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Pulse corticosteroid therapy for refractory thrombocytopenia in systemic lupus erythematosus.

A patient with systemic lupus erythematosus and severe thrombocytopenia was refractory to therapy with prednisone, alone or in combination with cyclophosphamide or vincristine. Intravenous pulse therapy with methylprednisolone was associated with an increased platelet count from 4,000/mm3, to 52,000/mm3, but the response was short-lived. A second course of pulse therapy was also successful, and splenectomy was safely performed. The platelet count has remained normal for 15 months after surgery, allowing prednisone to be tapered and discontinued. Intravenous pulse therapy with methylprednisolone may be a safe alternative in preparing patients with profound, refractory thrombocytopenia for splenectomy.

Adult↗

Optic neuritis in systemic lupus erythematosus.

Four patients with systemic lupus erythematosus (SLE) and optic neuritis are described. Optic neuritis is uncommon in SLE but may be the presenting feature of the disease. A review of 8 similar cases in the literature indicates that myelopathy is frequently associated, prompting the diagnosis of multiple sclerosis. Visual loss may be permanent despite therapeutic intervention, but rapid improvement occurs in some patients who receive early treatment with corticosteroids.

Adolescent↗

Bacterial monarthritis due to Neisseria meningitidis in systemic lupus erythematosus.

Two patients with systemic lupus erythematosus developed monarticular infectious arthritis, in which Neisseria meningitidis was recovered from knee synovial fluid. In one instance, the sole manifestation of meningococcal disease was a chronic, indolent, erosive monarthritis. In the second, a febrile, bacteremic illness presented with an acute, septic arthritis.

Adult↗

Total hip arthroplasty for avascular necrosis of the femur in systemic lupus erythematosus.

Six patients with systemic lupus erythematosus and avascular necrosis of the femoral head were treated by total hip arthroplasty (THA). The primary indication for surgery was persistent hip pain despite conservative therapy. Postoperatively, pain was relieved with corresponding improvement in range of motion in all six patients. There were no serious complications. At follow-up ranging from 23 to 76 months after surgery there was no recurrence of hip pain. These results suggest that THA is indicated in selected SLE patients.

Adult↗

Pulmonary hypertension and systemic lupus erythematosus.

A combination of systemic lupus erythematosus (SLE) and fatal pulmonary hypertension occurred in a patient who, to our knowledge, had the highest pulmonary artery pressure (120/65 mm Hg) reported without any clinical or autopsy findings of pulmonary interstitial disease or vasculitis. The gradual development of pulmonary hypertension over years is a rare complication in patients with SLE.

Adult↗

An infected ovarian hematoma as the presenting symptom of systemic lupus erythematosus.

A patient with systemic lupus erythematosus (SLE) and an associated coagulation defect presented with the clinical picture of an inflammatory pelvic mass. The laboratory data led to the diagnosis of SLE. A laparotomy revealed an infected hematoma of the left ovary. The SLE-induced coagulation deficit may have caused an intraovarian hematoma at the time of ovulation that became infected secondarily and led to an inflammatory pelvic mass as the first sign of SLE.

Adult↗

Polymyositis and polyarthritis associated with systemic lupus erythematosus in a dog.

Systemic lupus erythematosus with polymyositis and polyarthritis was diagnosed in a 7-year-old female Standard Poodle. Pertinent clinical signs included extreme muscular weakness, muscle wasting, atrial fibrillation, and dysphagia due to megaesophagus. Aspiration pneumonia secondary to the megaesophagus contributed to the death of the dog. Serum muscle enzyme activities were increased. Electromyographic findings included fibrillation potentials, positive sharp waves, increased insertional activity, and bizarre high-frequency repetitive potentials. Histopathologic findings in skeletal muscle included myofiber necrosis and phagocytosis; regeneration of myofibers; perivascular and interstitial infiltrations of macrophages, lymphocytes, and plasma cells; and type I and II myofiber degeneration and vacuolation.

Animals↗

Acute syphilitic meningitis in a patient with systemic lupus erythematosus.

A man with systemic lupus erythematosus receiving chronic steroid therapy presented with headache, fever and panophthalmitis. Cerebrospinal fluid analysis was consistent with syphilitic meningitis. This represents the first reported case of syphilitic meningitis in a lupus patient. Our case report highlights the need to remain alert for unusual manifestations of syphilis in immunosuppressed patients.

Acute Disease↗

[Relationship between higher cortical dysfunction and the findings of magnetic resonance imaging in systemic lupus erythematosus].

The relationship between systemic lupus erythematosus (SLE) and organic lesions was investigated by magnetic resonance imaging (MRI) to clarify the etiology of higher cortical dysfunction in SLE. The subjects were 10 patients with SLE, and higher cortical dysfunction was observed in 8 (80%) of the 10 patients. 5 (82.5%) of the 8 patients showed abnormal MRI findings. The findings of higher cortical dysfunction were consistent with the MRI findings in 1 of the 5 patients, but not in the remaining four. MRI revealed no lesion despite the presence of higher cortical dysfunction in three patients. These results suggest that the association of organic changes and functional changes in cerebral nerve cells is important in etiology of higher cortical dysfunction in SLE.

Adult↗

[The ocular manifestations in systemic lupus erythematosus].

Retinal involvement in systemic lupus erythematosus is dominated by a microangiopathy of unknown aetiology, with occlusive consequences. Although cases of severe retinal ischaemia have been recently described, from a statistic point of view, the occlusive phenomena seem to have declined over the last decades, under the influence of a better therapy and follow-up of the disease. The relatively high incidence of papilloedema and the fact that this oedema might be unilateral or asymptomatic are pointed out. The importance of ophthalmic examination in patients with SLE-like syndromes or in seronegative cases of SLE is revealed. Other ocular manifestation of the disease are reviewed.

Eye Diseases↗

Psychiatric disorders in Systemic Lupus Erythematosus.

The symptoms of Systemic Lupus Erythematosus (SLE) may include altered mental function. The present study sought to determine whether the psychiatric disorders are due to the disease itself or to the stress of having a chronic disease. Forty-five SLE patients attending outpatient clinics at the Port-of-Spain General Hospital in Trinidad were compared with two control groups: patients with chronic debilitating diseases similar to SLE in terms of chronicity and treatment (n = 44) and non-diseased individuals (n = 48). The Structured Clinical Interview for DSM III-R was used to identify psychiatric disorders. Both the SLE and the chronic illness groups had more psychiatric illness (44% and 39%, respectively) when compared with the non-diseased controls (2%) (p < 0.001). Major depression was the most common diagnosis among both diseased groups. However, psychotic illnesses (schizophrenic-type psychosis and bipolar disorders) were more prevalent in the SLE group (11.1% vs 0%, p = 0.02). These results indicate that major depression in SLE may be related more to the effects of a chronic illness than to SLE itself. However, the occurrence of psychotic symptoms may be related to SLE disease and needs further study.

Adaptation, Psychological↗

HIV infection in a male patient with systemic lupus erythematosus.

The association between systemic lupus erythematosus and human immunodeficiency virus infection is extremely rare. We report a case of this association and discuss the possible reasons for its rarity, together with the difficulties encountered in the interpretation of the clinical and laboratory features.

Acquired Immunodeficiency Syndrome↗

Herpes zoster myelitis occurring during treatment for systemic lupus erythematosus.

A woman with systemic lupus erythematosus (SLE) presented with a zoster eruption. Transverse myelitis developed at the site of the dermatomal distribution of the rash. SLE and varicella zoster virus (VZV) can both cause myelitis, and are difficult to differentiate. The topographic association between the cutaneous and the neurological involvement suggesting VZV myelitis was confirmed by polymerase chain reaction (PCR) for VZV in the cerebrospinal fluid. This case illustrates the potential role of the selective amplification of VZV DNA in cerebrospinal fluid to diagnose central neurological complications associated with VZV. The value of magnetic resonance imaging of the spinal cord in the evaluation of patients with myelitis is emphasized.

DNA, Viral↗

Seronegative systemic lupus erythematosus.

The diagnosis of systemic lupus erythematosus (SLE) often depens on positive tests for antinuclear antibody, LE cells, and antibody to DNA. In fact, the presence of such serological markers is virtually required for the diagnosis of active disease. This report describes 5 well-studied patients with SLE who initially presented with negative serology despite active multisystemic disease. When followed from 10 months to 7 yr, negative serologic markers, including the ANA, LE cell test, and anti-DNA were seen to change from negative to positive, analogous to the seronegative rheumatoid patient who may become seropositive. In a few cases of lupus, therefore, negative serologic tests do not always exclude SLE, and serial testing at periodic intervals may be indicated.

Adult↗

Rate, pattern and factors related to damage in Brazilian systemic lupus erythematosus patients.

The Systemic Lupus International Collaborating Clinics/American College of Rheumatology (ACR) Damage Index (SDI) is an accepted instrument to ascertain damage. It has been shown to vary among different SLE populations. The aim of this study was to assess SDI score, pattern and factors related to damage in Brazilian SLE outpatients. The SDI was obtained in 105 patients with a median age of 41 (5-95%, 19-61.7) years and a median SLE duration of 127 (17.6-345.9) months. Patients had a median SDI of 2 (0-8) and 81.9% had some damage (SDI > 0). Damage was associated with a higher number of ACR criteria for SLE in multivariate analysis (OR = 2.32, 95%CI = 1.23-4.37, P = 0.009). Antiphospholipid syndrome (APS) (OR = 9.82, 95%CI = 2.74-35.23, P < 0.001), methylprednisolone pulses (OR = 3.91, 95%CI = 1.19-12.81, P = 0.024), age (OR = 1.70, 95%CI = 1.02-1.13, P = 0.011) and prednisone use duration (OR = 1.01, 95%CI = 1.002-1.02, P = 0.020) were related to severe damage (SDI > or = 4). Hypertension was associated with renal, cardiac and atherosclerotic damage, as cyclophosphamide pulses were with premature menopause. In conclusion, damage was very frequent in Brazilian SLE patients, mainly due to skin involvement, compared to other SLE populations. The presence of APS was the major independent contributor to the development of severe damage. Arterial hypertension was identified as a common risk factor for renal, cardiac and atherosclerotic damage.

Adult↗

Central nervous system disease in patients with systemic lupus erythematosus.

Central nervous system (CNS) dysfunction in patients with systemic lupus erythematosus (SLE) is highly variable, although it is often described under a single heading of "neuropsychiatric" or "CNS" SLE. To clarify these CNS abnormalities, we studied 91 lupus patients, 63 of whom had CNS symptoms or signs, over 599 patient years. By placing patients in relatively homogeneous clinical groups (stroke, seizure, suicide attempt, hallucination, confusion, decreased alertness) we detected significant (but variable among groups) correlations with other manifestations of SLE, suggesting separate mechanisms for each CNS disorder. These correlations were lost if all "CNS-SLE" was considered as a single group. Patients with decreased alertness often had undetected systemic infections and had a high death rate from infection, rather than from CNS-SLE. The understanding of the pathogenesis and potential treatment of CNS disorders in lupus will depend on classifying the patients into homogeneous groups.

Behavior↗

Thallium-201 myocardial perfusion imaging in patients with systemic lupus erythematosus.

The cardiovascular system is frequently affected in systemic lupus erythematosus (SLE). The observation of clinical manifestations related to the presence of coronary artery disease has not been frequently documented in young SLE patients. In these patients, the presence of inflammatory or thrombotic vascular lesions is often documented by anatomo-histological studies in the absence of previous clinical manifestations. The purpose of this study was to evaluate the presence of myocardial perfusion defects in SLE patients. The study was carried out in 15 patients without clinical signs of myocardial ischemia, 1 male and 14 females, 24 to 64 years old, with a mean SLE duration of 10.2 +/- 7.5 years. All the patients had normal blood pressure; electrocardiogram and Doppler-echocardiographic analysis showed values in the normal range. All the patients underwent thallium-201 exercise stress imaging repeated 3 hours later at rest, with tomographic SPECT analysis. Exercise test was carried out until submaximal load, without induction of ST segment alterations or symptoms. Scintigraphic scan showed normal thallium-201 SPECT imaging in 11/15 patients, while the other 4 patients had a slight perfusion defect, 3 of them in the inferior segment, in 2 non reversible and in 1 reversible; 1 patient had a non reversible defect in the septal segment. These slight perfusion defects, prevalently non reversible, may sometimes be a false positive imaging. Our results are in contrast with the literature observations concerning the frequent incidence of thallium-201 perfusion defects in SLE patients. In young asymptomatic SLE patients, our study does not report very important data indicating myocardial ischemia and suggesting the presence of significant coronary obstruction or vasculitis.

Adult↗

Interactions of prednisolone and other immunosuppressants used in dual treatment of systemic lupus erythematosus in lymphocyte proliferation assays.

Systemic lupus erythematosus is an autoimmune disease primarily affecting women. Currently, systemic lupus erythematosus therapy is suboptimal due to adverse effects of immunosuppressants, particularly corticosteroids. This study determines the single effects of prednisolone, dehydroepiandrosterone, bromocriptine, tamoxifen, mycophenolic acid, 2-chloro-2'-deoxyadenosine, azathioprine, and chloroquine on lectin-stimulated proliferation of human T lymphocytes, as well as determining whether there are interactions in the joint effects of prednisolone and these agents. The T lymphocytes from the whole blood of 10 middle-aged women were stimulated by phytohemagglutinin and cultured with varying drug concentrations. The Hill function was used to evaluate single-drug response data. Isobolograms were constructed to qualitatively analyze interactions. Parametric analysis based on competitive and noncompetitive interaction models was further applied to quantify the joint interactions and predict steroid-sparing potential. The surface interaction parameter (psi) estimated from parametric analysis was in concordance with isobolographic inspection for all interactions studied. All interactions favored the noncompetitive model. Results suggest that dehydroepiandrosterone is additive in its effect with prednisolone, whereas tamoxifen interacts synergistically, both providing steroid-sparing effects. Novel immuno-suppressants such as mycophenolic acid may still provide added pharmacologic benefit during therapy despite a slight antagonistic interaction with prednisolone. These studies help rationalize actual or potential use of other drugs with prednisolone in the treatment of systemic lupus erythematosus.

Adult↗