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Mortality and causes of death in systemic lupus erythematosus.

Cohort studies of survival in systemic lupus erythematosus (SLE) often have been limited by methodologic problems. In studies of inception cohorts of patients followed since 1980, survival at 5 years has exceeded 90%. These estimates are generally higher than survival estimates from earlier studies, suggesting that short-term survival in SLE has improved. There is less evidence to support major improvements over time in survival after 10 years or more of SLE. Infections, atherosclerotic disease, and active systemic lupus erythematosus or organ damage caused by SLE are the main causes of death in patients with SLE, but the proportion of early deaths caused by active SLE has decreased over time.

Bacterial Infections↗

Adaptation to chronic pain in systemic lupus erythematosus: applicability of the multidimensional pain inventory.

OBJECTIVES: 1) Investigate psychosocial adaptation to chronic pain in patients with systemic lupus erythematosus; 2) Compare pain adaptation of lupus, chronic low back pain, and temporomandibular disorders patients; and 3) Evaluate the validity of Multidimensional Pain Inventory-based profiles of lupus patients. METHODS: Two hundred forty females with pain related to systemic lupus erythematosus (N =80), chronic low back pain (N=80), or pain related to temporomandibular disorders (N=80) completed the Multidimensional Pain Inventory, a 60-item psychosocial assessment instrument. All patients were classified into empirically derived profiles based on Multidimensional Pain Inventory responses. Systemic lupus erythematosus profile groups were compared on conceptually related variables external to the classification, including indices of lupus disease activity, pain, distress, and physical function. RESULTS: The scores of lupus patients resembled those of temporomandibular disorder patients, while chronic low back pain patients had higher pain and activity interference. 87.5% of lupus patients could be classified into one of three profiles: Dysfunctional (14%), interpersonally distressed (27.5%), or adaptive coper (46%). The Multidimensional Pain Inventory profiles for lupus patients demonstrated validity, based on external measures. CONCLUSIONS: Although many systemic lupus erythematosus patients cope well with their chronic pain, a substantial proportion exhibit pain-related distress, activity interference, or interpersonal difficulties.

Adaptation, Psychological↗

Splenic atrophy in systemic lupus erythematosus.

Five of 70 patients with systemic lupus erythematosus seen over a 5-year period had peripheral blood films suggestive of hyposplenism. Technetium-99m-sulfur colloid scans showed no splenic activity in three patients. One of these three patients, who had a spontaneous remission of persistent thrombocytopenia concurrent with the appearance of functional asplenia, had splenic atrophy shown by computed tomography; a second patient, who died of pneumococcal septicemia, was shown to have splenic atrophy with lymphocyte depletion at autopsy. The development of functional asplenia in the third patient has now, to date, had any obvious effect on her disease. In the two patients with normal scans, blood film findings returned to normal with treatment of the underlying disease. Polyvalent pneumococcal vaccine is recommended for patients with lupus erythematosus who have splenic atrophy.

Adolescent↗

Neonatal lupus erythematosus related to maternal leukocytoclastic vasculitis.

Neonatal lupus erythematosus (NLE) is an autoimmune disease whose major findings are skin lesions and congenital heart block. Affected infants have maternal, transplacentally acquired, autoantibodies to Ro/SSA, La/SSB, or U1-RNP antigens. Anti-Ro/SSA is the predominant autoantibody, present in about 95% of cases. Mothers of babies with NLE may be asymptomatic initially or may have Sjögren syndrome, lupus erythematosus, overlap syndrome or, uncommonly, leukocytoclastic vasculitis. When evaluating a young woman with a cutaneous leucocytoclastic vasculitis, dermatologists should be aware of the possible presence of antibodies related to NLE. If any patient suffering a disorder related to NLE becomes pregnant, testing for autoantibodies and close obstetric prenatal care with fetal echocardiogram is necessary. In cases of fetal bradycardia, treatment with dexamethasone or betamethasone should be considered, as these drugs are accessible to the fetal circulation.

Adult↗

Calcifying lupus panniculitis in a patient with subacute cutaneous lupus erythematosus: response to diltiazem and chloroquine.

Lupus panniculitis has been reported to occur with a frequency of 2-3% in patients with lupus erythematosus (LE). It is most often reported in association with lesions of discoid LE. We describe a patient with subacute cutaneous LE who developed calcified nodules that were histopathologically consistent with lupus panniculitis. She was treated with a combination of chloroquine and diltiazem with a good therapeutic response. The addition of diltiazem may be beneficial in patients with calcified nodules of lupus panniculitis.

Adult↗

Gender impact in systemic lupus erythematosus.

OBJECTIVE: Systemic Lupus Erythematousus (SLE), an autoimmune disease of unknown etiology manifesting as a pleomorphic systemic disease, affects mostly females, (female:male ratio 9:1). Clinical differences between genders, including a higher death rate in males, has been reported. Here we compared clinical manifestations and the 5-year survival probability in Mexican male and female crossbred cases living under similar socioeconomic conditions. A systematic review of published literature was also carried out. MATERIAL AND METHODS: SLE patients treated at the Instituto Nacional de Cardiología "Ignacio Chávez" México City who fulfilled at least four classification criteria (ACR) were included. The frequency of clinical variables with emphasis on cardiovascular findings before and after the diagnosis were described, disease activity based on a validated scale (SLEDAI) was determined, and the 5-year survival rate was estimated. RESULTS: There were 33 men and 158 women, average age of 31 in both groups ranging from 7 to 65 and from 10 to 75 year in male and female patients respectively; both groups were followed for 3.8 years (median), average activity was of 12 points with a range of 5 to 23 in men, and 11 with a range of 2 to 24 in women. Main clinical characteristics in men were: discoid lupus, psychosis, pericarditis, lymphopenia, thrombocytopenia and SLE kidney disease. Immunological tests showed gender-linked differences in regard auto-antibodies (U1-nRNP, Sm, anticardiolipine and false VDRL) and hypocomplementemia. Cardiovascular features and survival rate were not different between gender. CONCLUSION: Male Mexican SLE patients share clinical findings with other male SLE cases reported everywhere as can be deducted from systematic literature review covering 25-year.

Female↗

Increased superoxide production by polymorphonuclear leukocytes in systemic lupus erythematosus.

Normal and lupus PMN show an enhancement in superoxide production in vitro when stimulated with lupus serum. When N-formyl-methionyl-leucyl-phenylalanine (FMLP) was used, lupus PMN showed an O2- production of 2.1 nmol/min/10(7) cells, which is 5.2 times the response of normal PMN stimulated by FMLP. Our results show the existence of serum factors in SLE patients that can stimulate O2- production by PMN. Lupus neutrophils showed an increased response to membrane stimuli such as FMLP, capable of triggering the cell respiratory burst. Lupus neutrophils appeared more responsive to membrane stimuli. The serum and cellular factors seemed to indicate an increase rate of superoxide production by PMN in lupus patients, which could be relevant factors in the development of vasculitis and tissue damage.

Chemotactic Factors↗

Early detection of neurological involvement in systemic lupus erythematosus patients.

In lupus the neurological abnormalities usually mimic many primary neurological diseases and often represent the only manifestation of SLE for several years before the onset of other features of the disease. The aim of this study was to investigate lupus patients to obtain electrophysiological data of possible neurological abnormalities even in the absence of neuropathy symptoms. Thirty-eight SLE patients, twenty healthy volunteers and 10 steroid-using control subjects were included. Eighteen were asymptomatic but 20 of them had neuropathy symptoms. Median, ulnar and peroneal nerve motor, median, ulnar and sural nerve sensory conduction velocities, median and tibial somatosensory evoked potentials (SEP) were recorded. 23.6% of all lupus patients had peripheral nerve conduction slowing and 39.5% had SEP abnormalities; almost half of them being asymptomatic. 44.7% of the total had carpal tunnel syndrome; again almost half of them (47%) were asymptomatic. There was no significant correlation between the electrophysiologic parameters and the clinical and laboratory parameters of the disease. In lupus patients, SEP abnormalities were more pronounced than peripheral findings, reflecting the CNS involvement more common than the peripheral neuropathy. Therefore, we suggest performing the electrophysiological studies in lupus patients, even in the asymptomatic ones, early in the course in order to detect the nervous system involvement.

Adolescent↗

Papulonodular mucinosis with subacute cutaneous lupus erythematosus.

A 30-year-old woman with subacute cutaneous lupus erythematosus presented with a facial eruption, painful and swollen ulcers of her digits, and asymptomatic lesions on her abdomen. The laboratory findings were consistent with subacute cutaneous lupus erythematosus with an anticardiolipin antibody. A biopsy specimen of the asymptomatic lesions on her abdomen showed mucin within the upper reticular dermis. Papulonodular mucinosis is a rare but well-documented finding associated with systemic and cutaneous forms of lupus erythematosus. The etiology and pathogenesis of this finding remain to be elucidated.

Adult↗

Antibody-dependent cellular cytotoxicity of human vascular endothelium in systemic lupus erythematosus.

Sera from 35 patients with systemic lupus erythematosus were examined for a cytotoxic effect on human umbilical vein endothelium. Although none of these sera produced direct cytotoxicity of 51Cr-labelled endothelial cells, even with added complement, 3 sera regularly produced increased 51Cr release when co-cultured with endothelial cells and normal human peripheral blood mononuclear cells. The effector cells involved in this cytotoxicity possessed Fc-receptors but were non-T and non-adherent while fractionation studies indicated that the responsible serum factor(s) was IgG, probably in the form of immune complexes of small size. Control studies, using sera from both 27 normal controls and 19 patients with either diabetes or extensive atherosclerotic vascular disease failed to reveal any similar cytotoxicity. Two of the 3 patients, whose sera produced this antibody-dependent cellular cytotoxicity, had had clinical episodes of major vascular thrombosis, raising the possibility that the cytotoxicity might provide an additional pathogenic mechanism in certain patients with systemic lupus erythematosus.

Adult↗

[Cutaneous lupus erythematosus following argon laser treatment].

BACKGROUND: It is well known that exposure to ultraviolet light can trigger lupus manifestations. Other light sources may have the same effect. We report a case of argon laser-induced lupus erythematosus. CASE REPORT: A 59-year-old women developed an erythematous edematous infiltrated and sensitive lesion over the right cheek ten days after an argon laser treatment of the retina. The lesion spread towards the chin despite antibiotic treatment. Histology examination of a biopsy specimen and direct immunofluorescence suggested the diagnosis of cutaneous lupus erythematosus. The lesions regressed in one month with hydroxychloroquine (400 mg/d) treatment. DISCUSSION: Our patient developed argon laser induced cutaneous lupus erythematosus. It is known that ultraviolet light and non-ultraviolet frequencies (x-rays, visible light) can induce lupus manifestations. One case of discoid lupus erythematosus after argon laser has been reported. In our case, due to a technical error the laser beam was directed onto the ipsilateral cheek during the laser treatment of the retina. The low-energy beams used in ophthalmology would explain the absence of local burn but would be sufficient to trigger lupus. This case demonstrates that argon laser, a visible blue or green beam, can provoke cutaneous lupus erythematosus even if there is no heat-induced burn. It is important to be aware of this adverse effect due to the widespread use of lasers in dermatology, particularly for the treatment of cutaneous lupus lesions.

Argon↗

Influenzal vaccine response in systemic lupus erythematosus.

The response of patients with systemic lupus erythematosus and normal subjects to systemic immunization and boosting with influenza A vaccines was studied. Symptoms after vaccination were somewhat more frequent in the patients than in the normal subjects; however, all symptoms were minor and no major flare of illness occurred. No significant induction or increase of pre-existing autoantibodies among the patients was detected after vaccination. The immunogenecity of the vaccinations, as assessed by antibody titers, was similar in the patient and control groups. No correlation between serologic response to influenzal antigens and HLA was found. Thus, in this group of patients with systemic lupus erythematosus, who were either in remission or had mild-to-moderate disease activity, killed influenzal vaccination caused no apparent worsening of disease activity.

Adult↗

Subacute cutaneous lupus-erythematosus in childhood.

Subacute cutaneous lupus erythematosus (SCLE) has been described in adults in the last decade and represents a particular homogenous subgroup in which the skin lesions can be distinguished from scarring discoid lupus lesions both on a morphologic and histopathologic basis. This entity, however, seems to be very uncommon in childhood. In the present study we describe the clinical, immunological and histopathologic characteristics of a young girl who, at the age of 3, developed SCLE.

Antibodies, Antinuclear↗

Mechanisms of cutaneous tissue damage in lupus erythematosus.

Cutaneous disease in lupus is clinically heterogeneous, and it is likely that mechanisms of disease are also heterogeneous. Nevertheless, in many cutaneous forms of lupus and in the systemic disease itself, there is compelling evidence that autoantibodies are involved. It is our belief that autoantibodies are of primary importance in the etiology of the cutaneous lupus lesions discussed in this chapter and that antibody specificities are critical in most cases in determining the clinical expression of disease. As discussed earlier, antibodies can effect tissue injury by several mechanisms. There is evidence that ADCC may be an important mechanism of keratinocyte cytotoxicity in cutaneous lesions characterized by antibody deposition and a mononuclear cell infiltrate. Vasculitic lesions that have an infiltrate of polymorphonuclear cells may be related to antibody- and complement-dependent neutrophil-mediated endothelial cytotoxicity. Ultraviolet light, sex steroids, and certain genes such as those of the major histocompatibility complex appear to be important modulating factors in lupus.

Adult↗

Serological levels of apoptotic bodies, sFAS and TNF in lupus erythematosus.

In our study we have investigated the presence of apoptotic bodies, soluble FAS receptor and TNF (tumor necrosis factor) in three clinical forms of lupus erythematosus. Determinations were performed in attack period of: systemic lupus erythematosus (SLE) for 20 patients, 20 patients with subacute cutaneous lupus erythematosus (SCLE), 20 patients with chronic discoid lupus erythematosus (DLE). Determinations were performed by ELISA (for apoptotic bodies, kit Boehringer, normal values 400-800 mU), (for sFAS, kit R&D Systems, normal values 4500-17000 pg/ml) (for TNF, ELISA kit R&D Systems, normal values 0.4-3.6 pg/ml). Results in SLE: apoptotic bodies were increased in 16 cases (980-1030); sFAS in 18 cases (17000-24000 pg/ml) TNF was increased in all 20 cases (40-140 pg/ml). In SCLE with multiple cutaneous lesions and without internal organs disturbance the apoptotic bodies were increased in 10 cases (960-1030 pg/ml), sFAS in 9 cases (17000-22000 pg/ml), and TNF alpha in 9 cases. In DLE, apoptotic bodies were increased in 2 patients (980-1010 pg/ml), sFAS in 3 patients (17000-20000 pg/ml) and TNF in 2 patients (20-40 pg/mil). Investigated values were slightly correlated with immune parameters (anti dsDNA antibodies), but they were correlated with the presence of renal disturbances or extension of cutaneous lesions. We consider that the presence of increased apoptotic bodies as a result of peripheral mononuclear cells apoptosis appear as a nauto-limiting mechanism in a pathological immune response. The increase of sFAS in lupus patients serum might be interpreted as an alteration of apoptosis respectively a deficit in apoptosis which has as a first consequence the persistence of B and T lymphocytes, activated, in the pathogen immune response.

Apoptosis↗

Central nervous system vasculopathy in neonatal lupus erythematosus.

Central nervous system involvement in neonatal lupus erythematosus (NLE) has not been previously reported. We report four patients with NLE, all with complete congenital heart block and three with cerebral ultrasound and color Doppler flow imaging (CDFI) studies demonstrating evidence of associated vasculopathy in the gangliothalamic vasculature. CDFI confirmed blood flow through the affected vessels, indicating that blood flow was not compromised at this early stage. Short-term follow-up revealed no signs of progression of the vasculopathy, focal ischemia, gangliothalamic atrophy, or neurological impairment. Nevertheless, the implications of this finding with respect to the natural history of NLE remain to be defined, particularly in cases in which the disease develops into systemic lupus erythematosus later in life. Besides specific diagnostic studies for NLE, cerebral ultrasound, and CDFI studies are mandatory in all cases of complete congenital heart block, regardless of whether mothers are diagnosed as having connective-tissue disease or not. Neonates with signs of vasculopathy in the gangliothalamic region should be examined for NLE.

Autoantibodies↗

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↗