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Query: UMLS:C0042963 (vomiting)
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The clinical picture of venous or arterial thrombosis in the presence of circulating antiphospholipid antibodies is referred to as the antiphospholipid syndrome. A 5-month-old baby girl who was quite healthy so far was referred to our clinic with irritability, vomiting, and abdominal distension for 30 hours. Surgical exploration exposed a gangrenous ileal segment about 15 cm long. The postoperative period was unremarkable. Investigation to identify the risk factors for mesenteric thrombosis found anticardiolipin antibodies (isotype Ig G) and decreased protein C level. Protein S and antithrombin III were within normal levels. Hb electrophoresis results showed no HbS, and neither Factor V Leiden nor prothrombin 20210 mutations were detected. Eight months postoperatively, anticardiolipin antibodies were found within normal levels. Lupus anticoagulant, ds DNA, and ss DNA were negative. Direct coombs test and protein C, C3, and C4 were also within normal levels. She had no thrombotic episode in the 24 months postoperatively, although no anticoagulant medication was administered. To the authors' knowledge this case is the first report of segmental intestinal infarction in transient antiphospholipid syndrome in the pediatric population.
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PMID:Transient antiphospholipid syndrome in an infant with segmental small bowel infarction. 1469 90

In patients with systemic lupus erythematosus(SLE), interstitial cystitis(lupus cystitis) is an uncommon, but important manifestation. We report two Japanese patients with lupus cystitis. Case 1 was a 49-year-old woman diagnosed as having rheumatoid arthritis and membranous nephropathy. She was treated with prednisolone(5 mg daily). Case 2 was a 41-year-old woman also diagnosed as having rheumatoid arthritis previously and treated with a non-steroidal anti-inflammatory drug. Both cases presented abdominal pain, vomiting, dysuria and frequency of micturition. We diagnosed these cases as SLE on the basis of arthritis, renal disorder(proteinuria and hematuria), and positive antinuclear and anti-dsDNA antibodies. In addition, bilateral hydronephrosis was found in both cases. Thus, they were also diagnosed as probable lupus cystitis. The patients were treated with one cycle of methylprednisolone pulse therapy. Thereafter they were treated with 60 mg/day of prednisolone and their symptoms resolved promptly. Furthermore, no abnormal finding was found by abdominal ultrasonography and/or the intravenous pyelogram after therapy. Renal biopsies were performed and both cases showed lupus glomerulopathy (case 1: WHO class Vb, case II: WHO class IVb). Abdominal pain and/or dysuria, which is common in SLE patients, requires further examinations to evaluate the lupus cystitis.
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PMID:[Two cases of lupus cystitis complicated by lupus nephritis treated successfully with steroid therapy]. 1473 94

Lupus cystitis was rare but frequently resulted in obstructive uropathy and had a strong association with gastrointestinal (GI) symptoms. We treated six patients with systemic lupus erythematosus (SLE) and obstructive uropathy from January 1996 to December 2001 in a university hospital. Evidence of cystitis was obtained from cystoscopic biopsy or the presence of thickened bladder wall in image study. Similar to other reports, five patients had GI manifestations such as abdominal pain, nausea/vomiting, diarrhoea or ileus. In addition, mesenteric lymphadenopathy or pancreatitis was noted in three patients. Two patients had been treated for idiopathic thrombocytopenic purpura (ITP), four and 20 years ago, respectively. All six patients had antibodies to double-stranded DNA (dsDNA). Five patients each had antibodies to cardiolipin (IgG aCL) or SSA. The high prevalence of anti-SSA had also been reported in Chinese lupus patients with intestinal pseudo-obstruction, a clinical manifestation frequently associated with bilateral ureterohydronephrosis. Two patients died of intractable infection after the surgical procedures for persistent ureterohydronephrosis and both patients had antibodies to ribosomal P proteins. Lupus cystitis might not be so rare in Chinese patients with SLE. The diagnosis should be kept in mind when lupus patients have urinary and/or GI symptoms.
Lupus 2004
PMID:Is there an ethnic difference in the prevalence of lupus cystitis? A report of six cases. 1517 63

Intravenous immunoglobulins (IVIgs) exert a variety of immunomodulating activities and are, therefore, increasingly being used for the treatment of immune-mediated as well as autoimmune diseases. There is also accumulating evidence that high-dose IVIg (hdIVIg) is highly efficacious in the treatment of skin diseases, despite the lack of evidence from randomized, double-blind, placebo-controlled trials. A major advantage of hdIVIg in comparison with other commonly used immunomodulating therapeutic strategies is the excellent safety profile. Accordingly, IVIgs have been used successfully for the treatment of bullous autoimmune diseases such as pemphigus and bullous pemphigoid, dermatomyositis, scleroderma, cutaneous lupus erythematosus, toxic epidermal necrolysis, and erythema exudativum multiforme. In most cases, hdIVIg is effective only in combination with other immunomodulating strategies and allows for the reduction of adjuvants. Adverse effects of hdIVIg are generally mild and self-limiting. These include headache, myalgia, flush, fever, nausea or vomiting, chills, lower backache, changes in blood pressure, and tachycardia. To avoid infusion-related rigors, headaches, and other adverse events, pre-treatment with analgesics, NSAIDs, antihistamines, or low-dose intravenous corticosteroids may be beneficial. Controlled, double-blind, long-term clinical trials and a better understanding of the complex immunomodulating mechanism of IVIg are required to ultimately optimize dose, frequency, duration, and mode of IVIg administration.
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PMID:Efficacy and safety of intravenous immunoglobulin for immune-mediated skin disease: current view. 1518 94

A case of West Nile virus (WNV) infection in a captive 4-month-old Arctic wolf (Canis lupus) is described. The animal had vomiting, anorexia, and ataxia before death. Histopathology revealed multifocal severe renal lymphoplasmacytic vasculitis, mostly affecting small arterioles, with fibrinoid degeneration of some vessel walls. Many small foci of gliosis were detected in the cerebral cortex. West Nile virus was demonstrated in the kidneys and cerebrum by immunohistochemistry and polymerase chain reaction. The described renal changes represent a novel pathological finding of WNV infection.
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PMID:Natural West Nile virus infection in a captive juvenile Arctic wolf (Canis lupus). 1530 45

We report the case of a 66-year-old woman with diabetes mellitus and disseminated lupus treated with immunosupressive drug. She was admitted for an inflammatory oedema of the right lower limb associated to diffuse abdominal pain and vomiting. The occurrence of septic shock with multiple organ failure and surgical abdominal picture led us to perform urgent laparotomy without taking in account the evolutive signs of cutaneous lesions i.e. purpuric elements and haemorrhagic phlycthena. The abdominal cavity exploration showed peritonitis without digestive tracts perforation. The patient died few hours after surgical procedure. All bacteriological samples i.e. peritoneal effusion, blood cultures and phlycthena liquid were positive for Streptocoque pyogenes (group A) and peritonitis was considered to be related to haematogen diffusion from rapid course necrotizing fasciitis.
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PMID:[Secondary Streptococcus pyogenes peritonitis following necrotizing fasciitis]. 1532 64

A 39-year-old white woman presented with a history of aortoiliac occlusive disease diagnosed in 1992 attributed to oral contraceptive use. Shortly thereafter, aortoiliac replacement was performed. Mild hyperlipidemia was diagnosed in 2001. At the current clinic visit, she presented to her primary care physician with a 3-month history of postprandial midepigastric abdominal pain relieved by vomiting and a 30-pound weight loss. Her evaluation included an esophagogastroduodenoscopy, a colonoscopy, and an abdominal ultrasound, all of which were within normal limits. Because of her medical history, the patient underwent an arteriogram, which revealed brachiocephalic stenosis (Figure 1), occlusion of the left subclavian artery (Figures 2a and 2b), and narrowing of the superior and inferior mesenteric arteries (not shown). Since she had discontinued her oral contraceptives in 1992 and her hyperlipidemia was mild, the rheumatology service was consulted to evaluate this patient. On physical examination, she had decreased left brachial and radial pulses and a right carotid bruit. Laboratory evaluation revealed a normal complete blood count, comprehensive metabolic panel, erythrocyte sedimentation rate, and C - reactive protein. Subsequent testing included a prothrombin time, activated partial thromboplastin time, protein S, protein C, reptilase time, antithrombin III, anticardiolipin antibody, antiphospholipid antibody, lupus anticoagulant, homocysteine, RPR, and a lipid profile. All test results were within normal limits. Due to the severity of her abdominal pain, the patient underwent superior mesenteric artery (SMA) bypass surgery. Sections from the aorta resected in 1992 are shown in Figures 3 and 4.
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PMID:Pathology case of the month. 39-year-old woman with abdominal pain and weight loss. Takayasu's arteritis (TA). 1555 91

Intestinal pseudo-obstruction (IPO) is a rare complication of systemic lupus erythematosus (SLE). We present a 32-year old female with SLE for seven years. She was admitted with profound fatigue, frequent vomiting, colicky abdominal pain, diarrhoea and intermittent dysuria for the past 12 months. Imaging studies revealed dilated small and large bowel loops with thickened intestinal wall and multiple fluid levels. Urinary tract involvement was also demonstrated. The patient responded well to immunosuppressive treatment. IPO in the context of SLE has been described only in anecdotal case reports. Half of the cases developed this complication during the course of lupus as in the present case. Concomitant ureterohydronephrosis was present in approximately two-thirds of the cases. Early recognition of the syndrome is necessary for the institution of the appropriate medical treatment and prevention of inappropriate surgical intervention.
Lupus 2004
PMID:Intestinal pseudo-obstruction and ureterohydronephrosis as the presenting manifestations of relapse in a lupus patient. 1564 52

We report a case of subcutaneous panniculitis-like T-cell lymphoma (SPTCL), associated with macrophage activation syndrome, mimicking a lupus erythematosus panniculitis (LEP). A 29-year-old woman presented with high fever, general malaise, nausea, vomiting, and subcutaneous nodules and ulcerating lesions located on the lower extremities. The histopathology showed an infiltration of the panniculus, mostly involving fat, and periadnexial and perivascular structures consistent with lymphocytic lobular panniculitis (LLP). LLP is a shared feature of LEP and SPTCL. The immunophenotyping of the cell infiltrate was crucial for a correct diagnosis.
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PMID:Subcutaneous panniculitis-like T-cell lymphoma misdiagnosed as lupus erythematosus panniculitis. 1640 99

The patient was a 13-year-old girl. In August 2000, she presented with a fever, together with diarrhea, vomiting, arthralgia, nasal bleeding and malaise, and was examined by another physician. Because her platelet count was low, and there were positive reactions for anti-nuclear antibodies, anti-DNA antibodies and platelet-associated IgG, idiopathic thrombopenic purpura, and systemic lupus erythematosus (SLE) was suspected. From January 2001, when she caught measles, she reported abdominal pain, and urinalysis indicated urinary protein and occult blood, and the left kidney was found hydronephrotic. At the same time left ureter stenosis and dilatation were demonstrated. Symptoms were disappeared by hydration and treatment with NSAIDs, but 2 months later fever and erythematous patches seen on both cheeks led to the proper diagnosis of SLE, and she was admitted to our hospital. Intravenous pyelography revealed hydronephrosis on left kidney, constriction and dilatation of the left ureter, and intracystic endoscopy showed erythema at the orifice of the left ureter. The pathological examination indicated the presence of vasculitis, and finally lupus cystitis was diagnosed. Intravenous cyclophosphamide (IVCY)-pulse therapy was introduced to a total of 8 times over the period of a year, and maintenance therapy with predonisolone and azathioprin was also used. After completion of the IVCY-pulse therapy, the hydronephrosis and constriction of the ureter were disappeared. No side effects of IVCY-pulses were observed, and the patient is now in remission. We reported a case of childhood SLE complicated with lupus cystitis and successfully treated by IVCY-pulse therapy and maintenance predonisolone and azathioprin.
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PMID:[A case report of childhood systemic lupus erythematosus complicated with lupus cystitis]. 1681 64


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