1/28. Systemic lupus erythematosus with C1q deficiency.We report a case of systemic lupus erythematosus associated with C1q deficiency. Our patient presented at the age of 6 years with cutaneous lupus. She later developed Raynaud's phenomenon, non-scarring alopecia, oral ulceration and grand mal seizures due to cerebral vasculitis. complement c3 and C4 levels were consistently normal during flares of her lupus and haemolytic activity of her complement was absent, suggesting a deficiency of an early component of the complement cascade. No C1q could be detected.- - - - - - - - - - ranking = 1keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, systemic lupus (Clic here for more details about this article) |
2/28. Lamotrigine-induced lupus.A 57-year-old woman was under treatment for epilepsy with lamotrigine 2 mg/kg/d since 1996. In April 1998 she was admitted to the rheumatology Unit for arthralgias affecting the small joints of the hands, wrists and knees, erythematosus skin rash and myalgias. She referred a vascular abnormality at the hands exposed to cold, compatible with Raynaud's phenomenon. serum antinuclear antibodies (ANA) were positive (1:320, speckled pattern); moreover, a positivity for anti-Ro/SSA was observed. rheumatoid factor was negative, serum complement was normal. LAC and anticardiolipin antibodies were negative. Drug-related lupus diagnosis was made with resolution of symptoms and gradual normalisation of positivity of ANA and anti-Ro/SSA upon lamotrigine withdrawal. To our knowledge, this is the first case report of an association between lamotrigine and the onset of SLE (systemic lupus erythematosus).- - - - - - - - - - ranking = 0.29873882166047keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, systemic lupus (Clic here for more details about this article) |
3/28. A juvenile case of overlap syndrome of systemic lupus erythematosus and polymyositis, later accompanied by systemic sclerosis with the development of anti-Scl 70 and anti-Ku antibodies.We describe a 16-year-old girl with an overlap syndrome consisting of systemic lupus erythematosus (SLE) from the age of 7 and polymyositis (PM) from the age of 10, later accompanied by systemic sclerosis (SSc) from the age of 15. She was diagnosed as having SLE with exudative malar erythema, photosensitivity, and discoid rashes with positive antinuclear antibody (ANA) and anti-dna antibody titers. The diagnosis of PM was also made in accordance with findings of a high titer of muscle enzymes and a muscle biopsy specimen demonstrating marked degeneration of the muscle fibers and perivascular infiltration of mononuclear cells. She developed Raynaud's phenomenon and pitting ulcers on her fingers with positive anti-Scl 70 and anti-Ku antibodies, leading to a diagnosis of SSc. The patient was treated with prednisolone. To our knowledge this is the youngest case of SLE-PM overlap syndrome later accompanied by SSc.- - - - - - - - - - ranking = 1.1885845898049keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, systemic lupus (Clic here for more details about this article) |
4/28. Case report. Two cases of pulmonary hypertension with Raynaud's phenomenon. Primary pulmonary hypertension and systemic lupus erythematosus.Two cases of hypertensive pulmonary vascular disease with Raynaud's phenomenon are reported. One is a 32-year-old housewife of primary pulmonary hypertension with some immunological disorders and the other is a 28-year-old female of systemic lupus erythematosus. It may be considered that some immunological process could play a role in the etiology of some cases of primary pulmonary hypertension and the relationship of pathogenesis between these 2 cases was discussed.- - - - - - - - - - ranking = 1.1885845898049keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, systemic lupus (Clic here for more details about this article) |
5/28. Complete heart block and systemic lupus erythematosus.An 18-year-old girl with systemic lupus erythematosus developed progressive electrocardiographic abnormalities over a period of 16 years, culminating in complete heart block with Adams-Stokes attacks. A permanent ventricular pacing system was implanted successfully.- - - - - - - - - - ranking = 1.1885845898049keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, systemic lupus (Clic here for more details about this article) |
6/28. Antibodies to glycyl-transfer rna synthetase in patients with myositis and interstitial lung disease.OBJECTIVE. We have previously described anti-EJ antibodies, and provided evidence that these antibodies react with glycyl-transfer rna (gly-tRNA) synthetase. The aim of the present study was to identify patients with anti-EJ antibodies and describe the clinical associations of the antibody, in particular, whether it is associated with the syndrome of myositis and interstitial lung disease (ILD) that has been previously associated with autoantibodies to the aminoacyl-tRNA synthetases for histidine, threonine, and alanine. methods. Sera from patients with suspected or proven polymyositis or dermatomyositis (DM), sera with anticytoplasmic patterns, and control sera were tested for anti-EJ antibodies by immunoprecipitation (IPP). Positive sera and controls were tested for the ability to inhibit gly-tRNA synthetase by preincubation of the enzyme source with the serum. RESULTS. Anti-EJ antibodies were demonstrated in the sera of 5 patients, by IPP of characteristic tRNAs and protein. Original serum EJ and each of the new sera significantly inhibited the enzymatic activity of gly-tRNA synthetase but not histidyl-tRNA synthetase. All 5 of the new patients had inflammatory myopathy, a typical DM rash, and ILD. One, who had an overlap syndrome with systemic lupus erythematosus, had anti-EJ at least 4 months before the development of clinical myositis. arthritis and Raynaud's phenomenon, other features associated with antisynthetases, were also seen. CONCLUSION. Anti-EJ is associated with the syndrome of myositis and lung disease that is seen in association with other antisynthetases. The finding of specific inhibition of gly-tRNA synthetase by all anti-EJ-positive sera strongly supports the identification of EJ antigen as gly-tRNA synthetase.- - - - - - - - - - ranking = 0.23771691796098keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, systemic lupus (Clic here for more details about this article) |
7/28. erythema elevatum diutinum in the setting of connective tissue disease and chronic bacterial infection.erythema elevatum diutinum (EED) is a rare and chronic cutaneous leukocytoclastic vasculitis. It is predominantly seen on the extensor surfaces of the extremities. Although a specific cause is largely unknown, EED has been noted to occur in association with a wide variety of diseases. A 28-year-old man with systemic lupus erythematosus (SLE) and a 53-year-old woman with an overlap syndrome of rheumatoid arthritis and polymyositis are presented. Both patients developed EED in the setting of chronic recurrent bacterial infections. patients with a connective tissue disease are at increased risk for such infections secondary to immunosuppression, either from the disease itself or secondary to immunosuppressive therapy. EED has been independently reported to occur in the setting of connective tissue disease as well as in the setting of chronic infection. Our patients had both of these underlying conditions, which are known to predispose patients to immune complex-mediated vasculitides, in this case EED. One patient's EED responded to treatment of the SLE and the other improved, as has been previously reported with dapsone.- - - - - - - - - - ranking = 0.23771691796098keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, systemic lupus (Clic here for more details about this article) |
8/28. Colour duplex sonography of finger arteries in vasculitis and in systemic sclerosis.case reports: Three patients-two with Wegener's granulomatosis and one with an overlap syndrome of rheumatoid vasculitis, systemic lupus erythematosus, and antiphospholipid syndrome-are described. All patients experienced a sudden onset of Raynaud's phenomenon or acrocyanosis when they had a flare of their disease. DISCUSSION: ultrasonography (US) showed dark (hypoechoic) arteries without colour signals, resembling the US pattern of embolism. In contrast, US in patients with systemic sclerosis is entirely different, delineating a smaller artery lumen, reduced pulsation, and thickened, slightly hyperechoic artery walls.- - - - - - - - - - ranking = 0.23771691796098keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, systemic lupus (Clic here for more details about this article) |
9/28. Atlantoaxial subluxation in a patient with mixed connective tissue disease.We describe a 55-year-old woman with an 8-year history of mixed connective tissue disease (MCTD). Her condition was characterized by severe Raynaud's, swollen fingers, digital ulceration and gangrene, esophagitis, polyarthropathy, myositis and restrictive lung function. She consistently had antibodies to U1-RNP. rheumatoid factor was present in low titer. She developed atlantoaxial subluxation, a feature seen commonly in rheumatoid arthritis, reported in spondyloarthropathy and a small number of patients with systemic lupus erythematosus but not described in MCTD.- - - - - - - - - - ranking = 0.23771691796098keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, systemic lupus (Clic here for more details about this article) |
10/28. Isolated trigeminal sensory neuropathy: early manifestation of mixed connective tissue disease.A young woman with mixed connective tissue disease (MCTD) had an isolated trigeminal sensory neuropathy as an early manifestation of the disease. Raynaud phenomenon occurred almost synchronously with the onset of trigeminal neuropathy and was followed by myositis, diffuse hand swelling, synovitis, and increased ribonucleoprotein antibody. mixed connective tissue disease has overlapping features of systemic lupus erythematosus, scleroderma, and polymyositis, and is differentiated from them by high-titer antibody to ribonucleoprotein.- - - - - - - - - - ranking = 0.23771691796098keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, systemic lupus (Clic here for more details about this article) |
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