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Query: UMLS:C0024141 (systemic lupus erythematosus)
 44,322 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Acute pancreatitis (AP) rarely complicates the clinical course of systemic lupus erythematosus (SLE). AP as the initial manifestation of SLE is exceptional, but its outcome is often fatal. Corticosteroids have been suspected to play a role in the development of AP, but the therapeutic benefit seems to be far above the risk of exacerbation of pancreatic lesions. We report a 13-y-old girl presenting with arthralgia and malaise, followed by abdominal pain, generalized oedema and haemodynamic instability. Increased CRP (325 ng/ml), serum amylase (14,000 IU/l) and lipase (2500 IU/l) levels suggested AP. Acute anuric renal failure required haemodialysis. Multiorgan involvement suggested SLE, which was confirmed 3 d later by increased anti-ds-DNA levels. Three methylprednisolone pulses were administered promptly, followed by oral prednisone (1.5 mg/kg/d) and six pulses of cyclophosphamide (500 mg/1.73 m2/2 wk). Mycophenolate mofetil was introduced for long-term disease control. Amylase and lipase levels decreased over 4 wk. Renal function was normal after 3 wk and proteinuria negative after 6 wk. This case suggests that steroid pulse therapy should be promptly administered if clinical and biochemical investigations suggest SLE to be responsible for AP. Aggressive treatment may be life saving.
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PMID:Acute pancreatitis in paediatric systemic lupus erythematosus. 1637 10

A 39-years-old woman was admitted to our hospital with musculoskeletal complaints (myalgias and symmetric arthralgias in proximal interphalangeal, metacarpophalangeal joints of the hands and in knees), systemic symptoms like fever, fatigue, malaise and a six months previous history of a transient ischemic attack. The presence of antibodies to double-stranded deoxyribonucleic-acid (DNA) and antiphospholipid antibodies led to the diagnosis of systemic lupus erythematosus with secondary antiphospholipid syndrome. Cerebral infarction develops significantly more often in patients with lupus and antiphospholipid antibodies, but other clinical syndromes are associated with lupus anticoagulant: cognitive dysfunction, seizures, polyneuropathy, aseptic meningitis, myelopathy.
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PMID:[Systemic lupus erythematosus with neurologic onset and secondary antiphospholipid syndrome. A Case Study]. 1660 81

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

Visceral involvement in systemic lupus erythematosus (SLE) extends beyond renal and cutaneous management. Pleuro-pulmonary lesions have been recognised and diffuse alveolar damage and hemorrhage are the most difficult patterns to control. Pulmonary compromise in clinical evolution of SLE differs from children to adults, both in morphological patterns and in clinical presentation, depending on immunocompetence and the treatment prescribed. A 16-year-old boy presented asthenia, malaise and bilateral cervical painless adenopathies understood as EBV infection as serological EBV IgG, IgM and EBNA were positive. The symptoms persisted for eight months when discrete erythematous and desquamative nasal and malar rash expressed together with persistent fever, dispnoea and bibasilar crackles. Lymph node and pulmonary biopsies were performed. Lymph node presented follicular hyperplasia and LMP1 (EBV) immunostaining was negative. In lung biopsy bronchovascular lesions were consistent with vasculitis and bronchiolitis due to intense macrophage infiltration, validated with CD68 antibody and intra-alveolar macrophages were also present with septal compromise; LMP1 (EBV) positive cells were not visualized. The lung pattern seen in CAT as diffuse micronodules all over the lung parenchyme resolved after corticosteroid therapy. The diagnosis of SLE was confirmed by ANA, anti-dsDNA, anti-nDNA and anti-histones positivity. To the best of our knowledge this is the first reported case of pulmonary SLE involvement with vasculitis and diffuse panbronchiolitis - like pattern as the first clinical sign of the disease.
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PMID:[Vasculitis and diffuse panbronchiolitis-like in systemic lupus erythematosus--case report]. 1749 36

We report a 12 years old female patient with an overlap syndrome involving autoimmune hepatitis (AIH) and systemic lupus erythematosus (SLE). The patient presented with jaundice, hepatosplenomegaly, malaise, polyarthralgia, arthritis and butterfly rash on the face. Laboratory tests revealed severe liver dysfunction, Coombs positive hemolytic anemia and a positive ANA/anti-dsDNA test. Renal biopsy showed class IIA kidney disease, while liver biopsy showed chronic hepatitis with severe inflammatory activity. The patient satisfied the international criteria for both SLE and AIH. Clinical symptoms and laboratory findings of SLE improved with high dose treatment with corticosteroids and azathioprine, however, remission of the liver disease could not be achieved. Repeat biopsy of the liver after three years of therapy revealed ongoing chronic hepatitis with high level of inflammatory activity. The present case indicates that children with liver dysfunction and SLE should be investigated for AIH. There is much diagnostic and therapeutic dilemma in patients with AIH-SLE overlap syndrome.
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PMID:An overlap syndrome involving autoimmune hepatitis and systemic lupus erythematosus in childhood. 1756 52

We describe an unusual case of elderly onset systemic lupus erythematosus (SLE) that presented with disseminated intravascular coagulation (DIC). An 86-year-old man who complained of general malaise was admitted for evaluation and treatment of thrombocytopenia. He was diagnosed as having SLE and DIC based on the criteria of the American College of Rheumatology for SLE (renal involvement, hematological abnormalities, and positivity for antinuclear antibody and lupus anticoagulant) and the criteria for DIC presented by the subcommittee on DIC of the ISTH (a large increase of fibrin degradation products [3 points] and a platelet count <50 x 10(3)/ml [2 points], resulting in a score of 5; a score > or =5 is compatible with DIC). The patient was treated with corticosteroid therapy (30 mg/day); the DIC and SLE remitted, and his renal function improved, but he developed pulmonary tuberculosis. Timely diagnosis, appropriate treatment, and an awareness of the potential for serious infections are of utmost importance when dealing with patients with elderly onset SLE.
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PMID:Elderly onset systemic lupus erythematosus (SLE) presenting with disseminated intravascular coagulation (DIC). 1809 13

A 19-year-old female was admitted with general malaise and systemic edema. She had been diagnosed as having autoimmune hemolytic anemia (AIHA) eight years earlier and was successfully managed with oral prednisolone. During the current admission, she was diagnosed as having systemic lupus erythematosus (SLE) based on the presence of renal involvement, hematological abnormalities, and antinuclear and anti-double-stranded DNA antibodies, along with a recurrence of AIHA; her serology revealed a high myeloperoxydase-antineutrophil cytoplasmic antibody (MPO-ANCA) titer. She was treated with prednisolone (50 mg day(-1)), but her renal function started to deteriorate. She responded to treatment with hemodialysis, plasmapheresis, and methylprednisolone pulse therapy; her MPO-ANCA titer and renal function improved. Treatment with intravenous cyclophosphamide gradually suppressed her AIHA and SLE activity. A renal biopsy revealed a diffuse proliferative lupus nephritis (class IV-G (A)) with necrotizing crescentic glomerulonephritis that was presumed to be associated with MPO-ANCA. The association of MPO-ANCA with SLE in this refractory case is discussed.
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PMID:Lupus nephritis associated with positive MPO-ANCA in a patient with underlying autoimmune hemolytic anemia. 1846 93

Kikuchi-Fujimoto disease (KFD) or histiocytic necrotizing lymphadenitis is a rare benign disease, more common in Japanese women, with only a few cases reported from other parts of the world. The pathogenesis is poorly understood. A 22-year-old man noticed a node growth a month before his admittance. At first he noticed one enlarged node in the left axilla, later also the nodes on his neck and in his right axilla. Before that he had felt shivery and reported night sweats. Physical examination revealed bilateral enlarged (up to 2 cm) painful but not inflamed lymph nodes on the neck and in both axillae. The patient was diagnosed with KFD on histological examination of the nodes and on the basis of typical changes. No medical treatment was prescribed and the patient has been without symptoms for the past two years. The clinical picture of KFD is nonspecific and variable. Most patients complain of enlarged cervical nodes (up to 2 cm) accompanied by various symptoms (fever, malaise, night sweats, weight loss). The enlarged nodes, which are mobile, rarely appear in other locations. The only way of proving whether the disease is present or not is by histological examination of the node, which may show the following changes typical for KFD: paracortical necrotic foci, surrounded by histiocytic aggregates, irregular rounded eosinophilic areas of different sizes in the paracortex and cortex with the presence of numerous histiocytes, lymphocytes, immunoblasts, plasmacytoid monocytes and eosinophilic granulated cellular debris. Histiocytes had mostly turned into foamy macrophages. It is typical not to find epithelioid cells and neutrophil granulocytes in the necrotic centers. In particular, the disease needs to be distinguished from high-grade lymphoma and SLE lymphadenitis. There is no specific medical treatment for KFD since the disease usually resolves spontaneously over four months. Disease recurrence or fatalities are unusual.
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PMID:Necrotizing cervical lymphadenopathy: Kikuchi-Fujimoto disease in a young male. 1870 24

We present a 43-year-old woman with relapsing-remitting multiple sclerosis (MS) who developed lupus syndrome after 32 months of IFN-beta-1a therapy. She presented with malaise, myalgia, arthralgia and fever. Laboratory tests showed high erythrocyte sedimentation rate, anaemia and lymphopenia. Antibodies to double stranded DNA (dsDNA) of IgG, IgM and IgA classes were detected on Critidia luciliae. Additionally, high levels of anti-nucleosomal antibodies, low levels of anti-histone and anti-Ro/SSA antibodies were also found. Diagnosis of drug-induced SLE was established. Treatment with IFN-beta was discontinued and oral prednisone was started. Twelve weeks after cessation of IFN-beta therapy, the patient's symptoms completely resolved and autoantibodies disappeared. To the best of our knowledge, this is the first report of a patient with MS in whom treatment with IFN-beta induced lupus syndrome and antibodies to dsDNA and nucleosome.
Lupus 2009 Jan
PMID:Anti-double stranded DNA and lupus syndrome induced by interferon-beta therapy in a patient with multiple sclerosis. 1907 73

We report two elderly male patients with hemophagocytic syndrome (HPS) associated with systemic lupus erythematosus (SLE). They were admitted to the hospital because of general malaise. At admission, they showed fever of unknown origin and hematological abnormalities without typical symptoms for SLE such as arthralgia or malar rash. Chest X-rays, computed tomography and cardiac sonogram demonstrated unilateral pleural and pericardial effusions. Bone marrow aspiration revealed hypocellular marrow with increased macrophages phagocytosing blood cells in the both cases. One patient had positive reactivity for direct Coombs's test and high level of platelet-associated antibody, whose symptoms were ameliorated only by 20 mg per day of prednisolone. The other patient, however, progressive worsened and died regardless of intensive treatment containing methyl-prednisolone pulse therapy. Late-onset lupus (LO-SLE) patients tend to have a more insidious onset of disease, and less frequently show typical symptoms of early-onset SLE such as malar rash. So it seems to be important that clinicians make a fast diagnosis and proper treatment for LO-SLE and associated HPS by careful observation for the latent symptoms and laboratory findings.
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PMID:[Hemophagocytic syndrome in late-onset SLE patients]. 1940 10


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