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Co-twin control study on cerebral manifestations of systemic lupus erythematosus.

All available twin pairs systemic lupus erythematosus (SLE) derived from the Finnish Twin Cohort were studied by clinical evaluation, magnetic resonance imaging (MRI), anticardiolipin (aCL), and antineurofilament (ANFA) antibodies. One of the five monozygotic and one of the eight dizygotic pairs were concordant for SLE. 10 of the 15 patients showed clinical neurological abnormalities, and 11 had abnormal MRI of the brain. Altogether, 12 patients were considered to have neuropsychiatric lupus (NPSLE). Seven of the 11 patients with long-term corticosteroid treatment had either central or cortical atrophy. High or moderate aCL level was found in eight patients and two co-twins. Of them, six patients had at least two manifestations of the antiphospholipid syndrome. ANFAs were found in five patients and four co-twins. Five co-twins fulfilled some of the SLE criteria. Of them, three MZ twins and one additional DZ co-twin with no ARA criteria had findings suggesting central nervous system (CNS) involvement. The results indicate that the majority of SLE patients has cerebral abnormalities either as a result of SLE, or concomitant risk factors. The co-twins without clinical SLE often have minor signs of SLE, and even they may have neurological and MRI abnormalities. However, their aCL and ANFA levels seem not to correlate with MRI abnormalities.

Adolescent↗

Systemic lupus erythematosus: medical and nursing treatments.

Systemic lupus erythematosus is a multisystem autoimmune disease that may affect skin, joints, mucous membranes, heart, lungs, kidneys, nervous system and all the blood cell lines. Although its cause is unknown, abnormal immune function results in the formation of antibodies directed against various components of the human body (autoantibodies). Treatment depends of the severity of the illness and may include nonsteroidal antiinflammatory agents for arthritis; antimalarial therapy for skin disease and other mild lupus manifestations; and corticosteroids and immunosuppressive agents including azathioprine, cyclophosphamide, and methotrexate for more severe lupus manifestations. Persons affected by lupus and their families need help in understanding the condition and require support as they deal with fear, depression, and possible disability. Implications for nursing are varied and include patient/family education about medication, joint protection principles, energy conservation, pain and stress management, and coping techniques.

Female↗

Systemic lupus erythematosus: two patients treated with danazol.

Two women with systemic lupus erythematosus who showed premenstrual flaring of the disease were treated with danazol, which resulted in clinical improvement.This form of treatment needs to be assessed more widely, as there is little reported evidence that hormonal regulation has therapeutic value in systemic lupus erythematosus.

Adult↗

Kikuchi's disease (necrotizing lymphadenitis) with cutaneous involvement associated with subacute cutaneous lupus erythematosus.

Necrotizing histiocytic lymphadenopathy (Kikuchi's disease) is a rarely observed clinical entity characterized by fever, and solitary or multiple lymphadenopathy predominantly in the posterior cervical region. Kikuchi's disease has been reported to precede, coexist with or follow the diagnosis of systemic lupus erythematosus. In only rare instances has its association with cutaneous lupus erythematosus without systemic involvement been reported. We report a 45-year-old woman who presented characteristic systemic and cutaneous manifestations of Kikuchi's disease. Several months later, after sun exposure, she developed lesions of subacute cutaneous lupus erythematosus. The American Rheumatism Association criteria for systemic lupus erythematosus were not fulfilled. The possible pathogenic relationships between the two processes are discussed.

Female↗

Synchronization of plasmapheresis and pulse cyclophosphamide in severe systemic lupus erythematosus.

Two patients with severe systemic lupus erythematosus, who had not responded to conventional therapy, were treated with plasmapheresis and subsequent pulse cyclophosphamide. This approach uses the plasmapheresis-induced proliferation of pathogenic clones for partial clonal deletion by giving large doses of cytotoxic drugs during the assumed period of increased B-cell vulnerability. Both patients had a rapid and distinct improvement in clinical symptoms and laboratory parameters, including the control of a life-threatening case of lupus pneumonitis. Their conditions were stabilized by giving low-dose cyclophosphamide for the next 6 months. At 14-month follow-up, there were no clinical signs of relapse.

Administration, Oral↗

Anti-SS-A antibodies and fetal outcome in maternal systemic lupus erythematosus.

In 155 women with systemic lupus erythematosus, there were no significant differences in the rates of fertility or adverse fetal outcome between the 47 (30%) with serum anti-SS-A antibody and the 108 without anti-SS-A, except in the frequency of congenital heart block. This complication occurred in 6 of 96 pregnancies in women with anti-SS-A and was associated with high-titer maternal antibody. The overall risk of a woman with lupus having an infant with congenital heart block was estimated to be 1:60, but the risk was considerably higher (1:20) if anti-SS-A antibody was present.

Abortion, Spontaneous↗

Oxygen-15 brain scanning for detection of cerebral involvement in systemic lupus erythematosus.

In 47 patients with systemic lupus erythematosus seen during fifty-one clinical episodes, oxygen-15, a short-lived gamma-emitting isotope, has been employed in a scannng technique to study cerebral oxygen utilisation and blood-flow. Abnormalities in regional distribution of oxygen utilisation and blood-flow were seen in twenty-three out of twenty-four instances of definite central-nervous-system disease, in fourteen out of fifteen instances of suspected C.N.S. lupus, and in ten out of twelve instances in which C.N.S. disease was not clinically apparent. The technique reflected remissions and relapses. It may prove valuable in diagnosis of subclinical cerebral disease, in monitoring of responses to therapy, and in study of the pathophysiology of cerebral lupus.

Adolescent↗

A comparison of damage accrual across different calendar periods in systemic lupus erythematosus patients.

Therapeutic approaches in systemic lupus erythematosus (SLE) have evolved over the last few decades, but their impact on prevention of organ damage is unknown. The objective of this study was to compare new cumulative damage in SLE patients across different calendar periods. Patients from a large SLE cohort were divided into two subcohorts; the first diagnosed and followed between 1978 and 1988 (cohort #1, n=100) and the second between 1989 and 1999 (cohort #2, n=51). Initial Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SLICC/ACR DI) scores, and changes in scores over the observation intervals, were compared for the two groups. Logistic regression estimated adjusted odds ratios (OR) comparing damage accrual between the two cohorts. Medication exposures were noted. Baseline characteristics were similar between the two groups. At first assessment, the adjusted OR for a SLICC/ACR DI score > or =1 was 1.79 (95% CI 0.82, 3.88) for cohort #1 versus cohort #2. At the end of the observation interval, the adjusted OR for a SLICC/ACR DI score > or =1 was 1.22 (0.58, 2.55) for cohort #1 versus cohort #2. The adjusted OR for accruing damage over the observation interval in cohort #1 versus cohort #2 was 0.94 (0.39, 2.44). Increased medication exposure was evident for cohort #2 compared to cohort #1. Despite increased therapeutic measures used for patients in more recent periods, our data do not establish a clear difference in damage accrual. This emphasizes the need for strategies to effectively treat lupus-specific manifestations, while minimizing side effects and comorbidities.

Adrenal Cortex Hormones↗

Malignant fibrous histiocytoma arising from discoid lupus erythematosus.

While the association of discoid lupus erythematosus (DLE) and subsequent squamous cell carcinoma is well known, there are no reports in the English-language literature concerning the association of DLE and soft-tissue sarcoma. We describe a patient with DLE and malignant fibrous histiocytoma.

Histiocytoma, Benign Fibrous↗

[Pulmonary hypertension in generalized lupus erythematosus].

Two young women with systemic lupus erythematosus and pulmonary hypertension are reported. Both patients had Raynaud phenomenon and antibodies to cardiolipin. They seem to be potentially associated in the pathogenesis. Also, laboratory and hemodynamic findings were discussed. Although the treatment, the prognosis still bad.

Adult↗

Choroidopathy in a case of systemic lupus erythematosus.

Choroidopathy in association with systemic lupus erythematosus (SLE) has rarely been reported. We report a patient with SLE and choroidopathy manifested by bilateral multifocal, serous elevations of the neurosensory retina. Control of the systemic disease resulted in resolution of the serous detachment.

Adult↗

Chilblain lupus erythematosus lesions precipitated by the cold.

Lupus erythematosus (LE) may exhibit a broad array of clinical presentations. Cutaneous manifestations include malar rash, discoid lesions, alopecia, and panniculitis. Cold-induced lesions are uncommon. To illustrate this unusual presentation, we describe a case of typical chilblain LE.

Abdomen↗

Paraneoplastic pemphigus: a case of long-term survival associated with systemic lupus erythematosus and polymyositis.

A 35-year-old man presented with a generalized bullous eruption and oral ulceration. He had been diagnosed as having systemic lupus erythematosus and pelvic Castleman disease (hyaline-vascular type) in the past. Histologic, direct and indirect immunofluorescence and immunoprecipitation studies confirmed a diagnosis of paraneoplastic pemphigus (PNP). Initially several medical treatments were tried unsuccessfully. The pelvic tumor was surgically removed and the mucocutaneous lesions slowly regressed. Four years after presentation, he developed polymyositis which was completely controlled with short courses of corticosteroids. There was no evidence of relapse of PNP or lupus erythematosus at that time. At the 6-year follow-up he showed no clinical evidence of PNP, lupus erythematosus or polymyositis without requiring immunosuppressive therapy. This case emphasizes the fact that patients with benign-neoplasm-associated PNP may undergo complete remission of the autoimmune disease upon complete excision of the tumor. This case also stresses the possible association of PNP with other autoimmune diseases such as lupus erythematosus and polymyositis.

Adult↗

Intermittent cyclophosphamide for the treatment of autoimmune thrombocytopenia in systemic lupus erythematosus.

STUDY OBJECTIVE: To determine the effect of monthly intravenous cyclophosphamide therapy in patients with systemic lupus erythematosus and autoimmune thrombocytopenia. DESIGN: Uncontrolled, retrospective clinical study. SETTING: Government referral-based research hospital. PATIENTS: Seven patients with systemic lupus erythematosus and 2 or more months of thrombocytopenia refractory to or requiring excessive doses of corticosteroids. Two patients had also failed to respond to splenectomy and repeated intravenous methylprednisolone infusions. Six patients had severe active renal disease at the time of treatment. INTERVENTIONS: Cyclophosphamide, 0.75 to 1.0 g/m2 body surface area, was given intravenously every month for at least 4 months. Prednisone dose ranged between 0.5 to 1.0 mg/kg.d. MEASUREMENTS AND MAIN RESULTS: All seven patients had normal platelet counts within 2 to 18 weeks after cyclophosphamide treatment (one to four doses). Prednisone was tapered to 0.25 mg/kg on alternate days in all patients. All six patients had significant improvement in their renal disease and lupus serologies. Cyclophosphamide was discontinued after four to six doses in five patients. Four patients maintained normal platelet counts on low dose, alternate-day prednisone for a mean of 5.6 years of follow-up. Two patients had recurrence of thrombocytopenia 1 to 3 years after discontinuing cyclophosphamide. CONCLUSIONS: Monthly intravenous cyclophosphamide is potentially useful for the management of autoimmune thrombocytopenia in patients with systemic lupus erythematosus who are refractory to or dependent on unacceptably high doses of corticosteroids, or are experiencing side effects of conventional medical or surgical treatment.

Autoimmune Diseases↗

Optic neuritis and myelopathy in systemic lupus erythematosus.

The optic neuritis of systemic lupus erythematosus (S.L.E.) more frequently results in the persistence of a central scotoma or complete blindness after a single attack than demyelinating optic neuritis, although the initial clinical presentations may be identical. A significant number of patients, however, recover normal vision. Optic neuritis may be the presenting symptom of S.L.E. and as myelopathy may also occur in the course of the disease, confusion with multiple sclerosis may result, especially if there are no arthritic, cutaneous nor visceral manifestations. We report a case of lupus optic neuritis associated with anticardiolipin antibodies and a circulating lupus anticoagulant and suggest these may be a marker for vasculitic optic neuritis and play a role in its aetiology.

Antibodies↗