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PubMed1983

Prognostic significance of renal angiitis in systemic lupus erythematosus (SLE).

Analysis of 80 cases of SLE with renal involvement revealed the following. Angiitis involving arterioles and interlobular arteries was observed in 17 out of 42 cases with diffuse proliferative lupus GN. This was not observed in other types of lupus GN. Out of 17 cases of angiitis, 7 were complicated by thrombosis. Irregular deposits of immunoglobulins and complement were demonstrated in the vessel walls and in luminal thrombi and suggested an immune-complex origin. Fibrinogen was also demonstrable frequently. The vascular lesions were associated with severe glomerular and tubulointerstitial injury and a poor prognosis. A relatively favorable outcome was observed in SLE without renal angiitis.

Arteritis↗

PubMed1984

Binding capacity of sera from systemic lupus erythematosus (SLE) and rheumatoid arthritis (RA) to C3c. Evaluation by enzyme linked immunosorbent assay (ELISA).

Immunoconglutinins (IKs) are autoantibodies directed against antigenic determinants on C3 complement component. An ELISA was performed to detect IKs in sera from 50 RA patients, 50 SLE patients and 50 normal subjects. Comparison showed significantly higher levels in patients than in normal subjects (p less than 0.001) and higher IKs levels in RA than in SLE (p less than 0.001). IKs were not related to others biological tests, except a statistically significant inverse correlation between IKs and circulating immune complexes levels detected by conglutinin binding assay in RA.

Adult↗

PubMed1983

Solubilization of immune complexes and inhibition of immune precipitation in SLE sera.

The ability of serum to in vitro process immune complexes both preformed (ICSC) and during their formation (ICPIC) has been evaluated in 37 patients affected by systemic lupus erythematosus (SLE). ICSC was found to be significantly lower in these patients than in normal controls (p less than 0.001), and strictly related to the phase of the disease. Correlations were evidenced between ICSC and haemolytic activity of the classical and alternative pathways of complement, levels of the complement components C3 and C4, and incidence of circulating immune complexes. Low values of ICPIC were demonstrated almost only in sera of patients with active SLE; in fact, ICPIC was impaired in 6 sera only, and five of them were collected in the acute phase. All sera with decreased ICPIC also displayed a very low level of the C4 complement component.

Antigen-Antibody Complex↗

PubMed1978

Association of systemic lupus erythematosus and SLE-like syndromes with hereditary and acquired complement deficiency states.

The most prominent association of rheumatic diseases with hereditary complement deficiency is systemic lupus erythematosus (SLE) and discoid lupus erythematosus with homozygous C2 deficiency in females. The lupus disease in these patients differ from classic lupus in 1) the increased incidence of discoid lesions, 2) the low incidence of renal disease, 3) the low or absent titers of antibodies to native DNA, and 4) the infrequent finding of immunoglobulin and complement in skin lesions. The strong positive linkage disequilibrium between C2 deficiency and HLA genes raises the possibility that genes other than those determining C2 levels may have the primary role in determining predisposition to disease in these patients. However, the finding of similar diseases in certain patients with hereditary angioedema and SLE-related syndrome who have acquired deficiency of the early components of complement supports a primary role of the C2 deficiency gene in predisposing to lupus disease in these patients.

Arthritis, Rheumatoid↗

PubMedDate not supplied

Acute pancreatitis in systemic lupus erythematosus (SLE). Successful treatment with plasmapheresis after failure of prednisone.

A 33-year old Caucasian woman with SLE, who had been treated with chloroquine and non-steroidal anti-inflammatory drugs for one year, suddenly presented with a rapidly progressive exacerbation of SLE featuring fever, arthritis, cutaneous manifestations, cerebral dysfunction, pleuritis, pericarditis and pancreatitis. Clinical deterioration and a rise in the serum amylase occurred during a month of high dose prednisone treatment. Plasmapheresis, while maintaining prednisone at a constant dosage, resulted in a complete remission of all symptoms within four weeks. Plasmapheresis was discontinued and improvement was maintained whilst tapering off prednisone and adding azathioprine.

Acute Disease↗

PubMed1984

Reticuloendothelial Fc receptor function in SLE patients. II. Associations with humoral immune response parameters in vivo and in vitro.

We studied the relationship between reticuloendothelial Fc receptor function and some parameters of the humoral immune response in vivo and in vitro in 18 SLE patients. Fc receptor-mediated immune clearance correlated remarkably well with (a) a decrease of antigen specific IgG after immunization with a primary test antigen (alpha-Helix pomatia haemocyanin) (P less than 0.01) and (b) the spontaneous IgG release in vitro of B cells obtained from peripheral blood (P less than 0.01). These two parameters were significantly interrelated. Reticuloendothelial Fc receptor function was not related to serum IgG levels. The study provides evidence for an association between polyclonal B cell activation and Fc receptor-mediated immune clearance in SLE patients. The possible nature of this association is discussed.

Adult↗

PubMed1983

Myocardial infarction in four young patients with SLE.

Four patients with chronic systemic lupus erythematosus (SLE) in whom myocardial infarction occurred at an unusually early age are described. The evidence suggests that the coronary occlusion was due to atherosclerosis. There was no evidence that active arteritis played any role. The only risk factor for atherosclerotic disease was hypertension. All patients had had both central nervous system and renal disease and had been taking corticosteroids for a minimum of 9 years. It is suggested that hypertension aggravated by chronic corticosteroid administration may be an important risk factor for atherosclerosis in patients with SLE.

Adolescent↗

PubMedDate not supplied

Penicillamine and the SLE syndrome.

Penicillamine-induced systemic lupus erythematosus (SLE) and penicillamine-immune complex nephritis are histologically and serologically distinct. One hundred and twenty patients with Wilson's disease treated with penicillamine have been analyzed. Eight developed the serologic changes of SLE; in 4, it was necessary to discontinue treatment. In addition, 6 patients developed immune complex nephritis, which necessitated discontinuing treatment. All but 1 have since been managed on triethylene tetramine 2HCl (Trien).

Adolescent↗

PubMed1980

SLE: idiopathic or drug-induced?

Procainamide has become the most frequent cause of the drug-induced lupus syndrome. Like other drugs that produce this syndrome, procainamide induces disease that closely resembles idiopathic SLE but differs from it in race and sex distribution, in rare involvement of the kidneys and central nervous system, and in the absence of antibody to native DNA. Although complete remission of the signs and symptoms of the disease occurs in most patients following discontinuation of the drug, some patients continue to be symptomatic and require treatment with corticosteroids. With prolonged procainamide therapy, antinuclear antibody develops in at least 50% of patients, and the lupus syndrome develops in approximately 20%. At present, it does not appear that a cumulative or daily dose of procainamide can be exclusively implicated in the appearance of antinuclear antibody or symptoms. Acetylation status may be one of the determining factors in the development of the SLE syndrome.

Adult↗

PubMed1980

Skin immunoflorescence in patients with systemic lupus erythematosus, SLE. Relationship between the immunohistochemical pattern and systemic activity.

19 SLE patients between 12 and 18 years of age were studied. In all cases a renal histological study was performed and patients received prednisone daily or on alternate days; 10 also received azathioprine. Patients were hospitalized for disease activity assessment from the clinical and biochemical standpoints. A healthy skin biopsy was simultaneously performed in the antero external face of the forearm and the fragment was subject to direct immunofluorescence study. IgG, IgM or C3 granular deposits were fund in six patients (33 per cent). Presence, type and intensity of deposits were related with other clinical and biochemical activity aspects, type of nephropathy, evolution and treatment time, and none was statistically significant. We can conclude that immune deposits in the skin of SLE patients can be useful as an auxiliary method for the diagnosis of this disease not having a relationship with the type and severity of the nephropathy or clinical activity of the disease.

Adolescent↗

PubMed1995

The use of single photon emission computerized tomography in neuropsychiatric SLE: a pilot study.

OBJECTIVE: To test the sensitivity of single photon emission computerized tomography (SPECT) in detecting brain abnormalities in cases of definite active neuropsychiatric systemic lupus erythematosus (NPSLE) in a blinded, prospective pilot study. METHODS: Fourteen patients fulfilling at least 4 of the American College of Rheumatology criteria for the classification of SLE plus positive serology manifested by either elevated DNA binding or decreased serum complement and a recent neuropsychiatric event were evaluated with cerebral SPECT using hexa-methyl-propylene-amine-oxime labeled 99Tc. Secondary causes such as infection, uremia, hypertension, drugs, and metabolic abnormalities were excluded. Patients underwent brain scan and electroencephalogram (EEG) for comparison. When clinically indicated, CT scan, magnetic resonance imaging, angiography, and lumbar puncture were performed. RESULTS: SPECT scan abnormalities were noted in 12/14 patients and brain scan was abnormal in 12/14 patients. SPECT and brain scan were in accordance in 12/14 patients (11 patients both positive and 1 both negative) and the combination of SPECT and brain scan yielded 13/14 positive results. In the 3 patients with headache, SPECT scan was negative in 2/3, despite positive EEG and one with a positive brain scan. The positive SPECT in the patient with headache showed an old cerebrovascular accident (CVA), which was confirmed by CT scan. The most consistent CT finding was cortical atrophy; however, SPECT identified a lesion in the occipital cortex in a patient with seizure, and a lesion in the basal ganglia in a patient with ataxia. CONCLUSION: In clinically and serologically active NPSLE, SPECT is a sensitive diagnostic tool. When further stratifying NPSLE into focal (seizure, ataxia, CVA) and diffuse (headache, organic brain syndrome, psychosis), SPECT appeared to be sensitive for focal disease and for most diffuse manifestations, with the exception of headache. The high sensitivity of SPECT in patients with true, positive NPSLE merits further controlled studies in unselected patients with SLE.

Adult↗

PubMed1995

[Left atrial ball thrombus in the SLE patient without organic mitral lesion].

A case of 77-year-old female patient with the left atrial ball thrombus without organic mitral valve lesion is reported. She had a history of SLE for one year and experienced pericarditis 2 months before this admission. A 2-D echo examination at that time did not show any abnormal shadow in the left atrium. The repeated 2-D echo on this admission demonstrated peduncular left atrial thrombus of 2 x 1.5 cm in size. Surgical removal of the thrombus was performed successfully. Her cardiac rhythm was atrial fibrillation, but other possible factors related to the intracardiac thrombus formation such as bradycardia, left atrial dilatation or low cardiac output were not noted. Although lupus anticoagulant and anticardiolipin antibody were negative before the operation, participation of SLE in formation of the left atrial thrombus was highly suspected.

Aged↗

PubMed1995

SLE and HIV infection, two systemic conditions that can be clinically indistinguishable.

Systemic lupus erythematosus (SLE), the prototypic auto-immune disease, commonly affects Black patients. This connective tissue disease may involve the lymphoid organs, kidneys, skin, lungs and the central nervous system. The same organs are involved in HIV disease. We report on a patient who fulfilled both the American Rheumatism Association (ARA) (1982) criteria for the diagnosis of SLE and WHO (Bangui) clinical criteria for the diagnosis of AIDS in Africa. The patient had no laboratory evidence of HIV infection. The pitfalls associated with reliance on cheap guidelines are discussed in the context of the resources required for the effective differentiation of the disparate systemic conditions.

Adult↗

PubMed1995

[Optic neuropathy in systemic lupus erythematosus (SLE)--analysis of 54 cases including our case].

A 46-year old woman with systemic lupus erythematosus (SLE) who had been treated with oral administration of prednisolone (7.5 mg/5 mg on alternate days) at our outpatient clinic suddenly developed a loss of visual acuity, visual field defect, and photophobia on Dec. 15th, 1993. When she was admitted three days later, she was diagnosed as having optic neuropathy, and immediate treatment with methylprednisolone (1 g/day for three days) followed by prednisolone (50 mg/day) resulted in complete disappearance of the above symptoms by Feb. 9th, 1994. As far as we know, fifty-three cases of SLE with optic neuropathy have been reported previously. The result of analysis of the 54 cases including our case indicates that rapid diagnosis and corticosteroid treatment in an early phase of optic neuropathy leads to significant improvement.

Female↗

PubMed1995

Dyspnea in ambulatory patients with SLE: prevalence, severity, and correlation with incremental exercise testing.

OBJECTIVE: We sought to determine the prevalence and severity of dyspnea, and to correlate dyspnea with clinical features and exercise limitation in ambulatory patients with systemic lupus erythematosus (SLE). METHODS: Twenty-five consecutive patients were evaluated with a validated pulmonary questionnaire, chest radiograph, 2-dimensional echocardiography, resting pulmonary function tests, and incremental exercise testing. RESULTS: Dyspnea was reported by 60% (95% CI 39-79) of patients; 20% (95% CI 7-40) had severe dyspnea (inability to dress without dyspnea) and 12% (95% CI 3-31) had moderate dyspnea (dyspnea after walking 100 yards). Compared to patients without dyspnea, patients with dyspnea were more likely to have had a history of clinical lupus involving the lung (80 vs 40%, p = 0.05), a lower total lung capacity (77.5 vs 94.8%, p = 0.002), and a reduced maximum oxygen consumption (VO2max of 53.4 vs 67.7%, p = 0.01). Patients with severe dyspnea and patients without dyspnea did not differ in duration of prednisone use, activity of disease, weight, or in frequency of Raynaud's phenomenon (p > 0.05). Only 4% of all patients had abnormal left ventricular motion on 2-dimensional echo; patients with moderate or severe dyspnea had normal left ventricular motion. Of the 5 patients with severe dyspnea, 4 (80%) had restrictive lung disease and 1 (20%) had an isolated diffusion defect. All patients with dyspnea had an abnormal exercise test, but so did 9/10 without dyspnea (p > 0.05). Severity of dyspnea correlated highly with maximum exercise tolerance measured by VO2max (R2 = 0.51, p = 0.0001). CONCLUSION: In ambulatory patients with SLE, dyspnea is common, frequently disabling, associated with a history of lupus involvement of the lung, and correlates highly with objective measures of exercise limitation.

Adult↗

PubMed1994

[Limb myokymia with involuntary finger movement caused by peripheral neuropathy due to systemic lupus erythematosus (SLE)--a case report].

A 24-year-old woman with SLE since the age of 12 developed sensory-motor peripheral neuropathy in 1990. Her sensory neuropathy was symmetrical in all limbs and distally dominant. Electrophysiological study showed marked reduction of motor and sensory conduction velocities. Sural nerve biopsy revealed vasculitis and axonopathy. Symptoms of neuropathy and nerve conduction velocities were improved by high dose steroid treatment. After one year she had a fever of unknown origin, and then presented limb myokymia with involuntary finger movement which was fine, irregular, and continuous. Needle EMG revealed myokymic discharge at dorsal interosseous and flexor pollicis brevis muscles. Because myokymia was increased by hyperventilation and suppressed by local anesthesia at the wrist, her myokymia was thought to originate in the peripheral nerve. This is the first case report of limb myokymia with finger involuntary movement caused by peripheral neuropathy due to SLE.

Adult↗

PubMedDate not supplied

Frequency and clinical significance of anti-RNP antibodies in Italian SLE patients.

The frequency and clinical significance of anti-RNP antibodies in patients with systemic lupus erythematosus (SLE) are still a matter of debate. In this study we report our experience with a series of 123 unselected Italian patients (93 adults and 30 children) suffering from SLE according to the ARA criteria. Anti-RNP were detected by counterimmunoelectrophoresis in 25 patients (20%), 19 of whom did not show other antibodies to extractable antigens (isolated RNP). Our study shows a striking association between anti-RNP and arthritis as well as between anti-RNP and Raynaud's phenomenon. In addition, we found an association between anti-RNP and lack of renal involvement. This association was limited to those patients with isolated RNP and was independent of the presence of anti-dsDNA antibodies. These data were confirmed by a 50 month follow-up study.

Adolescent↗

PubMed1994

[Hypopituitarism associated with empty sella after steroid pulse therapy in a patient with SLE].

A case of systemic lupus erythematosus (SLE) complicated with hypopituitarism after steroid pulse therapy is reported. A 46-years-old-female with a history of SLE starting in 1975 was admitted to our hospital in February 1991 for lupus nephritis. Steroid pulse therapy, 1000 mg methyl-prednisolone for 3 successive days as one therapy unit, was administered. Proteinuria improved remarkably, however, general fatigue and headache appeared 2 weeks after initiation of therapy. Endocrinological examination revealed hypopituitarism including the levels of TSH, FSH, GH and ACTH. The secretion of FSH and LH gradually improved after replacement therapy of dried thyroid. MRI examination of the brain revealed an empty sella. It is known that pituitary tumor, cerebrovascular accident and autoimmune lymphocytic hypophysitis cause hypopituitarism. In this case, it is unlikely that the pulse therapy may be responsible for the infarction of the anterior pituitary artery furthermore, there has been no articles describing such incidence after steroid pulse therapy. This case may be indicative of a very rare case in which the empty sella might have been exacerbated by the pulse therapy in the causation of hypopituitarism.

Drug Administration Schedule↗