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To describe the clinical and immunologic characteristics of patients with adrenal involvement and antiphospholipid syndrome (APS), we conducted a computer-assisted (PubMed) search of the literature to identify all cases of primary adrenal insufficiency associated with antiphospholipid antibodies published in English, French, and Spanish from 1983 (when APS was first defined) through March 2002. We reviewed 86 patients (80 from the literature plus 6 from our cohort); 55% were male, and the mean age at presentation was 43 +/- 16 years. Sixty-one (71%) patients had primary APS, and 14 (16%) had systemic lupus erythematosus. In 31 (36%) patients, adrenal insufficiency was the first clinical manifestation of APS. Abdominal pain was present in 55% of patients, followed by hypotension (54%), fever (40%), nausea or vomiting (31%), weakness or fatigue (31%), and lethargy or altered mental status (19%). The main finding in imaging techniques was compatible with adrenal hemorrhage (59%) and in histopathologic study was a hemorrhagic infarction with vessel thrombosis (55%). Lupus anticoagulant was detected in 97% of patients and the anticardiolipin antibodies titer was positive in 93% of patients. Most patients (95%) were positive for the IgG isotype of anticardiolipin antibodies, whereas 40% were positive for the IgM isotype. Baseline cortisol levels were decreased in 98% of patients, ACTH hormone levels were increased in 96% of patients, and the cosyntropin stimulation test was positive in 100% of patients tested. Steroid replacement therapy was the most frequent treatment (84%), followed by anticoagulation (52%) and aspirin (6%). Thirty-two of 35 (91%) patients with prolonged anticoagulant therapy were in good health with a mean follow-up of 25 months, whereas 25 of the 69 (36%) patients with outcome data available had died. The results of the present review stress the clinical importance of systematic screening for lupus anticoagulant and anticardiolipin antibodies in all cases of adrenal hemorrhage or infarction. An initial screening for hypoadrenalism is mandatory in any antiphospholipid antibody-positive patient who complains of abdominal pain and undue weakness or asthenia.
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PMID:Adrenal involvement in the antiphospholipid syndrome: clinical and immunologic characteristics of 86 patients. 1264 Jan 87

The authors report a case of a 56-year-old Thai woman with a history of recurrent venous thrombosis, spontaneous abortion and Graves' disease who presented with bilateral flank pain, nausea, vomiting and low-grade fever followed by hypotension. Adrenal crisis from bilateral adrenal hemorrhage was diagnosed by a low serum cortisol level during hypotension and bilateral hyperdense oval masses in each of the adrenal glands in a computerized tomographic study. Her hemostatic and serologic profile was compatible with primary antiphospholipid syndrome. Rapid improvement was observed after the administration of intravenous hydrocortisone. She was discharged on long-term glucocorticoid replacement for her primary adrenal insufficiency as well as an anticoagulant for prevention of thrombosis. The antiphospholipid syndrome should be suspected in a patient presenting with adrenal crisis without a distinct etiology.
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PMID:Adrenal crisis due to bilateral adrenal hemorrhage in primary antiphospholipid syndrome. 1614 61

Anti Phospholipid Syndrome (APS) is a relatively new conception of syndrome complex first noticed in 1983. It may be primary or secondary to other diseases like SLE, RA, Systemic sclerosis, behchet's syndrome, temporal arteritis, sjogren's syndrome psoriatic arthropathy etc. Clinical manifestations are consequences of vascular thrombosis and embolism like DVT, pulmonary embolism, stroke, TIA, complication of pregnancy with pregnancy loss. We report a 34 years married female housewife who presented with sudden onset of nausea, vomiting, vertigo, dysphagia, dysarthria and ataxia. She had a chronic leg ulcer. Neurological findings were consistent with lateral medullary syndrome due to stroke though she was normotensive, nondiabetic with normal lipid profile. She had history of two abortions in last three years. Investigations were done accordingly and she fulfilled the diagnostic criteria of APS. No secondary cause was detected after thorough clinical examination and laboratory investigations. She was treated symptomatically along with oral anticoagulation. She improved slowly but steadily.
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PMID:Anti phospholipid syndrome. 1918 54

The antiphospholipid antibody syndrome is the most common acquired thrombophilia; it is a systemic autoimmune disease characterized by recurrent arterial and venous thrombosis and/or pregnancy loss, in association with circulating antiphospholipid antibodies. The pathogenic mechanisms in antiphospholipid antibody syndrome that lead to in vivo injury are incompletely understood. Like other autoimmune diseases, a combination of genetic and environmental factors is involved. We report the case of a 50-year-old woman suffering from an antero-lateral non-ST-elevation myocardial infarction. After few days, coronary angiography showed a severe occlusive arterial disease, involving anterior descending, circumflex e right coronary arteries. Percutaneous coronary intervention was performed with the implantation of a drug-eluting stent in the proximal segment of the anterior descending coronary artery. One day after discharge (10 days after the first hospitalization) the patient experienced dizziness, nausea, vomiting, swelling in absence of any electrocardiographic abnormalities or myocardial enzyme elevation; then she was hospitalized in the neurology department. Because of a similar episode, urgent cerebral computed tomography scan was performed 5 days later; it revealed two different acute ischemic areas, parietal in the right hemisphere and cerebellar in the left hemisphere. The diagnosis of antiphospholipid antibody syndrome was confirmed by high anticardiolipin antibody titers, also present in medium titer at 5 and 17 weeks apart. She was discharged without any sequelae, on warfarin and double antiplatelet therapy (aspirin and clopidogrel for 6 months), then warfarin and aspirin.
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PMID:[Myocardial and cerebral infarction as initial presentation of antiphospholipid syndrome]. 1947 82

A 33-year-old male with systemic lupus erythematosus (SLE) presented with acute abdominal pain and was found to have lupus mesenteric vasculitis on imaging and during exploratory laparotomy. Post laparotomy he continued to have persistent nausea and dyspepsia and an electrocardiogram showed evidence of an inferior ST elevation myocardial infarction (STEMI). Emergency cardiac catheterization showed evidence of thrombotic right coronary artery occlusion. His coronaries were otherwise normal with no evidence of underlying coronary artery disease. Extensive workup with trans-esophageal echo, serologies for antiphospholipid antibody syndrome (APS) and bubble study was negative. This effectively ruled out Libman-Sacks endocarditis, APS-induced arterial thrombus and paradoxical emboli as potential causes of his STEMI. By exclusion of other causes, the etiology of his STEMI was felt to be secondary to in-situ coronary artery thrombosis in the setting of active SLE. To the best of our knowledge, this is the first report of a patient with SLE presenting with both lupus mesenteric vasculitis and in-situ coronary arterial thrombosis in the absence of APS.
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PMID:Spontaneous coronary artery thrombosis in the setting of active lupus mesenteric vasculitis. 2567 73

Stomach's output part and small intestine, combining with damaging of the urinary tract is a rare systemic lupus eritematosus (SLE) manifestation. The patient is 32 years old, suffering from SLE with damaged join, blood system, secondary antiphospholipid syndrome with pulmonary embolism in the history and formation of high postembolic pulmonary hypertension on therapy with hydroxychloroquine and low doses of corticosteroids, was hospitalized because of persistent nausea, vomiting, loss of more than 10 kg body weight 1.5 months. The research have shown the obstruction's formation of the stomach's output part, small bowel obstruction at several levels, as well as thickening of the bladder wall and the unilateral expansion of the ureter. Against the backdrop of strengthening of immunosuppressive therapy these lesions completely regressed.
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PMID:Clinical observation pseudoobstruction syndrome of the stomach's output part and small intestine of a patient with systematic lupus erythematosis. 3070 83

New treatment options constitute unmet needs for patients diagnosed with systemic lupus erythematosus (SLE). Inhibition of the mammalian target of rapamycin (mTOR) pathway by sirolimus, a drug approved and in clinical use to prevent transplant rejection, has shown promising effects in lupus animal models as well as in patients with both antiphospholipid syndrome and SLE. Sirolimus inhibits antigen-induced T cell proliferation and increases the number of circulating regulatory T cells. Recently, sirolimus was tested in an open label phase 1/2 trial, including 43 patients with active SLE, resistant or intolerant to conventional medications. The results were encouraging showing a progressive improvement, including mucocutaneous and musculoskeletal manifestations. At our university unit, we have more than 16 years' experience of sirolimus as treatment for non-renal manifestations of SLE. Herein, we retrospectively evaluated data on tolerance, dosage, affected organ systems, disease activity measures, corticosteroid reduction, concomitant immunosuppressive therapies, and patient-reported outcome measures (PROMs) such as pain intensity, fatigue, well-being and quality-of-life (QoL) in 27 Caucasian patients with mildly active SLE. Musculoskeletal manifestation was the main reason for sirolimus treatment followed by skin involvement and leukocytopenia. Mean time on sirolimus was 47.1 (range 2-140) months. Decreasing global disease activity was observed, as measured by the clinical SLE disease activity index-2000, with a mean reduction of 2.5 points (range -10 to 0) and a corresponding mean reduction of the physician's global assessment (0-4) of 0.64 (range -2 to 0). The mean daily dose of corticosteroids (prednisolone) was reduced by 3.3 mg (-12.5 to 0). Non-significant trends toward improvements of QoL and pain intensity were found. Serious side-effects were not seen during sirolimus treatment, but early withdrawal due to nausea (n = 4) and non-serious infections (n = 2) appeared. This observational study, including longtime real-life use of sirolimus in SLE, is the largest to date and it essentially confirms the results of the recent phase 1/2 trial. Our data indicate that sirolimus is efficient in patients with musculoskeletal SLE manifestations, particularly arthritis and tendinitis. Further randomized controlled trials evaluating the potential benefits of sirolimus in SLE are warranted, but should aim to enroll patients with shorter disease duration, less accrued damage, and more diverse ethnicities.
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PMID:Clinical Experience of Sirolimus Regarding Efficacy and Safety in Systemic Lupus Erythematosus. 3078 78

Catastrophic antiphospholipid syndrome (CAPS) is a rare but severe form of antiphospholipid syndrome (APS). The syndrome manifests itself as a rapidly progressive multiorgan failure that is believed to be caused by widespread micro-thrombosis. Seldom does bleeding comanifest with thrombosis. We present a patient with APS who presented with nausea, vomiting and fatigue, and rapidly progressed into multiorgan failure before being diagnosed with CAPS. The clinical course was complicated by an atraumatic intracranial haemorrhage which demanded discontinuation of anticoagulation. The patient was treated with high dose steroid, intravenous immunoglobulin, followed by weekly rituximab infusion. Although the trigger for CAPS was not obvious during her hospital stay, she was diagnosed with acute cytomegalovirus (CMV) infection soon after discharge. In this case report, we explore the differential diagnoses of CAPS, investigate the possibility of CMV infection as a potential trigger, present the therapeutic challenges of anticoagulation and discuss the emerging use of rituximab.
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PMID:Rare case of catastrophic antiphospholipid syndrome with spontaneous intracranial haemorrhage. 3089 56

Thrombophlebitis of the portal vein (pylephlebitis) is a rare but serious condition with a high mortality rate of 11-50%. A 56-year-old male patient presented with a two-day history of postprandial, colic-like epigastric pain, nausea, fever, chills, and diarrhea. Clinical workup showed peritonism, leukocytosis, and elevated C-reactive protein (CRP). A computed tomography (CT) scan revealed a long-segment, partial thrombosis of the superior mesenteric vein as well as gas in the portal venous system. Additionally, extensive jejunal diverticulosis was present. Pylephlebitis mostly results from intestinal infections, e.g., appendicitis or diverticulitis. We assumed that the patient had suffered from a self-limiting episode of jejunal diverticulitis leading to septic thrombosis. Initially, antibiotic therapy and anticoagulation with heparin were administered. The patient deteriorated, and due to increasing abdominal defense, fever, and hypotension, a diagnostic laparoscopy was performed. Bowel ischemia could be ruled out, and after changing antibiotic therapy, the patient's condition improved. He was discharged without any further complications and without complaints on day 13. An underlying coagulopathy like myeloproliferative neoplasm or antiphospholipid syndrome could be ruled out.
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PMID:Jejunal Diverticulosis Probably Leading to Pylephlebitis of the Superior Mesenteric Vein. 3301 5