Pure red cell aplasia in Thailand: report of twenty four cases

Twenty four cases of pure red cell aplasia were reported. No underlying diseases were found in two cases. Of the 22 cases with secondary form, 10 were from infections, mostly gram negative organisms. Three cases had systemic lupus erythematosus, two had autoimmune hemolytic anemia. The following conditions were found in one each: thymoma, thyroid carcinoma, protein calorie malnutrition, rheumatoid arthritis, non-Hodgkin lymphoma and Sheehan's syndrome. Three patients died, two from uncontrolled infection, the other from uncontrolled SLE and subsequently systemic fungal infection. Only one of the 2 primary cases responded to immunosuppressive drugs. The majority of patients with underlying infections, PRCA resolved after the infections were treated. This is the first reported series of PRCA in Thailand.     

Pregnancy outcome and autoantibodies in connective tissue disease

Pregnancy outcome before and after onset of disease and the association with present levels of anticardiolipin antibodies (aCL) and other autoantibodies were investigated in rheumatoid arthritis (RA) (117 patients), systemic lupus erythematosus (SLE) (74 patients), and systemic sclerosis (28 patients). Although 78% of the 81 pregnancies in patients with systemic sclerosis occurred before disease onset, pregnancy loss rate was highest in this disease (44%) but in RA (17%) and SLE (18%) was similar to a control population (16%). Elevated levels of IgG aCL were present in one of 4 patients with RA, in one of 5 patients with systemic sclerosis and in the only patient with SLE to have recurrent previous pregnancy loss. Congenital heart block occurred in one of 28 pregnancies from 17 anti-Ro (SSA) positive women with SLE.     

Coronary artery bypass grafting in patients with systemic lupus erythematosus

Cardiac involvement in patients with systemic lupus erythematosus (SLE) is common. The natural history of the cardiovascular manifestations has been altered by systemic corticosteroids used for the treatment of SLE; thus, young patients with SLE may suffer from angina and myocardial infarction. The surgical problems and special requirements in patients with SLE are discussed. CAD is one of the major complications limiting the prognosis of the patient with SLE. In the future, a large number of SLE patients may be candidates for myocardial revascularization. In our opinion, total autogenous arterial bypass grafting is advised and intraoperative biopsies of the LIMA are meaningful in patients with SLE.     

Lupus cystitis: a possible additive risk factor for emphysematous cystitis in diabetes mellitus: discussion about one case

Emphysematous cystitis (EC) is a rare condition in which gas-forming organisms are active in the bladder wall and lumen. Most of the cases have been described in patients suffering from diabetes mellitus due to glucosuria and subsequent anaerobic fermentation of glucose. To our knowledge this condition has never been described in association with systemic lupus erythematosus (SLE). We report here the first case of EC during the course of a chronic lupus cystitis (LC) in a woman suffering from SLE and type-I diabetes mellitus.     

Severe infections in systemic lupus erythematosus: disease pattern and predictors of infection-related mortality

Infection is a major cause of morbidity and mortality among patients with systemic lupus erythematosus (SLE). To describe the pattern of serious infections in patients with SLE and to identify the predictors of infection-related mortality among SLE patients with serious infections, we prospectively studied all SLE patients who were hospitalized with infections in Sarawak General Hospital during 2011–2015. Demographic data, clinical features, and outcomes were collected. Cox regression analysis was carried out to determine the independent predictors of infection-related mortality. There were a total of 125 patients with 187 episodes of serious infections. Our patients were of multiethnic origins with female predominance (89.6%). Their mean age was 33.4?±?14.2 years. The patients had a mean disease duration of 66.8?±?74.0 months. The most common site of infection was pulmonary (37.9%), followed by septicemia (22.5%). Gram-negative organisms (38.2%) were the predominant isolates within the cohort. There were 21 deaths (11.2%) during the study period. Independent predictors of infection-related mortality among our cohort of SLE patients were flare of SLE (HR 3.98, CI 1.30–12.21) and the presence of bacteremia (HR 2.54, CI 0.98–6.59). Hydroxychloroquine was protective of mortality from serious infections (HR 9.26, CI 3.40–25.64). Pneumonia and Gram-negative organisms were the predominant pattern of infection in our SLE cohort. The presence of flare of SLE and bacteremia were independent prognostic predictors of infection-related mortality, whereas hydroxychloroquine was protective of infection-related mortality among SLE patients with serious infections.     

系统性红斑狼疮并发感染的危险因素临床特点及早期诊断

感染是影响系统性红斑狼疮(SLE)患者病死率的重要因素之一,故早期诊断感染并发症能显著改善SLE患者预后。血清C-反应蛋白(CRP)、降钙素原(PCT)有助于鉴别SLE并发感染和SLE活动,血清KL-6有助于鉴别SLE伴发感染和SLE累及肺脏。SLE合并感染的病原体除了最多见的细菌感染外,病毒和真菌感染近几年有上升趋势,需要引起足够重视。     

The nature and outcome of infection in systemic lupus erythematosus

Infection remains a major cause of morbidity and mortality in systemic lupus erythematosus (SLE). To describe the nature and outcomes of infection and determine their associated risk factors in patients with SLE, we performed a nested case-control study at the University of Toronto Lupus Clinic, with prospective follow-up according to a standard protocol since 1970. Cases were SLE patients seen between January 1987 and January 1992 who had documented infections and controls were patients without infection from the same cohort matched for age, gender and time of visit. The type, site and outcome of infection were recorded for each case. A conditional logistic regression analysis was performed to compare factors associated with infection in cases and their controls. Ninety-three patients had 148 infection episodes; the majority were bacterial, but viral, fungal and protozoan organisms were also identified (multiple organisms in seven). Forty-eight patients required hospital admission and three patients died. Steroids at time of infection, as well as use ever, duration and dose, immunosuppressives at time of infection and use ever, active renal disease, CNS damage, SLEDAI at the time of infection, adjusted mean SLEDAI and variability measure were significantly associated with infection by univariate analysis. By multivariate analysis one factor remained statistically significant: use of steroids ever (P = 0.029). Infection carries a large burden for SLE patients. Until new medications which will control disease activity without predisposing to infection are developed, careful titration of steroids and cytotoxic drugs to control disease activity will remain crucial.     

Community-acquired pneumonia in Thai patients with systemic lupus erythematosus

Infection, particularly pneumonia, is a major cause of morbidity and mortality in patients with systemic lupus erythematosus (SLE). This study was performed to assess the prevalence, causative organisms, and outcomes of community-acquired pneumonia (CAP) in Thai SLE patients, and determine the predicting factors for death. A retrospective chart review of adult SLE patients, age >16 years, seen at the Division of Rheumatology, Chiang Mai University over an 18 year period was carried out. Cases diagnosed with CAP were selected for this study. Of 542 SLE patients, a total of 56 episodes of CAP occurred in 52 patients. Their mean age +/- SD and duration of SLE were 37.98 +/- 11.48 years and 34.99 +/- 54.53 months, respectively. Thirty-three CAP cases (58.9%) occurred within the first year of diagnosis with SLE. The causative organisms identifiable in 40 patients (71.5%) were Mycobacterium tuberculosis in 12, Nocardia spp in 6, Aspergillus spp in 5, Staphylococcus aureus in 3, Pneumocystis carinii, Haemophilus influenzae, Escherichia coli, and Pseudomonas aeruginosa in 2 each, and Acinetobactor baumanii, Burkholderia pseudomallei, and Strongyloides stercoralis in 1 each. The remaining 3 patients had mixed bacterial infection. The overall mortality rate was 26.8%. Use of high dose prednisolone (> or =15 mg/day), and ventilator support were significantly associated with death.     

Pneumococcal sepsis in patients with systemic lupus erythematosus

Severe infections by opportunistic agents and common pathogens are frequent in patients suffering from systemic lupus erythematosus (SLE) and have become one of the leading cause of death. Here we review all cases of Streptococcus pneumoniae septicaemia observed in a cohort of 208 SLE patients. Five cases were identified. We stress the severity of the clinical presentation and recommend immunization of SLE patients with a pneumococcal polysaccharide vaccine.     

Involvement of the eye in SLE and scleroderma. A study using fluorescein angiography in addition to clinical ophthalmic assessment.

General examination of the eye was carried out in 22 patients with systemic lupus erythematosus (SLE) and in 10 with scleroderma. 3 of the SLE and 2 of the scleroderma patients had keratoconjunctivitis sicca. Fluorescein angiography showed abnormalities of the retinal vasculature in one of a subgroup of 12 SLE patients and one of 10 scleroderma patients. None of the 12 SLE patients had abnormalities of the choroidal vasculature, while 5 of the 10 scleroderma patients had patchy areas of nonperfusion of the choroidal capillary bed.     

Choroidopathy of systemic lupus erythematosus

PURPOSE: To describe the ocular and systemic manifestations associated with systemic lupus erythematosus (SLE) choroidopathy. METHODS: Three new cases of choroidopathy in patients with active SLE were described. Twenty-five published cases of lupus choroidopathy were summarized. RESULTS: There have been 28 cases of lupus choroidopathy (47 involved eyes) that have been reported in the English literature since 1968, including the three current cases. Only two of the patients were male. The choroidopathy was bilateral in 19 patients (68%). All 28 patients (100%) had active systemic vascular disease at the onset of their choroidopathy; 18 (64%) had nephropathy and 10 (36%) had central nervous system (CNS) lupus vasculitis. All but one of the patients had a known diagnosis of SLE at the onset of choroidopathy. 30 of the 47 involved eyes had presenting visual acuity of 20/40 or better; 14 eyes showed improvement in visual acuity with therapy. 23 patients (82%) had resolution of their choroidopathy when their systemic disease was brought under control. Despite treatment, 4 of the 28 patients (14%) died from complications of SLE. CONCLUSIONS: Although less known than retinopathy, lupus choroidopathy may be more common than generally appreciated. It usually serves as a sensitive indicator of lupus activity. The presence of SLE choroidopathy is generally indicative of coexistent (although sometimes occult) nephropathy, CNS vasculitis, and other SLE visceral lesions. Immunomodulation of the systemic disease can lead to improvement and resolution of the systemic vasculitis as well as the choroidopathy.     

Systemic lupus erythematosus in North Indian Asians

Summary One hundred and one patients with systemic lupus erythematosus (SLE) from North Indian stock are presented. The clinical manifestations, laboratory parameters, causes of death, and survival are compared and contrasted with the other major reported series. SLE of North Indian Asians has several features comparable to those reported from the West, but other features are more similar to the SLE seen in Mongoloid races.     

Immunofluorescence studies of anti-T cell antibodies and T cells in systemic lupus erythematosus. Selective loss of brightly staining T cells in active disease.

Peripheral blood T cells from patients with systemic lupus erythematosus (SLE) and age- and sex-matched controls were studied by flow microfluorometry by using SLE anti-cell antibodies and fluorescein conjugated antibody to human IgM. Brightly staining cells were reduced in a number of patients with active SLE. Analysis of SLE T cells separated on a discontinuous stractin gradient indicated a preferential loss of cells, especially brightly staining cells, from one fraction. Quantitation of this phenomenon indicated that an average of greater than 90% of the brightly staining T cells from that fraction was lost in active SLE. This preferential loss of a subpopulation of T cells in patients with active SLE may be responsible for many of their immunologic abnormalities.     

Pulmonary hypertension in a lupus clinic: experience with twenty-four patients

The clinical and serological findings on 24 patients with pulmonary hypertension (PHT) seen at the Lupus Clinic of St. Thomas' Hospital, London are presented. Twenty-two patients had systemic lupus erythematosus (SLE), one other a primary antiphospholipid syndrome and another an SLE/progressive systemic sclerosis (PSS) overlap syndrome. In 21 of the 24 patients, the disease resembled the primary idiopathic variety with clear lung fields and no clinical evidence of pulmonary thromboembolism, although angiography and nuclear perfusion scans were not performed. Two patients clearly suffered from thromboembolic PHT, one with SLE and one with an antiphospholipid syndrome. One patient with SLE/PSS overlap syndrome developed pulmonary fibrosis. The frequency of antiphospholipid antibodies (lupus anticoagulant and antibodies to cardiolipin was 68% which appears to be higher than generally found in patients with SLE, and the clinical significance of this finding is unknown. Other associated features of the antiphospholipid syndrome in this group were uncommon. Death occurred in 13 of the 24 patients, 4 were lost to followup and 7 are known to be alive. The cause of death was circulatory failure in the majority; sudden death once this complication occurred was particularly common. One patient died from adult respiratory distress syndrome and one from hemorrhagic shock while undergoing heart/lung transplantation. Two patients underwent successful heart/lung transplantation. One, however, died of a mesenteric occlusion and bowel infarction following a second lung transplantation because of rejection of the first heart/lung transplantation after one year. The other patient is alive and well 2 years later.     

Lower limb pitting edema in systemic lupus erythematosus

We report two patients with lower limb pitting edema and systemic lupus erythematosus (SLE) who showed immediate response to systemic steroids. In one of the patients, the edema had been present for about 6 months and was the first manifestation of her SLE. In the second patient with a long history of SLE and antiphospholipid syndrome, a thrombosis was suspected, but not confirmed. Only after therapy with steroids did the edema disappear completely. Pitting edema of the lower limb could be a rare manifestation of SLE. Received: 11 August 1998 / Accepted: 11 September 1998     

Autoantibody against ribosomal protein L14 in patients with systemic lupus erythematosus

OBJECTIVE: To isolate a specific antibody against ribosomal protein L14 and to assess the relationship of this antibody with some of the clinicalfeatures in patients with systemic lupus erythematosus (SLE). METHODS: We screened the sera of SLE patients by immunoblotting analysis using rat total ribosomal proteins as antigen to determine whether sera had antibody activity against ribosomal proteins other than the P S10, and L12 proteins. The sera from 2 patients had antibody activity against a 30-kDa ribosomal protein. This antigenic protein was identified to be ribosomal protein L14 by two-dimensional gel electrophoresis and immunoblotting, so the antibody against L14 was tested by immunoblotting analysis using glutathione-S-transferase fusion human-L14 protein (GST-L14) as the antigen. We examined sera from 126 patients with SLE, and as controls sera from 67 patients with dermatomyositis and polymyositis (DM/PM), 71 patients with systemic sclerosis (SSc), and 74 healthy donors. RESULTS: Antibody activity against GST-L14 was detected in 7 out of 126 SLE, but not in any of the DM/PM, PSS, or healthy controls. CONCLUSION: Antibody against ribosomal protein L14 was specifically detected in sera from patients with SLE. Although this antibody activity was not so prevalent in the patients with SLE, it might be one of the useful tools for diagnosis of SLE.     

Urinary excretion of antinuclear antibodies

The frequency of antinuclear antibodies (ANA), the immunoglobulin class of ANA and their specificity for known nuclear antigens were determined in 24-h urine collections from patients with systemic lupus erythematosus (SLE) and progressive systemic sclerosis (PSS). Sixteen % of SLE patients had detectable urine ANA by indirect immunofluorescence using mouse kidney substrate. A higher incidence, 32% of SLE and 28% of PSS patients, had detectable ANA in a titer greater than or equal to 1:16 using HEp-2 cell substrate. IgG ANA was the most frequent immunoglobulin class of antibodies present in the urine; 56% of SLE and 29% of PSS patients with urine ANA had more than one immunoglobulin class of antibodies. Antibodies to Sm, nRNP, SS-A and dsDNA were detected in SLE urine; antibodies to SS-A and centromere were detected in PSS urine. Urine ANA detected on mouse kidney substrate and urine dsDNA antibodies correlated with diffuse proliferative glomerulonephritis in patients with SLE. Sixty-two% of SLE patients with urine ANA had proteinuria. In the remaining SLE patients and in all the PSS patients with urine ANA however, protein excretion was normal. SDS-PAGE revealed heavy and light immunoglobulin molecules in both SLE and PSS patients with urine ANA. The intact immunoglobulin was shown to have ANA activity. ANA present in the urine of SLE and PSS patients with apparently normal renal function may be an early sign of altered glomerular capillary membrane permeability.     

Systemic lupus erythematosus complicated with acute myocardial infarction and ischemic colitis

Acute myocardial infarction (AMI) is one of the most severe manifestations in patients with systemic lupus erythematosus (SLE). Ischemic colitis, mainly caused by intestinal vasculitis, is also one of the most serious, but uncommon, complications in SLE patients. "SLE vasculitis" simultaneously involving cardiac and gastrointestinal vessels has yet to be reported. This is the first report of SLE accompanying AMI, ischemic colitis and perforation of the digestive tract possibly due to SLE vasculitis, which was dramatically improved by treatment with high-dose glucocorticoid.     

DETECTION OF ANTIRIBOSOMAL P PROTEIN ANTIBODIES IN PATIENTS WITH SYSTEMIC SCLEROSIS

This study investigated the prevalence and clinical significanceof anti-ribosomal P protein (anti-P) antibodies in patientswith systemic sclerosis (SSc). Serum samples from 150 patientswith SSc were examined by indirect immunofluorescence, ELISAand immunoblotting. Anti-P antibodies were detected in four(3%) patients with SSc. Three of the four patients showed SSc/SLE(systemic lupus erythematosus) overlap syndrome, but psychiatricdisorders were not observed in these patients. By longitudinalimmunoblotting analysis one patient, who was initially diagnosedwith SSc, later developed anti-P antibodies along with clinicalmanifestations of SLE. Our data suggest that anti-P antibodiesare uncommon in SSc and that the presence of anti-P antibodiesin patients with SSc indicates an overlap with SLE. KEY WORDS: Anti-ribosomal P protein antibodies, Systemic sclerosis, Systemic lupus erythematosus     

Juvenile systemic lupus erythematosus in multicase families from Saudi Arabia: comparison of clinical and laboratory variables with sporadic cases

The object of this study was to compare patients with familial versus sporadic systemic lupus erythematosus (SLE) with respect to clinical, laboratory variables and outcome. The familial SLE group comprised 12 patients while the comparative group comprised 24 patients selected by systemic sampling from our pediatric rheumatology clinic database. Those patients are listed according to the date of referral, which represents a sampling frame. The first patient was chosen randomly and subsequent patients were chosen at intervals of three. The two groups were compared with respect to: demographic information, age of onset of SLE, disease and follow up duration, clinical and laboratory variables and outcome. The patients from the familial group were younger and had an earlier age of onset of disease (P = 0.03, 0.001 respectively). Seven patients with familial SLE were from the eastern region of Saudi Arabia (P = 0.006). The two groups were comparable with respect to gender, disease duration and follow-up. At diagnosis, the discoid rash was more frequent in the familial group (P = 0.03) while other clinical and laboratory variables including disease activity as measured by SLEDAI did not show significant differences. The mean dose of steroid and use of other immunosuppressive therapy were similar in both groups. Three patients from the familial group died; two of them had unusual complications (one patient had transverse myelitis and pancreatic pseudocyst and the other one had extensive pyoderma gangrenosum). All patients from the sporadic group are alive in stable condition but one patient had severe central nervous system disease. Familial SLE patients tend to be younger and more likely to have discoid rash, in addition a marked difference in the origin of patients was noted. These differences may be helpful in identifying SLE patients with a stronger genetic predisposition. The mortality among familial SLE patients is more frequent which may reflect the disease severity.