morbidity, and thrombocytopenia. Some APS patients develop rapid and disseminated microthrombosis and are known as having catastrophic APS or CAPS. We document here a case of CAPS in a patient who presented with various clinical symptoms and serious abnormalities of blood coagulation following initiation of hemodialysis after bilateral nephrectomy due to renal cancer. The patient developed multiple organ symptoms, including melena, visual disturbances, skin eruptions, lymph node swelling, urinary tract bleeding, backache, arteriovenous fistula occlusion, and chest pain. Based on the clinical course and serological and histological examinations, a diagnosis of CAPS was established. The patient recovered by following intensive anticoagulation and steroid therapy. Although CAPS is rare, once symptoms develop the condition deteriorates rapidly. Because of the associated high mortality, early diagnosis and prompt treatment are necessary.     

Catastrophic antiphospholipid syndrome in a 14-year-old child

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

Catastrophic antiphospholipid syndrome: report of 4 cases

Catastrophic antiphospholipid syndrome (CAPS), described by Asherson in 1992, is a rare form of antiphospholipid syndrome resulting in multiorgan failure with a mortality rate of about 50%. The syndrome occurs in patients with either systemic lupus erythematosus and other rheumatic diseases (systemic sclerosis, rheumatoid arthritis, primary Sjogren syndrome) or alone. Whereas in "classic" antiphospholipid syndrome (APS), medium-large vessels are involved, a diffuse small vessel ischemia and thrombosis (microangiopathic disease) leading to a severe multiorgan dysfunction is predominant in CAPS. "Trigger" factors have been demonstrated in 45% of patients, but in the majority, they remain unknown. Not infrequently, CAPS arises in patients without any previous thrombotic history. The kidney is the organ most commonly affected, followed by the lung, the central nervous system, the heart and the skin. Disseminated intravascular coagulation occurs in approximately 13% of patients. The present study reports the clinical and serological features of 4 patients affected by this rare form of antiphospholipid syndrome. Nephrologists should be aware of the possibility of this syndrome as a cause of multiorgan failure since prompt recognition is essential for effective treatment.     

Infarction of the right hemisphere in a patient with antiphospholipid antibody syndrome

Summary Hemicraniectomy as a surgical treatment for intracranial pressure following large ischemic lesions is widely practiced in selected patients. The antiphospholipid antibody syndrome (APS), a disorder characterized by recurrent arterial and venous thrombosis, is a very rare cause of space occupying ischemic lesions. We present a case of a 35 year old female diagnosed with APS who initially presented with small ischemic lesions and within days developed a massive near-total infarction of the right hemisphere. Because of central nervous system, skin and systemic manifestations Sneddon’s syndrome and catastrophic antiphospholipid antibody syndrome (CAPS) remained a possible diagnoses. Sneddon’s syndrome is a non-inflammatory occlusive arteriopathy of small and medium size arteries predominantly of the skin and brain, whereas the catastrophic antiphospholipid antibody syndrome is characterized by acute multi-organ system thrombosis of small and large vessels. In addition to the diagnostic criteria for APS a heterozygous factor V Leiden mutation was found in this patient, which may be a contributing risk factor for cerebral ischemia. When considering invasive decompressive procedures the neurosurgeon has to be aware of the poor prognosis of some forms of APS with systemic manifestations.These authors contributed equally to this work.     

Predictable removal of anticardiolipin antibody by therapeutic plasma exchange (TPE) in catastrophic antiphospholipid antibody syndrome (CAPS)

Catastrophic antiphospholipid antibody syndrome (CAPS) is a rare life-threatening variant of antiphospholipid antibody syndrome (APS), with an associated mortality rate of > 50%. Treatment recommendations are aggressive and consist of intravenous heparin, steroids, immunoglobulins and/or therapeutic plasma exchange (TPE). At present, insufficient data exist to make precise recommendations regarding the most effective therapy for CAPS. Accumulating evidence over recent years is encouraging and may lead to future guidelines. We report predictive and effective removal of pathological anticardiolipin antibody (aCL AB) in a patient with CAPS. The case report and discussion provide valuable insight into aCL AB production and its removal by first- order kinetics using TPE.     

Catastrophic antiphospholipid antibody syndrome

Antiphospholipid antibody syndrome (APS) is characterized by recurrent thrombosis with the presence of circulating antiphospholipid antibodies. A diagnosis of APS requires the presence of at least one clinical and one laboratory criteria (detection of aCL IgG or IgM antibodies or the presence of lupus anticoagulant on two or more consecutive occasions 6 weeks apart). A severe, rapidly progressive form characterized by clinical involvement of at least three different organ systems with histopathological evidence of small and large vessel occlusion is termed catastrophic antiphospholipid syndrome. Early recognition of APS is crucial since aggressive management can result in a favorable outcome. We present the case of a 12-year-old boy who presented with a devastating illness with multiple thrombotic episodes and rapidly progressive renal failure.     

Antiphospholipid syndrome nephropathy in systemic lupus erythematosus.

In the course of the antiphospholipid syndrome (APS), the existence of vaso-occlusive lesions capable of affecting numerous organs is now well established. The renal involvement attributable to primary APS, APS nephropathy (APSN), corresponds to vaso-occlusive lesions of the intrarenal vessels, associating side-by-side, acute thromboses with chronic arterial and arteriolar lesions, leading to zones of cortical ischemic atrophy. A retrospective study of 114 lupus patients undergoing renal biopsy was undertaken to determine the following: (1) if APSN can be found in the course of systemic lupus erythematosus (SLE); (2) if certain clinical and biologic factors can permit the prediction of the presence of APSN; and (3) if APSN is a superadded renal morbidity factor in lupus patients. This study shows the following: (1) APSN occurs in SLE (32% of patients with renal biopsies) in addition to, and independently of, lupus nephritis; (2) APSN is statistically associated with lupus anticoagulant but not with anticardiolipin antibodies; (3) APSN is associated with extrarenal APS, mainly arterial thromboses and obstetrical fetal loss, but not with the venous thromboses of APS; (4) APSN is an independent risk factor, over and above lupus nephritis, that contributes to an elevated prevalence of hypertension, elevated serum creatinine, and increased interstitial fibrosis. Thus, it seems likely that, because of its associations with hypertension, elevated serum creatinine, and increased interstitial fibrosis, APSN may worsen the prognosis in these patients. APSN may also have therapeutic significance in that its recognition should permit a better balance between immunosuppressor and antithrombotic and/or vasoprotective therapy. Finally, this study suggests that APSN should be considered as an element to be included in the classification criteria of APS.     

End-stage renal disease from glomerulonephritis associated with anti-phospholipid syndrome

Primary anti-phospholipid syndrome (APS) is perceived to be an uncommon disorder, infrequently recognized as a cause of renal disease in childhood. While renal involvement in APS classically manifests as thrombotic events, other renal diseases associated with APS have been reported in adults, including membranous nephropathy and minimal change disease. We report our experience of caring for a child who presented with acute anuric renal failure due to anti-neutrophilic cytoplasmic antibody-negative rapidly progressive glomerulonephritis (RPGN), with concomitant thrombotic microangiopathy (TMA). Recognition of the APS as a cause of the patients TMA facilitated institution of anticoagulation. Our patients renal failure did not improve and the patient remained dependent on dialysis until he was successfully transplanted. The purpose of our report is to make health-care professionals aware of the previously unreported association of pauci-immune RPGN and APS in children; early recognition of APS will allow initiation of anticoagulation to prevent recurrent thromboses and enable successful transplantation.     

Catastrophic antiphospholipid syndrome presenting as fever of unknown origin

Antiphospholipid syndrome is characterized by the presence of antiphospholipid antibodies with characteristic clinical manifestation, which include venous, arterial thrombosis, thrombotic microangiopathy, and recurrent fetal loss. The syndrome can be secondary to many causes including systemic lupus erythematosus (SLE) or "primary" antiphospholipid syndrome (APLS). We report a case of a man with catastrophic antiphospholipid syndrome (CAPS), which occurs when three or more organ systems are affected by thrombosis in less than a week. Catastrophic antiphospholipid syndrome is uncommon but often fatal. The patient received a successful treatment that controlled this disease and included intravenous heparin, antiplatelet, intravenous corticosteroid, and plasmapheresis.     

Recurrent ischemia in a young man with the antiphospholipid syndrome

Recurrent thromboses, cerebral disease, miscarriages, and antiphospholipid antibodies are characteristic of the antiphospholipid syndrome. A 31-year-old man presented with limb ischemic and isolated right ventricular failure. Antiphospholipid syndrome was suspected and limb salvage was accomplished by anticoagulation and tibial- to-plantar artery bypass surgery. However, recurrent ischemic episodes were successfully treated with thrombolytic therapy and anticoagulants. The surgeon should be aware that patients with antiphospholipid antibodies and lupus anticoagulant antibodies have a high propensity for recurrent arterial thromboses and should use multiple therapeutic approaches to effect successful long-term limb salvage.     

The intrarenal vascular lesions associated with primary antiphospholipid syndrome

Even 10 yr after the identification of the antiphospholipid syndrome (APS), renal involvement in the course of APS is still relatively unrecognized, and is probably underestimated. The association of anticardiolipin antibodies and/or lupus anticoagulant with the development of a vaso-occlusive process involving numerous organs is now confirmed. In a multicenter study, 16 cases of "primary" APS (PAPS) were found and followed for 5 yr or more, all with renal biopsy. In all 16 cases of PAPS, there was a vascular nephropathy characterized by small vessel vaso-occlusive lesions associated with fibrous intimal hyperplasia of interlobular arteries (12 patients), recanalizing thrombi in arteries and arterioles (six patients), and focal cortical atrophy (10 patients). In combination, these led to progressive destruction of the kidney, accelerated by acute glomerular and arteriolar microangiopathy in five patients. Focal cortical atrophy is a distinctive lesion, present in 10 biopsies, and likely represents the histologic and functional renal analogue to the multiple cerebral infarcts detected on imaging studies. The clinical hallmark of this vascular nephropathy in PAPS is systemic hypertension, only variably associated with renal insufficiency, proteinuria, or hematuria. The ensemble of histologic renal lesions defined in this study should aid in the separation of the lesions found in cases of secondary APS, especially systemic lupus erythematosus, into those lesions related to APS and those related to the underlying disease.     

Prevention of thrombosis with prostaglandin E1 in a patient with catastrophic antiphospholipid syndrome

PURPOSE: Catastrophic antiphospholipid syndrome (CAPS) is a variant of antiphospholipid syndrome and presents with life-threatening symptoms of multiorgan failure due to thrombosis. We present a patient with CAPS secondary to an ovarian cancer. In such cases, it is believed that the thrombotic risk disappears after surgical removal of the cancer. The intraoperative management was challenging because of the risks of two opposing complications: catastrophic exacerbation of the thrombotic tendency triggered by the surgical stimulus and major bleeding due to the necessary anticoagulation. We describe the intraoperative management of hemostasis in a patient with CAPS. CLINICAL FEATURES: A 44-yr-old female patient with CAPS underwent resection of an ovarian cancer, which was suspected to be associated with her coagulation abnormality. She had both arterial and venous thromboembolism, including cerebral infarction, embolic gangrene, and pulmonary emboli. Serological examinations revealed increased anticardiolipin IgG antibody and decreased protein C activity. Before surgery, an inferior vena cava filter was placed to prevent perioperative pulmonary embolism. Prostaglandin E(1) (PGE(1); 100 ng.kg(-1).min(-1)) was given intraoperatively to suppress platelet aggregation and thrombin generation and to maintain arterial blood flow. No apparent coagulation abnormalities were observed during surgery, neither hypercoagulation nor a tendency to bleed. No additional thrombotic symptoms developed during a six-month follow-up. CONCLUSION: The use of PGE(1), an inhibitor of thrombin formation and platelet function, and placement of an inferior vena cava filter were associated with the uneventful surgical resection of an ovarian cancer in a patient with CAPS.     

Transpedicular curettage and drainage versus combined anterior and posterior surgery in infectious spondylodiscitis

Background:

Hematogeneous infectious spondylodiscitis usually occurs in older immunocompromised patients with other comorbidities. They are usually unable to undergo reconstructive anterior and posterior surgeries. Therefore, an alternative, less aggressive surgical method of transpedicular curettage and drainage was suggested. This study was designed to compare the surgical outcomes for the treatment of hematogeneous infectious spondylodiscitis between transpedicular curettage and drainage technique and conventional combined anterior and posterior surgery.

Materials and Methods:

Between January 2002 and July 2011, 26 patients underwent surgical treatment for hematogeneous infectious spondylodiscitis. The patients were classified into two groups depending on surgical modality: a transpedicular curettage and drainage (TCD) group and a combined anterior and posterior surgery (CAPS) group.

Results:

The TCD group consisted of 10 patients (mean age 68.0 years), and the CAPS group consisted of 16 patients (mean age 58.4 years). The mean postoperative followup periods were 36.9 (months) in the TCD group and 69.9 (months) in the CAPS group. The operation time was 180.6 ± 33.6 minutes in the TCD group and 332.7 ± 74.5 minutes in the CAPS group (P < 0.05). Postoperative independent ambulation began at postoperative 4.9 ± 2.4 days in the TCD group but at postoperative 15.1 ± 15.3 days in the CAPS group (P < 0.05). The postoperative hospital stays were 19.9 ± 7.8 days in the TCD group and 35.4 ± 33.3 days in the CAPS group (P < 0.05). The level of C-reactive proteins decreased significantly in both groups after surgery (P < 0.05).

Conclusion:

Transpedicular curettage and drainage technique proved to be a useful technique for treating hematogeneous infectious spondylodiscitis in patients who were in poor heath with multiple comorbidities unable to undergo the conventional combined anterior and posterior surgery in a single day in terms of earlier ambulation, shorter hospitalization and similar clinical success rate.     

Antiphospholipid syndrome in cardiac surgery-an underestimated coagulation disorder?

OBJECTIVE: Antiphospholipid syndrome (APS) is a rare coagulation disorder associated with recurrent arterial and venous thrombotic events. We analysed our experience with five APS patients who underwent cardiac surgery. In three of them the diagnosis of APS had been established before surgery, two patients were diagnosed after surgery. METHODS: From March 1999 to March 2004 five patients with APS underwent cardiac surgery using cardiopulmonary bypass (CPB). We retrospectively reviewed their clinical data, operative and postoperative courses, and the long-term results. RESULTS: Procedures performed were heart and lung transplantation (patient 1), endoventriculoplasty and CABG (patient 2), biventricular resection of endoventricular fibrosis and thrombus (patient 3), mitral valve repair repair and coronary artery bypass grafting (CABG, patient 4), and mitral valve replacement with closure of a patent foramen ovale (patient 5). There were three perioperative deaths (patients 1, 2 and 3), two of three patients in whom the diagnosis was known before surgery, survived (patients 4 and 5). In these patients, only half the dose of protamin (patient 4) and no protamin at all (patient 5) was applied to reduce the probability of postoperative thromboembolic complications. At 1 year follow up, only patient 4 had survived, patient 5 had died of the complications of intestinal thromboembolism. CONCLUSIONS: Patients with APS undergoing cardiac surgery belong to a high risk subgroup. Thus, though rare, APS can be a critical issue in cardiac surgery. Some of the cardiac patients with unexplained perioperative thromboembolic complications, such as graft occlusion, may turn out to have an undiagnosed APS.     

Anesthetic management of autoimmune polyglandular syndrome (Schmidt's syndrome)--a case report-

BACKGROUND: Autoimmune polyglandular syndromes (APS) are complex diseases with diverse clinical presentations resulting from involvement of multiple endocrine glands. Surgery under anesthetic in these patients is challenging. A case of Schmidt syndrome (autoimmune polyglandular syndrome type 11) that developed adrenocortical insufficiency in the postoperative period is reported. Etiology, pathogenesis, types and anesthetic problems associated with these cases are discussed. CASE REPORT: A 41 yr old female patient, diagnosed to have APS (Schmidt syndrome) presented for uterine surgery. She had autoimmune glandular involvement of pituitary, thyroid, parathyroid, adrenals and melanocytes and was on hormone replacements for the deficiencies incurred, which were continued till the morning of surgery. Surgery was conducted under general anesthetic combined with epidural analgesia. In spite of supplementation of steroid in physiological doses prior to surgery, she developed hemodynamic instability in the early postoperative period, but could be successfully resuscitated with additional steroid dosage and fluids. CONCLUSION: This patient presented with multiglandular endocrine involvement necessitating timely, adequate hormone replacement and appropriate fluid management. These challenges require careful approach to anesthetic management.     

Human blood‐based anti‐inflammatory solution inhibits osteoarthritis progression in a meniscal‐tear rat study

Current osteoarthritis (OA) research searches for treatments that modify the course of disease progression. Autologous Protein Solution (APS) is derived from whole blood and is a unique autologous therapy that contains high concentrations of white blood cells, platelets, and concentrated plasma, providing cytokines that can target the underlying mechanisms of disease progression. The APS Kit is currently under investigation for clinical use in the USA (NCT02262364). The aim of this study was to determine if APS has disease‐modifying properties in the well‐characterized rat meniscal tear‐induced model of OA. Thirty male athymic rats underwent surgery to induce OA by a complete tear of the medial meniscus of the right knee. Seven days later, rats were administered 50 μl intra‐articular (IA) APS from a human donor or phosphate buffered saline (PBS) control. Rats were euthanized 3 weeks following treatment and knee joints were processed for histological analysis. Collagen and cartilage degeneration were decreased by APS treatment, resulting in a significantly improved total joint score in APS‐treated rats compared to those treated with the PBS control. No significant variations in gait analysis, weight gain, osteophyte score, or synovitis score were observed between APS‐ and PBS‐treated animals. There were no adverse effects of APS reported in the study. Treatment with a single IA injection of APS reduced the cartilage degeneration that characterizes the progression of OA. Further studies are necessary to determine if APS can modify OA disease progression in humans. © 2017 Orthopaedic Research Society. Published by Wiley Periodicals, Inc. J Orthop Res 35:2260–2268, 2017.

     

Cryopyrin-Associated Periodic Fever Syndrome and the Nervous System

Purpose of review

The purpose of this review is to highlight the molecular and clinical characteristics of the cryopyrin-associated periodic fever syndrome (CAPS) and its management. CAPS is an autosomal dominantly inherited autoinflammatory disorder associated with mutations in the NLRP3 gene, which ultimately lead to excessive production of interleukin-1β (IL-1β) and systemic inflammation. Typical systemic features include fever, urticarial rash and arthralgia, and ultimately amyloidosis. There are also multiple neurological manifestations including, but not restricted to, headache, sensorineural hearing loss, aseptic meningitis, myalgia and optic nerve involvement.

Recent findings

Since the recognition of CAPS as a single disease entity and discovery of the underlying causative gene, there has been a major breakthrough in terms of its treatment by pharmacological IL-1β inhibition. Highly targeted therapies against IL-1 have been shown to be remarkably effective in the treatment of CAPS and make early diagnosis of this condition crucial. It is hoped that starting pharmacological intervention in a timely manner will prove neuroprotective. There are three drugs licensed for treatment of CAPS; canakinumab, anakinra and rilonacept. The former two are widely used: canakinumab is a fully humanised anti-IL-1β monoclonal antibody administered as a subcutaneous injection once every 8 weeks starting at a dose of 150 mg in patients weighing more than 40 kg. Anakinra is a recombinant form of the IL-1 receptor antagonist and the adult daily dose is 100 mg subcutaneously.

Summary

CAPS is a highly debilitating disorder characterised by unregulated IL-1β production driven by autosomal dominantly inherited mutations in the NLRP3 gene. Effective therapies targeted against IL-1 are now available and are vital to prevent long-term complications.

     

Case of primary antiphospholipid antibody syndrome with repeated renal biopsies

Antiphospholipid antibody syndrome (APS) is characterized by the presence of repeated arterial and venous thrombosis, recurrent fetal loss and thrombocytopenia. Recently, renal involvement associated with APS is being increasingly recognized and discussed. In most cases, there has been a vascular nephropathy characterized by small vessel vaso-occulusive lesions associated with fibrous intimal hyperplasia of the interlobular arteries, thrombosis and focal cortical atrophy. We report a case of a 38-year-old patient with primary APS. Renal biopsies were performed three times in 26 years. Various glomerular and vascular lesions associated with APS were observed and discussed.     

Recurrent,Spontaneous Esophageal Ruptures Associated With Antiphospholipid Antibody Syndrome: Report of a Case

A 52-year-old man was admitted to our hospital with a spontaneous esophageal rupture (Boerhaave syndrome) and was successfully treated. Eight years after the first incident, he was readmitted with a recurrent rupture. Recurrence of Boerhaave syndrome is extremely rare, with only 7 cases reported in the English literature. During treatment, the patient was also diagnosed with antiphospholipid syndrome (APS). Although APS is known to cause a variety of symptoms due to vascular thrombosis, recurrence of Boerhaave syndrome, coincident with APS, has never been reported. The pathogenesis of Boerhaave syndrome has not been clearly determined. This report serves to increase awareness of the risk of APS, which results in an increased risk of spontaneous rupture of the esophagus.Key words: Boerhaave syndrome, Esophageal rupture, Antiphospholipid syndromeSpontaneous rupture of the esophagus (Boerhaave syndrome) is a relatively rare entity among digestive tract emergencies.¹ Because Boerhaave syndrome has a high rate of mortality, few reports describe the late complications of the syndrome. Further, recurrence of the syndrome is extremely rare, with only 7 cases having been reported in the English literature.^2–7 In addition, the pathophysiologic mechanisms responsible for Boerhaave syndrome have not been clearly determined.Antiphospholipid syndrome (APS) is a type of autoimmune syndrome and is known to cause a variety of complications, which are primarily caused by vascular disorders. In the present report we describe a case of recurrent Boerhaave syndrome associated with APS, and we discuss the pathogenesis of spontaneous esophageal ruptures.