Septic bursitis in systemic lupus erythematosus

Summary Septic olecranon bursitis developed in three patients with systemic lupus erythematosus. Although promptly treated with systemic antibiotics, the clinical course in two patients became prolonged and eventually required bursectomy to eradicate the infection. The occurrence of septic bursitis was a manifestation of the general susceptibility of SLE patients to infections.     

Lack of correlation between anticardiolipin antibodies and peripheral autonomic nerve involvement in systemic lupus erythematosus

Summary The presence of anticardiolipin antibodies has recently been related to a clinical complex in which both central and peripheral neurologic damage is included. A series of 27 female patients affected by systemic lupus erythematosus (SLE) was tested for the presence of peripheral autonomic neuropathy and serum anticardiolipin antibody (ACA) levels were determined in each patient by ELISA. Peripheral autonomic impairment was detected in 40.7% of SLE patients and a large number (77.7%) of patients had elevated levels of ACA. No relationship was found between presence of ACA (both for IgG and IgM classes) and the autonomic neuropathy.     

Prevalence and clinical associations of anticardiolipin antibodies in systemic lupus erythematosus: A prospective study

Summary The main objectives of the present study were to determine the prevalence of IgG and IgM anticardiolipin antibody (aCL) isotypes in systemic lupus erythematosus (SLE) in order to analyze their possible association with the clinical manifestations of the disease. Clinical features of 64 consecutive and unselected SLE patients were prospectively studied. Sera from the same patients taken during each clinical manifestation were tested for the presence of aCL. The prevalence of aCL was 43.75% for the IgG isotype and 9.4% for the IgM isotype. A strong linkage between the presence of these antibodies and the occurrence of both thrombosis and abortions was found: a weaker association with neurological events and thrombocytopenia was also demonstrated. The titre of aCL appeared to be linked with the probability of having the clinical manifestations associated with these autoantibodies. Our results suggest that thrombosis and abortion, and possibly thrombocytopenia and central nervous system involvement, may be associated with the presence of aCL at the time when these clinical events develop in SLE patients.     

Splendic and renal infarction in systemic lupus erythematosus: Association with anti-cardiolipin antibodies

Summary A 37-year-old female, known to have systemic lupus erythematosus (SLE) and markedly raised anti-cardiolipin antibody levels in association with the lupus anticoagulant, presented with a symptomatic segmental splenic infarction. There was a past history of cerebral infarction. Abdominal computed tomography (CT) demonstrated the area of splenic infarction, and an asymptomatic right renal infarct. This patient illustrates the unusual occurrence of multiple visceral infarcts, in association with anti-cardiolipin antibodies, complicating SLE.     

Asplenism and systemic lupus erythematosus

Summary Although functional hyposplenism, secondary to Fc-receptor blockage by circulating immune complexes saturation, has been described in systemic lupus erythematosus (SLE), only few cases of complete asplenism have been reported. We observed a 60-year-old woman with congenital asplenism who presented with active SLE. The course and the clinical characteristics of such patients are reviewed and the relationship between the asplenic state and initiation and severity of SLE are discussed. These patients are at high risk for fatal pneumococcemia and pneumococcal vaccine is recommended even if long term results are still conflicting.     

Recurrent acute proptosis in atypical systemic lupus erythematosus

Summary A patient is described who developed acute proptosis, a rare complication of systemic lupus erythematosus, 16 years after the onset of the disease. Treatment with intravenous methylprednisolone produced rapid improvement, but swelling recurred a few days later. Intravenous corticosteroid was again effective and was continued orally without further episodes of proptosis. This patient is unusual in the long period of mild disease before the development of systemic complications, and in having antibodies to nuclear ribonucleoprotein but persistently normal titres of DNA-binding antibodies in her serum.     

The coexistence of rheumatoid arthritis and systemic lupus erythematosus

Summary A 29-year-old white female with longstanding classical rheumatoid arthritis (RA) developed clinical and serological manifestations of systemic lupus erythematosus (SLE) with prominent signs of diffuse proliferative lupus nephritis. She fulfilled the ARA criteria for the classification of SLE as well as the ARA criteria for classical RA. The concomitant presence of these two affections in the same patient is rare and the discriminating features suggest that this coexistence may be coincidental. With respect to treatment, our patient had good relief of symptoms by a combined administration of methylprednisolone pulses and cyclophosphamide.     

Abnormalities of sex hormones in men with systemic lupus erythematosus

Summary Sex hormone concentrations were studied in 10 men with systemic lupus erythematosus (SLE) before and after HCG stimulation and compared with those of 10 healthy volunteers. The SLE men had significantly lower testosterone/estradiol ratios (before HCG test) and significantly lower mean increases in testosterone after HCG stimulation than the normals. Our findings confirm that there are sexual hormone abnormalities in SLE and suggest that these abnormal patterns may be important in the modulation of immune responses in SLE.     

Childhood systemic lupus erythematosus in songklanagarind hospital: a potential unique subgroup

Summary Twenty systemic lupus erythematosus patients under the age of 16 who had attended the Pediatric Department of Songklanagarind Hospital in the period 1985–1991 were reviewed retrospectively. The most common complaints were fever, allopecia, malar rash and oedema. The girl/boy ratio was 2.3/1. There were many unusual presentations such as Stevens-Johnson syndrome, psoriasis, rheumatoid nodules, papillitis and pseudotumor cerebri. Nephritis, haematological disorder and CNS involvement were found in 80, 50 and 35% respectively. All cases were positive for ANA. Sixty-five percent of cases had complications and mostly more than one. Common complications were infection, renal complication and side effects of chemotherapy. Five patients died because of renal failure, severe infections, cerebral infarction or GI bleeding.     

Fatal post-partum pulmonary vasculitis in systemic lupus erythematosus

Summary A patient with fatal acute pulmonary vasculitis complicating systemic lupus erythematosus (SLE) of ten years duration is described. The patient died seven days after an uneventful pregnancy and delivery. Pathologic examination demonstrated acute necrotizing changes as well as organized lesions in pulmonary arteries. Acute pulmonary arteritis is rare in SLE and may develop as one of the major complications of the disease in the potentially dangerous post-partum period.     

Methotrexate for steroid-resistant systemic lupus erythematosus

Summary We here report two patients with steroid-resistant systemic lupus erythematosus (SLE) who were successfully treated with methotrexate (MTX). In both cases, a steroid resistant high fever, associated with mild myositis and pancytopenia were the main common findings, and all these symptoms were alleviated within a few days either by 7.5 mg or 5 mg MTX per week. The number of CD4+ cells increased along with the clinical improvement, whereas the number of CD20+ cells and HLA-DR expressing cells also decreased. Taking into account the side effects of high dose corticosteroids and cyclophosphamides, treatment with a weekly low dose of MTX is known to contribute to an improvement in the long-term prognosis for patients with refractory SLE.     

Increased plasma fibronectin in patients with systemic lupus erythematosus

Summary To add to our knowledge of collagen diseases, plasma fibronectin (FN) in patients with systemic lupus erythematosus (SLE) has been measured, and it was determined that the plasma FN value in those with SLE was 454±36 g/ml, is significantly higher than the FN value in normal subjects (234±21 g/ml. Further, the plasma FN value of patients with active SLE was significantly higher (591±46 g/ml than that of patients with non-active SLE (287±31 g/ml. The plasma FN value of SLE patients was also seen to be associated with the peripheral blood platelet count and with the dose level of the corticosteroid hormone administered to patients. In active SLE patients, it was similarly found that the plasma FN value had a significant correlation with the peripheral blood lymphocyte count and with the dose level of the corticosteroid hormone given to patients. Since the plasma FN value is known to be high in untreated SLE patients, it was felt that the increase of the FN value in SLE patients is not due to the effect of the corticosteroid but to the disease itself.     

A case of Werner's syndrome associated with systemic lupus erythematosus

Summary The case of a 40-year-old woman with Werner's syndrome associated with systemic lupus erythematosus (SLE) is reported. The patient exhibited short stature, slender extremities, thinned hair, high-pitched voice, cataracts, ulceration of the fingers, and mental retardation. Malar erythema, photosensitivity, and proteinuria had been noted since age 34. The serum contained high titers of antibodies to dsDNA, Sm, nRNP, and SS-A/Ro. The simultaneous presence of Werner's syndrome and SLE could be a coincidental occurrence of the two diseases, although it might be due to an abnormality in replication or degeneration of DNA leading to the development of both diseases.     

Azathioprine in the treatment of systemic lupus erythematosus. A three-year prospective study

Summary In a prospective study, the effect of azathioprine on the clinical course and the anti-dsDNA profile was evaluated in 17 patients with systemic lupus erythematosus (SLE). During this prospective longitudinal study, exacerbations were never observed. Three periods of continuous anti-dsDNA increases with a doubling time (T2) shorter than 10 weeks were noted. Both the clinical symptoms and the anti-dsDNA levels improved after the administration of azathioprine. These results necessitate a careful double-blind trial for the use of this drug to prevent SLE exacerbations.     

Soluble intercellular adhesion molecule-1 (sICAM-1) in patients with systemic lupus erythematosus

Summary Circulating sICAM-1 is known to be elevated in various inflammatory disorders. It is further suggested that elevated levels correlate well with disease activity in several autoimmune disorders. The objectives of this study are to determine the serum sICAM-1 levels in patients with systemic lupus erythematosus (SLE) and correlate sICAM-1 levels with clinical and laboratory (ESR, CRP, anti-dsDNA) measures of disease activity. Forty-one patients (34 female, 7 male) all fulfilling 1982 ARA classification criteria for SLE and 16 healthy controls (8 female, 8 male) were included in the study. Disease activity was measured according to SLEDAI. sICAM-1 was determined by ELISA. Mean sICAM-1 in SLE patients (339±161 ng/ml) were significantly higher than in the controls (216±85 ng/ml) (p<0.005). Although slightly elevated in active patients, there was no statistically significant difference between mean sICAM-1 levels of active and inactive SLE patients (349±183 ng/ml and 316±103 ng/ml respectively) (p>0.05). We could not find a correlation between sICAM-1 levels and any organ involvements. Similarly, no significant correlation was found between ESR, CRP, anti-ds-DNA and sICAM-1. These results suggest that although higher than normal, sICAM-1 levels in SLE do not provide additional information over conventional activity markers.     

Recurrent peritonitis with ascites as the predominant manifestation of systemic lupus erythematosus

Summary We describe a patient with abdominal pain and ascites, mesenteric lymphadenitis and peritoneal panniculitis. Initially her ANA was negative. The abdominal pain recurred again three years later and in between the two episodes she had had skin rash, alopecia, arthralgia, and positive Coombs' test — haemolytic anaemia. Her ANA became positive a few years after the initial episode.     

Clinical and serological spectrum of systemic lupus erythematosus in greek children

Summary In the present study 19 Greek Caucasian children with systemic lupus erythematosus (SLE), onset before the age of 16, were followed up for 1–12 years (mean 5.6 yrs.). Diagnosis was determined early in 14 patients and delayed by 2 to 6 years in 5. The clinical manifestations and laboratory findings did not differ significantly from those reported in adults with lupus. The major organ system involvement at onset and early course were skin and joints (80%) followed by kidneys (42%). During the course of the disease 26% of the children developed central nervous system (CNS) involvement. All the patients were treated with steroids and/or cytotoxic drugs in severe uncontrolled progressive disease. At the mean 5.6 years follow-up most patients were in remission on small doses of steroids; one patient still presents signs of active lupus nephritis and one patient died from sepsis. All the patients with CNS involvement recovered without permanent CNS residue.     

Interleukin-1-production by monocytes from patients with systemic lupus erythematosus

Summary Production of interleukin-1 (IL-1) by glass-adherent monocytes from 18 patients with systemic lupus erythematosus (SLE) was measured. Patients were divided into three groups according to disease activity. A deficient production of IL-1 was found in monocytes of SLE patients both without stimulation and after stimulation with 5 g of lipopolysaccharide. The decreased production correlated with the degree of disease. Addition of phorbol myristate acetate to monocytes caused only partial normalization of the decreased IL-1 production. The IL-1 deficiency in SLE is postulated to be a part of complex abnormalities of cell-mediated immunity in this disease.     

Unusual eye manifestations in systemic lupus erythematosus patients

Summary One hundred and twelve records of patients with systemic lupus erythematosus were reviewed and four cases with unusual ocular manifestations are described. We found that anterior uveitis is not an uncommon manifestation of systemic lupus erythematosus and physicians must be aware of it during the patient's evaluation, since it can be treated without serious visual loss. Optic neuritis is uncommon in systemic lupus erythematosus and visual loss may be permanent despite therapy.     

Laminar periostitis and multiple osteonecrosis in systemic lupus erythematosus

Summary Bone affectation in systemic lupus erythematosus (SLE) is caused both by the disease itself and by the treatment used. We report the case of a woman diagnosed of SLE, who, in the course of her illness, develops multiple aseptic osteonecrosis (AON) and laminar periostitis, radiologically compatible with the diagnosis of hypertrophic osteoarthropathy (HOA), with no evidence of acropaquia. In this case, the patient shows all the risk factors involved in the pathogenesis of the development of ischemia in bone microcirculation.