Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0024141 (systemic lupus erythematosus)
 44,322 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 21-year-old woman with long-standing systemic lupus erythematosus developed extensive calcification of the soft tissues of the thoracic and abdominal walls and extremities early in her illness, and these calcifications gradually disappeared over the course of her disease. The extent of this calcinosis and apparent spontaneous regression are unusual events in systemic lupus erythematosus.
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PMID:Extensive soft tissue calcification (calcinosis universalis) in systemic lupus erythematosus. 49 53

The clinical and laboratory features of 29 patients who had one of three anti-aminoacyl-tRNA synthetase autoantibodies, anti-Jo1 (histidyl-tRNA synthetase), anti-PL12 (alanyl-tRNA synthetase) or anti-PL7 (threonyl-tRNA synthetase) were analysed and compared with the findings of other published reports. These autoantibodies were found to be associated with a syndrome delineated by inflammatory myositis (24 patients) and pulmonary fibrosis (23 of 29), but also including inflammatory arthritis (26/29), keratoconjunctivitis sicca (17/29), sclerodactyly (21/29), Raynaud's phenomenon (27/29), hepatitis (8/29) and subcutaneous calcinosis (7/29). The most important clinical determinant of outcome in this group of patients was the severity of the interstitial pulmonary disease. No patient fulfilled the classification criteria for systemic lupus erythematosus, although 10 had autoantibodies to extractable nuclear antigens including Ro, La, RNP, and Sm, and two patients had anti-dsDNA antibodies. Although it seems unlikely that anti-aminoacyl-tRNA synthetase antibodies are directly responsible for causing disease, they may provide an important clue to the aetiology of this unusual syndrome.
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PMID:Polymyositis, pulmonary fibrosis and autoantibodies to aminoacyl-tRNA synthetase enzymes. 226 80

Calcinosis cutis is a common clinical feature of dermatomyositis and scleroderma but is only rarely reported in association with systemic lupus erythematosus (SLE). We describe three patients with long-standing systemic lupus erythematosus in whom extensive calcinosis cutis developed. We identify characteristics our patients share in common with 23 previously described patients.
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PMID:Extensive calcinosis cutis with systemic lupus erythematosus. 238 31

One hundred and forty five serum samples from patients with a connective tissue disease and 30 serum samples from healthy blood donors were analysed by immunoblotting. The presence of anti-Scl-70, which seems to discriminate between progressive systemic sclerosis (PSS) and the CREST (calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, telangiectasia) syndrome, was found in 31/64 (48%) patients with PSS, in 6/55 (11%) patients with systemic lupus erythematosus, in 2/26 (8%) patients with mixed connective tissue disease, and in none of 30 healthy blood donors. These data resulted in a specificity of 93% for this antibody in systemic sclerosis. For patients with PSS the duration of disease was significantly shorter in those with anti-Scl-70 antibodies than in those without, whereas the presence of anti-Scl-70 did not correlate with severity of disease. An 82% prevalence of anticentromere antibodies in patients with the CREST variant compared with a 4% prevalence in patients with PSS or with overlap syndrome confirms the high diagnostic value of this autoantibody for the CREST variant of PSS.
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PMID:Anti-Scl-70 antibodies detected by immunoblotting in progressive systemic sclerosis: specificity and clinical correlations. 251 13

Serum samples from 401 subjects were screened for the presence of anticentromere antibody using HEp-2 cells as the substrate for an indirect immunofluorescence method. Anticentromere antibody was found in 16 cases; 8 out of 62 patients with systemic sclerosis, 3 out of 7 patients with primary Raynaud's disease, 2 out of 41 patients with systemic lupus erythematosus, 1 out of 54 asymptomatic relatives of systemic sclerosis and 2 out of 50 patients with miscellaneous diseases. In systemic sclerosis, the patients with anticentromere antibody were limited in CREST (calcinosis, Raynaud's phenomenon, esophageal involvement, sclerodactylia and telangiectasia) variant. As reported previously by many investigators, anticentromere antibody is considered as a useful immunologic marker for CREST variant of systemic sclerosis, although this antibody is widely distributed in other conditions with less frequency.
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PMID:Clinical distribution of anticentromere antibody in Japanese patients. 264 77

A case of female with a large cystic tumor in her infrascapular region is reported. The tumor was 8 cm x 7 cm in size. Histologically, it was a pseudocyst with calcification. The patient showed no abnormal renal functions other than proteinuria. Because she had suffered from overlap syndromes of systemic lupus erythematosus, progressive systemic sclerosis, and suspected dermatomyositis, we diagnosed this a unique case of dystrophic calcinosis. There have been several reports of cases with connective tissue diseases showing subcutaneous calcification, but to our knowledge no such giant calcifying pseudocyst has ever been reported.
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PMID:A giant calcifying pseudocyst seen in a patient with overlap syndrome. 277 90

This paper looks at the problem confronting a doctor evaluating a patient with anticentromere antibody who does not have evidence of disease along the spectrum from CREST (calcinosis, Raynaud's phenomenon, oesophageal dysmotility, sclerodactyly, telangiectasia) to progressive systemic sclerosis. Of 33 people with anticentromere antibody, 21 had CREST and two had scleroderma. Of the other 10 with a positive anticentromere antibody, three had systemic lupus erythematosus (two with digital vasculitis), three very active seronegative polyarthritis, three Raynaud's phenomenon, and one a claudication syndrome involving the legs. A positive antinuclear antibody test does not always indicate the presence of a connective tissue disease, but the presence of anticentromere antibody without systemic sclerosis or CREST often indicates the presence of another sometimes serious underlying rheumatic or connective tissue disease.
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PMID:Anticentromere antibody in patients without CREST and scleroderma: association with active digital vasculitis, rheumatic and connective tissue disease. 280

We performed thallium 201 muscle scans to assess muscular involvement in 40 patients with different connective tissue diseases (7 with dermatomyositis, 7 with systemic lupus erythematosus, 12 with progressive systemic scleroderma, 2 with calcinosis, Raynaud's phenomenon, esophageal involvement, sclerodactyly, and telangiectasia (CREST) syndrome, 3 with monomelic scleroderma, 6 with morphea, and 3 with Raynaud's disease). Only 12 of these patients complained of fatigability and/or myalgia. Electromyography was performed and serum levels of muscle enzymes were measured in all patients. Comparison of thallium 201 exercise recording with the other tests revealed that scan sensitivity is greater than electromyographic and serum muscle enzymes levels. Thallium 201 scans showed abnormal findings in 32 patients and revealed subclinical lesions in 18 patients, while electromyography findings were abnormal in 25 of these 32 patients. Serum enzyme levels were raised in only 8 patients. Thallium 201 scanning proved to be a useful guide for modifying therapy when laboratory data were conflicting. It was useful to evaluate treatment efficacy. Because our data indicate a 100% positive predictive value, we believe that thallium 201 scanning should be advised for severe systemic connective tissue diseases with discordant test results.
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PMID:Evaluation of muscular lesions in connective tissue diseases: thallium 201 muscular scans. 337 58

The pathological findings in the lungs and related organs of 26 patients (21 female, 5 male) with systemic lupus erythematosus (SLE), with onset of disease before age 20 years, were reviewed. Several categories of lung lesions were found. Chronic interstitial pneumonitis was present in all 26 patients and was severe in 5. Acute pneumonia was present in 20, mild in 13, moderate in 2, and severe in 5. Alveolar hemorrhage, massive enough to cause death in 5, was seen in 18 patients, and pulmonary edema was found in 13. Fourteen patients had hyaline membranes indicative of acute alveolar damage (DAD), 12 had alveolitis obliterans, indicative of prior episodes of DAD, and 9 had bronchiolitis obliterans. Other parenchymal lesions were mild interstitial fibrosis in 12, alveolar hemosiderosis and alveolar overinflation in 10 each, and alveolar septal calcinosis with chronic renal insufficiency in 3. Pleural effusion, pleuritis, or pleural thickening were noted in 15 of 26, 6 of 23, and 7 of 23 evaluable patients, respectively. Vascular lesions were present in 16 as intimal thickening (9), thromboemboli (8), medial hypertrophy (6), calcinosis (3), and vasculitis (2). A previously unreported lesion was chronic (proliferative) peribronchitis, noted in 11 patients. Diaphragmatic lesions included mild variation in fiber size in 7, mild fibrosis in 2, and calcinosis in 1 of 13 evaluable patients. Correlation of the above lesions with previously described lung syndromes in SLE such as lupus pneumonitis, hemorrhagic lung disease, chronic interstitial fibrosis, lupus cor pulmonale, pleurisy, and "shrinking lung syndrome" are discussed.
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PMID:Pulmonary lesions in childhood onset systemic lupus erythematosus: analysis of 26 cases, and summary of literature. 360 12

Radiographs and xerographs of the hands of 35 patients with progressive systemic sclerosis (PSS), as defined by the ARA, were reviewed. Patients with "overlap" syndromes (i.e., mixed connective tissue disease, systemic lupus erythematosus or rheumatoid arthritis) have been excluded. Soft tissue changes included atrophy (hide-bound skin), and dystrophic calcifications, particularly in CREST patients (calcinosis, Raynaud phenomenon, esophageal dysmobility, sclerodactily and telangectasia). The most common bony change is resorption of distal phalanges; diffuse osteoporosis is also frequent; the distal interphalangeal and first carpometacarpal joints involvement appear as a distinctive feature of this erosive arthritis.
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PMID:[Radiology of the hand in progressive systemic sclerosis]. 373 85


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