Gene/Protein Disease Symptom Drug Enzyme Compound
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A computer analysis was made of the data from a prospective study of the clinical course of systemic lupus erythematosus (SLE) in 234 patients followed for an average of 46 months. All fulfilled four ARA criteria for the diagnosis of SLE. Sixteen of the 234 patients were aged 51 or older. They were compared with the 218 younger SLE patients to determine the influence of age on the signs and symptoms of the disease, the organ systems involved, the laboratory data, amount and duration of corticosteroid or azathioprine therapy, and the prognosis. The older group showed more discoid lupus, photosensitivity and pulmonary fibrosis than did the younger group, but a similar incidence of malar rash, alopecia, arthritis, arthralgia, myalgia and serositis, and a lower incidence of oral ulcers, Raynaud's phenomenon, cutaneous vasculitis, neuropsychiatric manifestations, leukopenia, hypocomplementemia and profus proteinuria. The older patients needed a lower dosage of corticosteroids, and a shorter course of azathioprine therapy. These findings suggest a milder form of SLE with better response to therapy in the older group.
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PMID:Systemic lupus erythematosus in the older age group: computer analysis. 76 67

A 38-year-old man was hospitalized for proteinuria, and pitting edema. He had noticed Raynaud's phenomenon at about age fifteen. One month prior to admission, his urine contained protein and the serum creatinine was 3.0 mg/dl. On admission, sclerodactylia, digital pitting scar of fingertips, digital bone absorption and pulmonary fibrosis were observed and a diagnosis of progressive systemic sclerosis (PSS) was made. Laboratory investigations revealed: 24-hour urine protein excretion 3 g; serum creatinine 5.6 mg/dl; creatinine clearance 13.5 ml/min; antinuclear factor strongly positive in a speckled pattern; antibodies to nRNP positive with a titer of 1: 20, 480; antibodies to DNA, Sm, SS-A, SS-B, Scl-70, centromere and Jo-1 negative; serum complement normal. A renal biopsy revealed focal and segmental necrotizing glomerulonephritis with 70% crescents but no vascular changes. Circulating antiglomerular basement membrane antibodies were negative. Immunofluorescence disclosed granular deposits of IgM and C3 in the mesangium and along the capillary walls. Treatment was begun with methylprednisolone pulse therapy. After 5 month, serum creatine and creatinine clearance were 1.9 mg/dl and 35 ml/min, respectively. A year after the discharge, he was readmitted for hemoptysis and worsening of proteinuria and microhematuria. A chest radiograph demonstrated bilateral alveolar consolidation. Serum creatinine was elevated to 3.5 mg/dl. The continuous hemoptysis resulted in a severe dyspnea associated with a rapid fall in the hemoglobin. On the fourth hospital day, the PaO2 was 41 Torr on oxygen by mask that necessitated mechanical ventilation and pulse therapy was started. However, the patient died on the ninth hospital day of respiratory failure due to pulmonary hemorrhage.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[A case of progressive systemic sclerosis complicated by crescentic glomerulonephritis and diffuse pulmonary hemorrhage]. 147 23

A 22 year old male of lupus nephritis associated with minimal change nephrotic syndrome was described. The patient was well until 4 years earlier, when Raynaud's phenomenon and photosensitivity developed. One week before admission, edema appeared suddenly and proteinuria was pointed out on July 28, 1990. On admission, his legs, ankles, and eyelids were edematous. There was no sclerodactylia, although Raynaud's phenomenon was positive when his hand disclosed to the cold. Urinalysis showed heavy albuminuria (10.4g/day), but urinary sediment showed no abnormality. Immunological examination showed positive antinuclear antibody, determination at a titer of 1: 160 with a speckled pattern. Anti-RNP and anti-Sm antibody were positive. However, neither anti-DNA antibody nor hypocomplementemia was detected. There was high concentration of serum IgE (2564IU/ml). Renal biopsy was performed. Light microscopic study showed slight increase of mesangial cells and matrix. Immunofluorescence study showed mesangial localization of IgG and C3. Electron microscopic study showed electron dense deposits only in the mesangial area. The diagnosis of lupus nephritis associated with minimal change nephrotic syndrome (MCNS) was made and administration of PSL was started. Proteinuria disappeared after 3 weeks and nephrotic syndrome remitted completely. The case of lupus nephritis associated with MCNS is very rare. Therefore, the relationship between amounts of proteinuria and various histological types of 67 cases with lupus nephritis which we experienced at our hospital was evaluated. Results showed that nephrotic range proteinuria was not present in mesangial lupus nephritis. So, it was concluded that if heavy albuminuria was found in mesangial lupus nephritis, we should consider the possibility of lupus nephritis associated with MCNS.
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PMID:[A case report of lupus nephritis associated with minimal change nephrotic syndrome--comparison of various histological types of 67 cases with lupus nephritis]. 147 24

A 1987 questionnaire sponsored by the Health and Welfare Ministry concerning the clinical subsets and severity of systemic lupus erythematosus (SLE) was distributed to 93 medial facilities. A clinical analysis of the outcome and treatments was accomplished on one thousand six hundred and fourteen SLE patients fulfilling ARA criteria. The outcome was evaluated into 6 categories, namely; complete remission, incomplete remission, no change, gradual worsening, rapid worsening and unknown. Treatments included (1) anti-inflammatory drugs, (2) initial dose of prednisolone (PSL) below 29 mg/day, (3) initial dose of PSL from 30 to 59 mg/day, (4) initial dose of PSL above 60 mg/day, (5) pulse therapy, (6) immunosuppressants, (7) plasmapheresis, and (8) hemodialysis. Statistical significances were determined with ridit analysis. The severity of the disease for 1,614 SLE patients was evaluated by the judgement of each medical facility independently, separating it into 3 grades. As a result, 16.8% was evaluated as severe, 54.6% was evaluated as moderate, and 28.6% was evaluated as mild. Clinical subsets were divided into 3 categories according to the outcome; (1) those with high complete remission rates (serositis, convulsion, oral ulcers, unconsciousness, hemolytic anemia and so on), (2) those with high incomplete remission rates (lupus nephritis, digital gangrene, hypertension, peripheral neuropathy, erythema, Raynaud's phenomenon and so on), and (3) those with high rates of no change or worsening (aseptic bone necrosis, pulmonary hypertension, pneumonitis, chronic renal failure and so on). SLE patients with persistent proteinuria below 3.4 g/day, pulmonary hypertension, or pneumonitis treated with large doses of PSL such as an initial dose of PSL above 60 mg/day and/or pulse therapy had a significantly higher remission rate than those treated with small dosages of PSL. Hereafter, the establishment of modes of treatments for increasing the remission rates of intractable clinical subsets in highly desired.
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PMID:[Studies on clinical subsets and severity of systemic lupus erythematosus based on a 1987 questionnaire conducted in Japan--clinical analysis of the outcome and treatments in clinical subsets]. 160 13

A 40-year-old woman was admitted because of increasing exertional dyspnea. Right heart failure was suggested by the presence of hepatomegaly, pretibial edema and also echocardiographic findings. Physical examination and echocardiography showed no evidence of valvular disease or congenital heart disease except for right ventricular dilatation and tricuspid regurgitation. The ventricular septum deviated toward the left ventricle throughout the cardiac cycle, but left ventricular function was preserved. Severe pulmonary hypertension averaging 44 mmHg was revealed by cardiac catheterization. Digital subtraction angiography and pulmonary blood flow scintigraphy showed no evidence of pulmonary artery embolism, and no interstitial pulmonary lesions that might have caused pulmonary hypertension were recognized. Hypergammaglobulinemia suggested an autoimmune disorder, and signs of systemic lupus erythematosus (SLE), such as pleural effusion, proteinuria, lymphocytopenia, LE cell phenomenon and antinuclear antibodies were present. Several autoimmune diseases are known to be causative factors of pulmonary hypertension. However, only ten cases of SLE complicated by pulmonary hypertension have been reported the present one. These cases were characterized by a high incidence of Raynaud's phenomenon and positivity for anti-RNP antibody. In our present case, SLE activity was suppressed using prednisolone, but pulmonary hypertension persisted and the patient eventually died due to right cardiac failure. Judging from the clinical course of the ten reported cases of SLE-pulmonary hypertension, there seems to be no hope of improving the pulmonary hypertension once it has become established. Therefore it is important to detect and cure pulmonary hypertension as early as possible.
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PMID:[A case of lupus erythematosus preceded by right heart failure due to pulmonary hypertension]. 174 69

We studied the relationship between vascular complications and coagulation and fibrinolysis parameters in 75 subjects with collagen diseases. Thirty normal healthy persons served as controls. We found that patients with collagen diseases were in a state of a hypercoagulation and hyperfibrinolysis. In SLE (systemic lupus erythematosus) in particular, coagulation and fibrinolysis parameters appeared to be indices of vascular complications. Increases in the levels of thrombin-antithrombin III complex (TAT) and alpha 2-plasmin inhibitor-plasmin (PIP) were particularly associated with proteinuria, while increases in fibrinopeptide A (FPA) levels were associated with Raynaud's phenomenon. Administration of glucocorticoid seemed to improve the hypercoagulation and hyperfibrinolytic states of patients with collagen diseases. Analysis of the multimeric structure of von Willebrand factor (vWF) revealed a tendency for large and intermediate multimers (LIM) of plasma vWF to increase in SLE patients with accompanying vascular complications, whereas such increases were not observed in SLE patients without any vascular complications. Therefore, analysis of the multimeric structure of vWF appeared to be a useful indicator of vascular complications in collagen diseases.
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PMID:Plasma coagulation and fibrinolysis parameters in patients with collagen diseases, and analysis of the multimeric structure of von Willebrand factor (vWF). 175 53

A 39-year-old woman developed transient erythema and arthralgia in spring 1987. In June she had a tick bite followed by local erythema and later migrating skin changes. Furthermore she developed pain in various joints with Raynaud's phenomenon at the fingers, swelling of the knee joints and shoulder pain. Demonstration of antibodies against B. burgdorferi antigen was shown in one institution (IFL, Western blot) while the same serum in two other institutions remained negative (IHA, ELISA). Antibiotic treatment was only temporarily successful. While the demonstration of antinuclear factors could be attributed to cross-reacting antibodies in borreliosis failing effects of absorption of serum with this antigen led to the assumption SLE as the underlying disease. Further indications were lymphopenia, increasing titers of anti ds-DNA antibodies and renal involvement as erythrocyturia and proteinuria. Sudden relief of the symptoms after treatment with steroids may be taken as further prove for this assumption. The interference of both diseases and their similarity in symptoms may impede correct diagnosis.
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PMID:[Borrelia infection and systemic lupus erythematosus]. 269 42

Several recent studies have focused on the discrepancy between lupus nephropathy and clinical renal involvement and, consequently, question the relevance of renal biopsy in these patients. We analyze the clinical characteristics, histological renal findings and subsequent course of patients with silent renal disease. Renal biopsy was performed in 15 patients with systemic lupus erythematosus (SLE) who had no clinical signs of renal involvement (no urinary sediment abnormalities, absence of proteinuria and serum creatinine less than 1.3 mg/dl). All biopsies were classified according to a modified classification proposed by the WHO. Six cases (40%) showed no histological or immunofluorescence changes (type I), 7 (47%) had mesangial nephropathy (3 type IIa and 4 type IIb) and 2 (13%) had focal proliferative glomerulonephritis (type III). None of the patients had previous evidence of neurological abnormalities. Patients with type I only had arthritis, skin lesions and Raynaud's phenomenon. By contrast, 7 patients with histological renal involvement had serositis or hemolytic anemia. All cases with silent nephropathy were treated with steroids and showed a benign clinical course with stable renal function and absence of urinary abnormalities during follow-up. We concluded that in the absence of clinical renal abnormalities, renal involvement is not uncommon in SLE. We believe that a renal biopsy should be performed mainly in those SLE patients presenting with clinical manifestations other than arthritis or cutaneous lesions since this policy may allow detection of significant silent renal injury.
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PMID:Silent renal disease in systemic lupus erythematosus. 349

The presence of the discoid lupus erythematosus (DLE) skin lesion in a patient with systemic lupus erythematosus (SLE) has been suggested to be a marker of less frequent and less severe renal disease. The clinical and laboratory features of seventeen patients who were seen in a dermatology practice and who had DLE as a manifestation of SLE (DLE-SLE) are reported. DLE preceded the diagnosis of SLE in eight patients. In six patients, the onset was concurrent, whereas in three the SLE was present prior to the discoid skin lesions. Five of the patients had lesions of subacute cutaneous lupus erythematosus (SCLE), and Raynaud's phenomenon occurred in eight patients. Clinical evidence of a renal abnormality was present in six patients (hematuria in three, proteinuria in five, and abnormal renal function in two). Antibodies to nuclear and/or cytoplasmic components were abnormal in all patients. Antibody subsets did not correlate well with clinical findings; only half of those with Raynaud's phenomenon demonstrated a positive ribonucleoprotein; only one patient with SCLE demonstrated anti-Ro (SSA) antibody, but four of the six patients with a renal abnormality had an elevated anti-native deoxyribonucleic acid antibody titer. The cutaneous lesions were eventually widespread in all patients, although two had initial disease that was localized to the head and neck. Although renal disease occurs in this group, it is less common and usually milder than in previous groups of unselected SLE patients.
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PMID:Systemic lupus erythematosus in patients with chronic cutaneous (discoid) lupus erythematosus. Clinical and laboratory findings in seventeen patients. 387

Sera from 70 patients with systemic lupus erythematosus were studied for antinuclear antibodies and its clinical significance. Indirect immunofluorescence technique with mouse liver tissue and Crithidia luciliae flagella as substrates was used to detect antinuclear antibodies (FANA) and antibodies to double-stranded DNA (anti-ds DNA), respectively. Double immunodiffusion method identical with reference sera was used to detect antibodies to extractable nuclear antigens (anti-ENA). The prevalence of fluorescence antinuclear antibodies, antibodies to ds DNA and antibodies to ENA were 95.7%, 52.9% and 58.6%, respectively. Patients with homogeneous pattern of FANA presented clinically with high incidence of LE cell phenomenon. Patients with anti-ds DNA antibodies presented clinically with high incidences of serositis, profuse proteinuria, low serum C3 and high clinical activity. Patients with anti-RNP antibodies presented with high incidence of Raynaud's phenomenon and low incidence of anti-ds DNA antibodies. Patients with severe renal involvement showed a good correlation with high titer of anti-ds DNA antibodies and low serum C3. Therefore, these two factors together are helpful to predict the severity of renal involvement at the time of serum sample was obtained.
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PMID:Clinical significance of antinuclear antibodies in systemic lupus erythematosus. 702 88


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