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Antiphospholipid syndrome is often associated with systemic lupus erythematosus. Both syndromes have different clinical manifestations based on organ involvement. Antiphospholipid syndrome commonly causes spontaneous abortions, cerebral vascular occlusion, and deep venous thrombosis. Catastrophic antiphospholipid syndrome occurs when three or more...
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... 20-year-old African American woman presented to the emergency center with abdominal pain, nausea, and vomiting. Her medical history is significant for systemic lupus erythematosus and antiphospholipid syndrome. Systemic lupus erythematosus was diagnosed four years prior to presentation on the basis of fever, malar rash, arthralgias, and alopecia. The patient also has hypertension and chronic kidney disease with a baseline creatinine of 3 mg/dL (reference range: 0.8-1.5 mg/dL) secondary to her underlying disease. Antiphospholipid syndrome was diagnosed one year prior to admission when she presented with a deep venous thrombosis. She has been placed on subcutaneous low-molecular weight heparin for anticoagulation for the past year, but she has not been fully compliant. Two months prior to admission, the patient was hospitalized for acute pancreatitis. For a differential diagnosis of pancreatitis see Table 1. During her hospital course, she developed methicillin-resistant Staphylococcus aureus bacteremia, acute renal failure, and a right brachial deep venous thrombosis. Renal biopsy confirmed lupus nephritis with microangiopathic disease (Figure 1). She was discharged after re-initiation of anticoagulation with subcutaneous low-molecular weight heparin and warfarin and being apparently asymptomatic. Upon current presentation, her abdominal pain was localized to the epigastric and periumbilical regions without radiation. The pain was sharp, stabbing, and intermittent. She had vomited every other day for two weeks prior to admission, with her vomitus being non- bloody and consisting primarily of food contents. She also had diarrhea with three non-bloody, watery bowel movements the day prior to admission. Her blood pressure was elevated at 157/110 mmHg. Her laboratory data revealed lipase was 231 U/L (reference range: 6-51 U/L); amylase was 159 U/L (reference range: 30-110 U/L); anti-nuclear antibody was positive; Anti DNA titer was 1:80 (negative if less than 1:40) and equal to, or higher than, 1/2,560 SSA/Ro (positive if higher than 1/8); IgG level was normal (670 mg/dL; reference range: 751-1,560 mg/dL); C3 was 79 mg/dL (reference range: 79-152 mg/dL); C4 was 22 mg/dL (reference range: 16-38 mg/dL). Duplex ultrasound of the abdomen showed no biliary dilatation, no calculi or wall thickening in the gallbladder, negative sonographic Murphy's sign, and no portal vein or splenic vein thrombosis. A non- contrast CT of the abdomen and pelvis after her admission was limited, but showed inflammatory changes around the pancreas with small amounts of peripancreatic fluid, thickening of the stomach wall, and free fluid in the pelvis, all consistent with pancreatitis ( Figure 2). She was also found to have worsening kidney function with a creatinine of 5.3 mg/dL. APACHE II score was calculated to be 22, indicating 28.6% mortality. SLEDAI (systemic lupus erythematosus severity index) was calculated to be 29. The patient was treated as presumed catastrophic antiphospholipid syndrome. Initial treatment included six sessions of plasmapheresis without intravenous immunoglobulin to avoid further kidney damage, and one dose of cyclophosphamide. After six sessions of plasmapheresis, the patient showed significant clinical improvement with resolution of her abdominal pain. Kidney function also improved with creatinine returning to baseline. Her blood pressure was difficult control, but eventually stabilized on a combination of a beta blocker, a calcium channel blocker, and a loop diuretic. The patient was discharged 4 weeks after initial presentation on prednisone 30 mg once per day, metoclopramide 5 mg every 6 hours, clonidine 1 patch per week, nifedipine 60 mg twice per day, and hydroxychloroquine 400 mg once per day. A repeat abdominal CT scan 5 week after the acute event showed complete resolution of pancreatitis ( Figure ...
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Osteonecrosis is commonly present in patients with antiphospholipid syndrome (APS) and systemic lupus erythematosus (SLE). Treatment of this condition remains extremely controversial. We present a treatment strategy of avascular necrosis of the knee in a patient with catastrophic antiphospholipid syndrome with a history of SLE and APS. Aggressive t...
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... Це може бути специфічний вовчаковий (аутоімунний) гострий або хронічний панкреатит, лікарський панкреатит (викликаний кортикостероїдами, азатіоприном, 6-меркаптопурином або тіазидними діуретиками), вірусний панкреатит (у тому числі цитомегаловірусний), банальний панкреатит (алкогольний, біліарний та ін.). Можливі інфаркти ПЗ внаслідок тромбозів у рамках антифосфоліпідного синдрому [20]. ...
The purpose of the study was to evaluate the main pathogenetic mechanisms of pancreatic lesions on the background of systemic connective tissue diseases based on the analytical analysis of modern literature data. Materials and methods. Bibliosemantic and analytical methods were used in the research. Results and discussion. The main pathogenetic link of the pancreatitis development in patients with systemic connective tissue diseases is vasculitis of the pancreas. Increased expression of adhesion molecules with activation of leukocytes and endothelial cells, deposition of circulating immune complexes in the vascular wall, production of antibodies to endothelial cells, capillary basement membranes play an important role in this process. In systemic lupus erythematosus, according to various authors, the frequency of arteritis varies greatly: rates range from 6.2-7.4 to 53%. In rheumatoid arthritis, the frequency of arteritis of the pancreatic vessels reaches 50%, in systemic sclerosis – 17%. Secondary Sjogren's syndrome is associated with autoimmune pancreatitis in a quarter of cases, but is not the cause. In diseases such as rheumatoid arthritis, systemic scleroderma and systemic lupus erythematosus, antibodies that can attack phospholipids of cell membranes are produced. Antiphospholipid syndrome develops often in systemic lupus erythematosus (70% of cases). In rheumatic fever patients’ changes in the pancreas were studied only in single studies. The main mechanism of pathogenesis of both acute and chronic pancreatitis in nodular periarteritis is the involvement of small and medium arteries of the pancreas in the pathological process. In granulomatous polyangiitis in the pancreas reveals vascular-granulomatous changes, resulting in the formation of extravasations, necrotic foci, foci of atrophy, sclerosis. In IgA vasculitis, changes in the structure of the pancreas are minimal or there are isolated small subcapsular hemorrhages. It is established that metabolic disorders occur in many rheumatic diseases. Thus, reduced glucose tolerance is observed in 7–74% of patients, hypercholesterolemia and triglyceridemia – in 50–75%, hypertension – in 25–50% of cases. Conclusion. Thus, the diagnostic approach to the pancreatitis in systemic connective tissue diseases is very difficult. Its manifestations are masked by damage of the other organs. The availability of more sensitive diagnostic methods, their accessibility can provide an opportunity to detect symptoms of pancreatitis earlier, which will contribute to the appointment of optimal treatment, improvement of the prognosis, quality of life and survival of such patients
... A total of 10 APS related pancreatic lesions have been reported to date [24,[103][104][105][106][107][108][109][110][111]. Pancreatitis was the main manifestation in 8 cases and pancreatic duct injury in the other 2 cases. ...
Antiphospholipid syndrome (APS) is an autoimmune disease mainly characterised by vascular thrombosis and pregnancy morbidity. APS has broad spectrum of clinical manifestations. The digestive system involvement of antiphospholipid syndrome is a critical but under-recognised condition. Digestive system involvement may be the result of direct (autoimmune-mediated) or indirect (thrombotic) mechanisms. Liver is the most commonly involved organ, followed by intestines, oesophagus, stomach, pancreas and spleen. This review describes possible digestive system manifestations in APS patients, and illustrates the epidemiology and possible pathophysiology of APS. The role of different treatment strategies in the management of digestive system manifestations of APS were also discussed.
Key messages
Antiphospholipid syndrome is a multi-organ, multi-system disease and its clinical manifestation spectrum is gradually expanding. Since the first diagnosis of APS, the clinical manifestations of digestive system have been reported successively. This narrative review describes the major digestive system manifestations of APS and illustrates the epidemiology, pathophysiology and the role of therapeutic strategies of these patients.
... APS may occur isolated or in association with rheumatic diseases, mainly systemic lupus erythematosus (SLE). aPL antibodies are prevalent in the general population ranging from 1-5% of healthy subjects and are much more common in SLE patients ranging between 30 and 40% [7]. Sarcoidosis is diagnosed by a combined clinical, laboratory, radiological and histopathological findings [8,9]. ...
Background: Sarcoidosis is a clinically heterogenous disease of unknown etiology with a hallmark of the development and accumulation of non-caseating granulomas in any organ. The antiphospholipid syndrome (APS) is an autoimmune disorder characterized by an elevated risk for arterial and venous thrombosis and pregnancy-related morbidity. Celiac disease (CD) is a chronic, immune-mediated form of enteropathy that is now presenting later in life and often with extraintestinal manifestations. Co-existence of sarcoidosis, CD and APS is extremely rare.
Case presentation: We describe a 27-year-old Tunisian woman with a history of non-explored superficial vein thrombosis and 4 successive miscarriages, who was explored for abdominal pain, vomiting, hypercalcemia, hepatic cytolyses and cholestasis and was complicated later with pancreatitis and anterior uveitis. Hepatic biopsy revealed noncaseating-granulomas, a high serum angiotensin converting enzyme activity was detected and the diagnosis of sarcoidosis was considered. A high titer of antiphopsholipid antibodies concluded the diagnosis of APS. Duodenal biopsies showed a total villous atrophy indicative of CD, confirmed by positivity of serum anti-endomisium and anti-transglutaminase antibodies. She had normocytic anemia (hemoglobin 9 g/dl) and elevated transaminases. Thorough investigation established the diagnosis of extra pulmonary sarcoidosis associated with CD and APS.
Conclusion: Co-existence of sarcoidosis, CD and APS is extremely rare. APS should be recognized as an accompanying disorder of sarcoidosis and antiphospholipids measured especially when there is a history of thrombosis or miscarriages. CD should not be overlooked in association to sarcoidosis, given the shared immunological and genetic background, even in the absence of a typical presentation of the disease.
... Ischemia may result from vasculitis (polyarteritis nodosa, systemis lupus erythematosus), atheromatous embolism of cholesterol plaques from the aorta after angiography, intraoperative hypotension, hemorrhagic shock, ergotamine overdose, cocaine use, transcatheter arterial embolization for hepatocellular carcinoma, or liver metastases; hypercoagulable disorders (antiphospholipid antibodies, factor V Leiden mutation [75][76][77][78]. ...
Acute pancreatitis is a common disease with a benign course in the majority of patients, but it is associated with serious morbidity, and mortality close to 20% in up to 20% of cases. The incidence of acute pancreatitis seems to be rising in western countries. About 75% of all cases are caused by gallstones or alcoholism. The relative rate of gallstones versus alcohol as etiology depends on the age and the area of enrolment. A thorough evaluation allows cause identification in another 10% of cases, leaving about 15–20% as idiopathic. The most common causes, and a growing list of less frequent and sometimes very rare and controversial etiologies, are reviewed in this article. A classification on the pathogenic mechanisms of causes of acute pancreatitis based is used in this Review. The approach, or suggested plan of investigations, to determine the etiology of acute pancreatitis, based on the most recently published Guidelines is outlined.
... In most cases it is mild, but fatal necrotizing pancreatitis may happen. Ischemia may result from vasculitis (polyarteritis nodosa, systemis lupus erythematosus), atheromatous embolism of cholesterol plaques from the aorta after angiography, intraoperative hypotension, hemorrhagic shock, ergotamine overdose, cocaine use, transcatheter arterial embolization for hepatocellular carcinoma, or liver metastases; hypercoagulable disorders (antiphospholipid antibodies, factor V Leiden mutation [75][76][77][78]. Ischemia may be an explanation for pancreatitis after cardiopulmonary bypass [79,80]. ...
Acute pancreatitis (AP) is a local inflammatory response with systemic effects and an adverse evolution in 20% of cases. Its mortality rate is 5–10% in sterile and 15–40% in infected pancreatic necrosis. Infection is widely accepted as the main reason of death in AP. The evidence to enable a recommendation about antibiotic prophylaxis against infection of pancreatic necrosis is conflicting and difficult to interpret. Up to date, there is no evidence that supports the routine use of antibiotic prophylaxis in patients with severe AP. Treatment on demand seems to be the better option, avoiding excessive treatment and selection of bacterial. In infected acute pancreatitis, antibiotics of choice are imipenem, meronem or tigecycline in patients allergic to beta-lactams. Also fluconazole must be given in determinate clinical situations.
... 1 CAPS is an important clinical entity to consider due to mortality rates up to 50%. 2 Patients with CAPS and SLE are more likely to be female and young, to have cerebral and pancreatic involvement, to receive corticosteroids and cyclophosphamide, to demonstrate lower prevalence of high anti-cardiolipin immunoglobulin G antibody, and to have a higher risk of death compared with other patients with APL syndrome who do not develop CAPS after adjusting for age, sex, organ involvement, and treatment. 2 In a review of 250 cases of CAPS, the main cause of death was found to be cerebral involvement (27.2%) followed by cardiac involvement (19.8%) and infection (19.8%). 3 The kidney was the organ most involved (70-78 % of the cases) 3,4 Patients with CAPS and SLE who is treated with a combination of plasmapheresis and corticosteroids have a survival rate of 65%. ...
... However, the use of these alternate modalities has been limited to a few small case series. 2 Awareness of this rare and rapidly fatal medical condition is vital for prompt diagnosis and early intervention to improve patients' survival. Our patient had a severe form of multi-organ failure and SLE-like syndrome. ...
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.
Acute pancreatitis is a potentially life-threatening complication of severe hypertriglyceridemia. In some cases, inborn errors of metabolism such as lipoprotein lipase deficiency, apoprotein C-II deficiency, and familial hypertriglyceridemia have been reported as causes of severe hypertriglyceridemia. More often, severe hypertriglyceridemia describes various clinical conditions characterized by high plasma levels of triglycerides (>1000 mg/dL), chylomicron remnants, or intermediate density lipoprotein like particles, and/or chylomicrons. International guidelines on the management of acute pancreatitis are currently available. Standard therapeutic measures are based on the use of lipid-lowering agents (fenofibrate, gemfibrozil, niacin, Ω-3 fatty acids), low molecular weight heparin, and insulin in diabetic patients. However, when standard medical therapies have failed, non-pharmacological approaches based upon the removal of triglycerides with therapeutic plasma exchange can also provide benefit to patients with severe hypertriglyceridemia and acute pancreatitis. Plasma exchange could be very helpful in reducing triglycerides levels during the acute phase of hyperlipidemic pancreatitis, and in the prevention of recurrence. The current evidence on management of acute pancreatitis and severe hypertriglyceridemia, focusing on symptoms, treatment and potential complications is reviewed herein.
Detection of antiphospholipid antibodies (aPL) are central to the definition of the antiphospholipid (antibody) syndrome (APS). Thrombosis in certain vascular beds, such as the cerebral circulation, the veins of the lower legs and cutaneous vessels, and/or fetal loss, are common manifestations of APS. However, aPL have been found in association with a large range of other clinical conditions-these conditions constitute a rather heterogeneous group and are the subject of this review. Thus, aPL may rarely be found in association with thromboses of vascular beds other than those commonly associated with APS. The combination of thrombosis and aPL still satisfies the criteria for APS, and management of this group of patients is the same as that of APS associated with the more common manifestations of the disease. Alternatively, aPL may be detected in a range of conditions where thrombosis cannot be clearly demonstrated, such as duodenal ulcer or transverse myelopathy. The approach to management of patients who have aPL in association with these conditions is less clear, although in some cases interventions to remove the associated antibody have been associated with amelioration of the condition. Finally, in several studies, aPL have been detected in a proportion of patients with conditions occurring commonly in the normal population-these findings have to be treated with caution in view of inconsistent findings between the reported results and methodological limitation of studies purporting to show positive results.
