Disease outcomes and ovarian function of childhood-onset systemic lupus erythematosus.

The objective of this study was to determine the medical outcomes including the ovarian function childhood-onset SLE (cSLE). The medical records of all patients diagnosed with cSLE in the Greater Cincinnati area between 1981 and 2002 were reviewed. Patient interviews were performed to obtain additional information on current medication regimens, disease activity [SLE Disease Activity Index (SLEDAI-2k)], and damage [Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SDI)]. The occurence of premature ovarian failure (POF) and reduction of the ovarian reserve was assessed by timed gonadotropin levels. There were 77 patients (F : M = 70 : 7, 53% Caucasian, 45% African-American and 2% Asian) with a mean age at diagnosis of 14.6 years. Nine patients died (88.3% survival) during the mean follow-up of 7.1 years (standard deviation [SD] 5.6) and 88% of the patients continued to have active disease (SLEDAI-2k mean/SD: 6.6/6.7), with 42% of them having disease damage (SDI mean/SD: 1.62/2.1); Non-Caucasian patients had higher disease activity (mean SLEDAI-2k: 10 versus 3.4; P < 0.0001) and more disease damage (mean SDI : 2.1 versus 1.2; P < 0.02) than Caucasian patients. Cyclophosphamide was given to 47% of the patients during the course of their disease and associated with the presence of significantly reduced ovarian reserve (RR = 2.8; 95% CI: 1.7-4.8; P = 0.026). Patient mortality and disease damage with cSLE continue to be high. Although overt POF with cyclophosphamide exposure is rare, it is a risk factor for significantly decreased ovarian reserve cSLE.

Seropositive, symmetric polyarthritis in a patient with poorly differentiated lung carcinoma: carcinomatous polyarthritis, hypertrophic osteoarthropathy, or rheumatoid arthritis?

Polyarthritis resembling rheumatoid arthritis (RA) may be the presenting manifestation of occult malignancy. Hypertrophic osteoarthropathy (HOA) may also develop in association with pulmonary neoplasia and consists of clubbing, periostitis, and arthropathy. We describe a patient who presented with a seropositive, symmetric, inflammatory polyarthritis only 4 weeks before a lung tumor became clinically and radiographically apparent. After initiation of chemotherapy, she developed features characteristic of HOA. It appears that the patient had both RA and HOA. We discuss the differential diagnosis and review the relationship of RA, HOA, carcinomatous polyarthritis, and malignancy.

Accelerated atherosclerosis and coronary disease in SLE.

Atherosclerosis may represent a significant cause of death and morbidity in patients with systemic lupus erythematosus. Coronary involvement is more premature in lupus patients. We present the case of a young woman diagnosed with SLE at the age of 20 years who had a myocardial infarction at age 29 years. We review the mechanisms of atherosclerosis, the interrelations between atherosclerosis and autoimmunity, and between atherosclerosis and SLE. We also review the risk factors, influence of disease and treatment and the guidelines for management of accelerated atherosclerosis in lupus patients.

Etiology, environmental relationships, epidemiology, and genetics of systemic lupus erythematosus.

The etiology of systemic lupus erythematosus (SLE) and the many lupuslike syndromes continues to challenge investigators. Focus is now on the role of heat-shock proteins, apoptosis, the possible role of diet factors, and in particular, the role of lipids. The role of various infections as either triggering mechanisms or in contributing to morbidity is receiving close attention. In particular, retroviruses are being carefully studied with all the molecular tools available. This area has real promise and carries with it the possibility of anti-infection treatments. Considerably more attention is being paid to the hormonal aspects of SLE and their modulation of the immune system. Environmental associations continue to intrigue investigators and clinicians, and both drugs and other environmental factors provide excellent investigational models. We continue to need good prevalence and incidence studies. Genetically, there is an increasing sophistication in the type of studies, and the ensuing data may well provide real insights into various subsets of SLE.

Vasculitis complicating granulocyte colony stimulating factor treatment of leukopenia and infection in Felty's syndrome.

Recombinant myeloid growth factors have been increasingly used in recent years to combat induced and disease associated neutropenia. Their application in the management of Felty's syndrome with intercurrent infection has raised concern that resultant neutrophilia and activation of a diverse array of polymorphonuclear cell functions may have an adverse effect on the rheumatoid disease process. We describe a patient with Felty's syndrome receiving short term treatment with recombinant human granulocyte colony stimulating factor (GCSF), who then developed acute renal failure in conjunction with leukocytoclastic vasculitis and presumptive gout. We address the issue of "adding fuel to the fire" and review reported implications of GCSF in induction of vasculitis.

Epidemiology, genetics, etiology, and environment relationships of systemic lupus erythematosus.

Systemic lupus erythematosus continues to provide a major etiologic challenge. Current investigation is focusing on the possibilities of environmental factors, including infection. How these factors are related to genetic factors, including the major histocompatibility complex and more recently observed defects in apoptosis genes, remains unclear. Ethnic and geographic studies of systemic lupus erythematosus are providing important clues, as are continued clinical observations on the various subsets of disease and the patterns of therapeutic response. Different pathogenic mechanisms are constantly being uncovered and in turn need to be related to the various etiologies.