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Systemic lupus erythematosus relapse with lupus cystitis.

A 45-year-old woman who had been diagnosed as having systemic lupus erythematosus (SLE) at the age of 28 years and who had been in remission developed severe urinary frequency, watery diarrhea, vomiting and weight loss. She also developed acute renal failure and her serological examination was consistent with active SLE. She had a markedly decreased urinary bladder capacity of 20 ml with hydroureteronephrosis. Histopathological study of her urinary bladder biopsy specimen showed mucosal edema, infiltration by lymphocytes and granulocytes, and deposition of IgA in the epithelium and submucosal region. We diagnosed this as a case of lupus cystitis. The patient's symptoms were alleviated by bilateral nephrostomy and corticosteroid therapy. In the present episode the patient showed none of the usual symptoms of SLE. This case and others reported in the literature show that lupus cystitis presents with specific signs and symptoms and therefore, this syndrome may represent a specific clinical manifestation of SLE.

Acute Kidney Injury↗

Nocardiosis in patients with systemic lupus erythematosus. The Singapore Lupus Study Group.

Nocardia, a gram positive variably acid-fast aerobic bacterium is an opportunistic pathogen in immunocompromised hosts. We present 5 cases of nocardiosis in patients with systemic lupus erythematosus. We emphasize the clinical features, radiologic findings, and antibiotic sensitivity. Lung involvement was the predominant manifestation; others include brain abscess, retinitis, thyroiditis, and diaphragmatic infiltration. We describe the first cases of pulmonary nocardiosis presenting as pneumothorax and the use of fine needle aspiration cytology in diagnosing nocardial thyroiditis.

Adult↗

Hypertrophic cardiomyopathy in systemic lupus erythematosus and "lupus-like" disease. Chance association? A report of 2 cases.

Two patients, both women, one with "lupus-like" disease, age 51 years, the other a 45-year-old with systemic lupus erythematosus (SLE), developed symptoms and echocardiographic signs of hypertrophic cardiomyopathy. One patient had a family history of sudden maternal death. Neither patient had a history of sustained hypertension and there were no significant valvular lesions detectable to account for the septal and ventricular hypertrophy. The association of SLE or any related condition with hypertrophic cardiomyopathy has not been recorded. In one patient the question of a hereditary cardiomyopathy remains a possibility. The diagnosis of the condition was based on clinical and echocardiographic grounds alone. No endomyocardial biopsies were performed.

Cardiomyopathy, Hypertrophic↗

Pediatric systemic lupus erythematosus and neonatal lupus.

The clinical manifestations of pediatric systemic lupus erythematosus (SLE) are similar to those seen in adults with SLE with increased frequency of the following features: hepatosplenomegaly, chorea, nephritis, and avascular necrosis. Similarly, pediatric SLE patients are now showing the same improvement in survival as adult SLE patients, and it is no longer felt that the course of childhood-onset SLE is more severe than that seen in adult-onset SLE. Children of mothers with SLE can develop both transient and persistent features of SLE in the neonatal period. Transient features include photosensitive discoid rash, cytopenia, hepatosplenomegaly, myocarditis, and pericarditis; the permanent features include congenital complete heart block, endomyocardial fibroelastosis, and other structural cardiac defects.

Child↗

Increased factor VIIa levels in systemic lupus erythematosus patients with lupus anticoagulant.

Recurrent fetal loss, and/or arterio-venous thrombosis are frequent complications in patients with the antiphospholipid antibodies (aPL), anticardiolipin antibody (aCL) and/or lupus anticoagulant (LA). Furthermore, patients with LA have been found to be more susceptible to thrombosis than those with aCL, thus suggesting differences in the pathogenesis of aCL and LA. We examined the systemic lupus erythematosus (SLE) patients with aCL and/or LA for differences in the markers for hypercoagulable state, including thrombin-antithrombin complex (TAT), prothrombin fragment 1 + 2 (F1 + 2), thrombomodulin (TM) and activated factor VII (FVIIa), and lipoprotein (a) (Lp(a)), which is a well-known risk factor for thrombosis. The FVIIa concentration was significantly higher in the LA-positive patients than in the aCL-positive and aPL-negative patients. No significant differences in TAT, F1 + 2, TM, and Lp(a) values were found among the aCL-positive, LA-positive and LA-negative patients groups. These findings indicate that patients with LA were in a more prethrombotic state than those with aCL. The measurement of FVIIa may serve as a useful predictive marker for thrombosis, but further studies are needed to clarify the mechanisms of thrombosis in this clinical setting.

Adult↗

Psychological profiles and health status in Japanese female patients with systemic lupus erythematosus: the Miyagi Lupus Collaborative Study.

Psychological factors have been suspected to be associated with the development of systemic lupus erythematosus (SLE) and patient's health status. However, psychological profiles among Japanese patients with SLE have been poorly understood. We started a prospective study of female patients with SLE in 1995. Using the baseline data from 279 patients in this prospective study, we cross-sectionally analyzed the relations of clinical factors and social factors to psychological factors, and the association between psychological factors and mental and physical health status. We used the Japanese notion ikigai as an indicator of mental health, and ambulatory activity as an indicator of their physical health, respectively. To measure psychological factors, the short-form of the Eysenck Personality Questionnaire-Revised (short EPQ-R) and the Multidimensional Health Locus of Control (HLOC) scale were used. Active phase of the disease was significantly related to the neuroticism score in the short EPQ-R. Educational level was inversely related to the scores of powerful others and chance HLOC belief. As for health status, the internal HLOC belief was significantly associated with ikigai, and the chance HLOC belief was inversely associated with ambulatory activity. The scores on the short EPQ-R (Extraversion/Introversion and Neuroticism) were exclusively related to ikigai. This study suggests that psychological factors may have effects on both the development of SLE and patient's health status.

Activities of Daily Living↗

Late onset systemic lupus erythematosus and lupus-like disease in patients with apparent idiopathic glomerulonephritis.

We report 17 patients who presented with either apparent idiopathic glomerulonephritis (16 patients) or post-streptococcal glomerulonephritis (one patient). Doubts arose about the nature of these patients' disease, either because their initial renal histology was suggestive of systemic lupus erythematosus (SLE) in the absence of its clinical or serological features, or because they developed with time the clinical or serological features of SLE. Three patients had a positive antinuclear antibody (ANA) test at the onset of their illness, but normal levels of serum binding of double-stranded DNA (dsDNAB). In another four patients the dsDNAB was slightly raised but with a negative ANA. On renal biopsy the predominant appearance was membranous glomerulonephritis (GN) in 10, subendothelial mesangiocapillary GN (MCGN) in three, and focal segmental glomerulosclerosis in two; one patient each had a focal proliferative GN and a diffuse endocapillary GN. On 1 micron renal sections stained with toluidine blue, 10 patients had immune deposits at multiple sites within the glomeruli. Over a period of one to 14 years, six patients developed extrarenal features suggestive of SLE, nine a positive ANA, and 12 increased serum levels of dsDNAB. Five patients became hypocomplementaemic. Cryoglobulins were isolated from the sera of 10 out of 12 patients; seven contained DNA. Separated cryoglobulin IgG from eight patients showed antibody activity directed against both ss and dsDNA in four, and against dsDNA only in three. On the basis of the clinical, histological and serological observation during follow-up five patients were reclassified as definite SLE, four as probable SLE and two as possible SLE. Rarely, SLE may present with nephritis as the sole disease manifestation, antedating other clinical features and even immunological markers of the disease by years. In addition, some patients with a glomerulonephritis may show clinical and immunological, or histological features of SLE, but do not fit accepted definitions of the disease.

Adolescent↗

Verrucous lupus erythematosus: ultrastructural studies on a distinct variant of chronic discoid lupus erythematosus.

Ten patients with classic discoid lupus erythematosus of the face associated with verrucous, papulonodular lesions on the arms and hands were studied by electron microscopy. The ultrastructural findings on the verrucous lesions included apoptotic keratinocytes, intraepidermal lymphocytes, and gapping, detachment, and reduplication of the basal lamina. Also, tubuloreticular inclusion bodies were present in the endothelial cells. These observations, together with the clinical, histopathologic, and immunofluorescence findings, suggest that the verrucous lesions represent a rare, but distinct, variant of chronic discoid lupus erythematosus.

Chronic Disease↗

Systemic lupus erythematosus.

Systemic lupus erythematosus is a polysystemic disease with a high incidence of associated glomerulonephritis. Patients with sle rarely have the destructive arthritis so characteristic of rheumatoid arthritis. An unusual case is presented in which both glomerulonephritis and destructive arthritis occurred simultaneously, justifying the diagnosis of both systemic lupus erythematosus and rheumatoid arthritis.Immunohistochemical studies in lupus glomerulonephritis suggest that the pathogenetic mechanisms involve the deposition of immune complexes containing "nuclear" antigens and antinuclear antibodies in the lesions. The detection of mixed cryoglobulins in the sera of patients with sle suggests that a portion of the circulating immune complexes may precipitate at reduced temperatures and be detected as mixed cryoglobulins. The therapy of lupus glomerulonephritis with combinations of corticosteroids and azathioprine, though still in an investigative state, holds great promise. Similar abnormalities in diseases of minks and mice and in sle suggest similar pathogenetic mechanisms in the three species involved. Since the diseases in the lower animals have been associated with persistent viral infection, the investigation of the role of persistent infection in sle seems warranted.

Adolescent↗

Lupus erythematosus in childhood.

Lupus erythematosus in childhood comprises the following distinctive lupus subsets: neonatal lupus erythematosus, systemic lupus erythematosus, discoid lupus erythematosus, subacute cutaneous lupus erythematosus, complement deficiency syndromes with subacute cutaneous lupus lesions, and lupus panniculitis. The clinical features and pathogeneses of each of these lupus syndromes is discussed in this review.

Antibodies, Antinuclear↗

The CLASI (Cutaneous Lupus Erythematosus Disease Area and Severity Index): an outcome instrument for cutaneous lupus erythematosus.

We developed and validated a measurement instrument (CLASI-Cutaneous Lupus Erythematosus Disease Area and Severity Index) for lupus erythematosus that could be used in clinical trials. The instrument has separate scores for damage and activity. A group of seven American Dermato-Rheumatologists and the "American College of Rheumatology Response Criteria Committee on SLE (systemic lupus erythematosus)" assessed content validity. After a preliminary session, we conducted standardized interviews with the raters and made slight changes to the instrument. The final instrument was evaluated by five dermatologists and six residents who scored nine patients to estimate inter- and intra-rater reliability in two sessions. Consultation with experts has established content validity of the instrument. Reliability studies demonstrated an intra-class correlation coefficient (ICC) for inter-rater reliability of 0.86 for the activity score (95% confidence interval (CI) = 0.73-0.99) and of 0.92 for the damage score (95% CI = 0.85-1.00). The Spearman's rho (Sp) for intra-rater reliability for the activity score was 0.96 (95% CI = 0.89 to 1.00) and for the damage score Sp was 0.99 (95% CI = 0.97-1.00). Clinical responsiveness needs to be evaluated in a prospective clinical trial, which is ongoing.

Humans↗

Tc-99m DTPA aerosol lung clearance test in systemic lupus erythematosus.

Systemic lupus erythematosus (SLE) can affect every organ. Involvement of the lung in systemic lupus erythematosus may be a significant cause of morbidity and mortality. To evaluate alveolar epithelial damage in SLE, we studied lung epithelial permeability by measuring the clearance of inhaled Tc-99m DTPA aerosol. Twenty-three SLE patients without clinical pulmonary manifestations were studied. Of the 23 patients, 16 had normal clearance rates of Tc-99m DTPA (T1/2 > 60mins) and normal chest X-ray findings. A mild increase in the clearance rate was found in 5 cases (T1/2 between 40-60 min) and a significant increase in clearance rate in the remaining 2 (T1/2 < 40mins). Totally, 30% (7/23) of SLE patients have abnormal lung permeability. Of the 7 patients with abnormal clearance of Tc-99m DTPA, 3 had abnormal findings in chest X-rays and four had normal chest X-rays. Our study suggests that the clearance of Tc-99m DTPA aerosol may be a useful complementary study to assess pulmonary involvement in SLE.

Adolescent↗

Possible discoid lupus erythematosus in newborn infant. Report of a case with subsequent development of acute systemic lupus erythematosus in mother.

Cases of a newborn infant with an eruption clinically and histologically consistent with lupus erythematosus and of his mother developing acute disseminate lupus erythematosus 11 months after delivery are presented. It is urged that in the future in cases of death of a fetus of a mother who has lupus erythematosus of any type or who gives a history of this disease, the fetus be examined for stigmata of lupus erythematosus. The possibility of a transmittable etiological agent of lupus erythematosus from mother to fetus is suggested.

Adult↗

Stroke subtypes among young patients with systemic lupus erythematosus.

PURPOSE: Systemic lupus erythematosus (lupus) is a systemic inflammatory disease associated with premature atherosclerosis, vasculitis, coagulopathy, and excessive incidence of stroke, especially among young patients. Little is known about subtypes of stroke in lupus. METHODS: A 20% sample of all the hospitalizations in the United States in the years 2001 and 2002 (N approximately 15 million) were analyzed to identify hospitalizations of young patients (age < or =50 years) with systemic lupus erythematosus (n=25704). Proportions of hospitalization for stroke subtypes were compared between the lupus group and the general population group. Age- and sex-adjusted odds ratios for stroke were calculated with logistic regression models. RESULTS: In the lupus group, there were 313 hospitalizations for stroke of which 206 hospitalizations had stroke as the primary diagnosis. Age- and sex-adjusted stroke risk was higher among the lupus group (odds ratio 1.5, 95% confidence interval 1.3-1.8). Patients with lupus had higher risk for all stroke subtypes except in subarachnoid hemorrhage in which a trend toward a lower risk was observed (odds ratio 0.57, 95% confidence interval 0.34-0.96). Although 12.3% (n=38) of stroke admissions in the lupus group resulted in in-hospital death, this case fatality rate was not statistically different from that for stroke in the general population group. CONCLUSIONS: Stroke is an important poor outcome in young patients with lupus. Compared with the general population, patients with lupus are more likely to be hospitalized for the risk of ischemic stroke and intracerebral hemorrhage. The risk of subarachnoid hemorrhage, however, seems to be lower in patients with lupus.

Adolescent↗

The in vitro effect of levamisole on histamine inhibited E-rosette forming T-lymphocyte subpopulation in patients affected by disseminated erythematosus lupus.

Decreased activity of T-suppressor cells plays an important role in the pathogenesis of lupus erythematosus disseminatus. The authors investigated the in vitro effect of levamisole on the histamine inhibited E-rosette forming T-lymphocyte subpopulation in patients affected by LED and in healthy persons. Histamine significantly inhibited the E-rosette forming T-lymphocyte subpopulation in the patient group as compared to controls. This effect of histamine could be reversed by levamisole. It may be anticipated that the E-rosette forming T-cell subpopulation which can be inhibited by histamine is identical to histamine-receptor carrying T-lymphocyte subpopulation with suppressor properties. The possible mechanisms of histamine induced inhibition of E-rosette forming T-cells has been discussed on the basis of literary data.

Histamine Antagonists↗

Sex-dependent effect of melatonin on systemic erythematosus lupus developed in Mrl/Mpj-Faslpr mice: it ameliorates the disease course in females, whereas it exacerbates it in males.

In this study, the effect of chronic administration of melatonin on MRL/MpJ-Fas(lpr) mice has been studied. These mice spontaneously develop an autoimmune disease that has many features resembling human systemic lupus erythematosus. In fact, histological studies showed that all female mice and most male mice exhibited glomerular abnormalities, arteritic lesions, and cellular interstitial inflammatory infiltrate ranging from mild to severe patterns. Treatment with melatonin improved the histological pattern in females and worsened it in males. Moreover, female mice treated with melatonin showed a diminution of titers of total serum IgG, IgM, and anti-double-stranded DNA and anti-CII autoantibodies; a decrease in proinflammatory cytokines (IL-2, IL-6, interferon-gamma, TNF-alpha, and IL-1beta), an increase in antiinflammatory cytokines (IL-10), and a decrease in nitrite/nitrate. In male mice, treatment with melatonin exhibited the opposite effect, worsening all the immunological parameters with an elevation of titers of autoantibodies and a prevalence of proinflammatory vs. antiinflammatory cytokines. Similar results were obtained when lymphocytes from spleen and lymph nodes were cultured. Again, melatonin treatment in females decreased proinflammatory cytokines and increased antiinflammatory cytokines produced by lymphocytes; in males, the effect was the opposite. These findings suggest that melatonin action in MRL/MpJ-Fas(lpr) mice is gender dependent, probably through modulation and inhibition of sex hormones.

Animals↗